wikidoc - User contributions [en]

Glucagonoma medical therapy

2017-11-20T16:52:16Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}
==Overview==
The predominant medical therapy for primary glucagonoma is [[Somatostatin|somatostatin analogs]] ([[octreotide]]). Metastatic tumors need [[Therapeutic embolization|hepatic artery embolization]], [[radiofrequency ablation]], and molecular therapy.

== Management of Primary Tumor ==
* [[Somatostatin|Somatostatin analogs]] ([[octreotide]]) are the treatment of choice to control symptoms.<ref name="pmid25489112">{{cite journal| author=Rosenbaum A, Flourie B, Chagnon S, Blery M, Modigliani R| title=Octreotide (SMS 201-995) in the treatment of metastatic glucagonoma: report of one case and review of the literature. | journal=Digestion | year= 1989 | volume= 42 | issue= 2 | pages= 116-20 | pmid=2548911 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2548911 }}</ref>
* [[Doxorubicin]] and [[streptozotocin]] have also been used successfully to selectively damage [[alpha cells]] of the pancreatic islets.
===Drug regimen===
Preferred regimen (1): [[Octreotide]] 400 micrograms/day

== Metastasis Therapy ==
=== [[Therapeutic embolization|'''Hepatic artery''' '''embolization''']] ===
*Hepatic arterial [[embolization]] is a palliative treatment in patients with symptomatic [[Hepatic metastasis|hepatic metastases]] who are not candidates for surgical resection.
*[[Therapeutic embolization|Embolization]] can be performed via the infusion through an [[Angiography|angiography catheter]] into [[Hepatic artery|hepatic arteries]].

=== '''[[Radiofrequency ablation]]''' ===
*[[Ablation]] can be performed [[percutaneously]] or [[Laparoscopic surgery|laparoscopically]] in patients with symptomatic hepatic metastases who are not candidates for surgical resection.
*Ablation is applicable only to smaller lesions less than 3 cm.<ref name="pmid12967136">{{cite journal| author=Gupta S, Yao JC, Ahrar K, Wallace MJ, Morello FA, Madoff DC et al.| title=Hepatic artery embolization and chemoembolization for treatment of patients with metastatic carcinoid tumors: the M.D. Anderson experience. | journal=Cancer J | year= 2003 | volume= 9 | issue= 4 | pages= 261-7 | pmid=12967136 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12967136 }}</ref>

=== '''Molecular therapy''' ===
*[[Sunitinib]] is a radio-labeled [[somatostatin]] analog which has a role in the management of glucagonoma's that are not symptomatic or have rapidly progressive metastasis.

==References==
{{reflist|2}}
{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma other imaging findings

2017-11-20T16:43:36Z

Anthony Gallo: /* Other Imaging Findings */

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}
==Overview==
Other imaging studies for glucagonoma include [[positron emission tomography]] scan and somatostatin receptor [[scintigraphy]]. Scintigraphy is less sensitive than [[Positron emission tomography|PET]] scan but still useful.

==Other Imaging Findings==

===Positron Emission Tomography===
* When performing a PET scan, a small amount of a radioactive medium is injected into your body and absorbed by the organs or tissues.
* This radioactive substance gives off energy which in turn is used to produce the images.
* Positron emission tomography can provide more helpful information than either CT or MRI scans. It is useful to see if cancer has spread to the [[lymph nodes]] and it is also useful to locate where cancer has spread.

=== Octreoscan ===
* Many glucagonoma's express high levels of somatostatin receptors and can be imaged with a radiolabeled form of the [[somatostatin analog]] [[octreotide]].<ref name="pmid12021920">{{cite journal| author=Papotti M, Bongiovanni M, Volante M, Allìa E, Landolfi S, Helboe L et al.| title=Expression of somatostatin receptor types 1-5 in 81 cases of gastrointestinal and pancreatic endocrine tumors. A correlative immunohistochemical and reverse-transcriptase polymerase chain reaction analysis. | journal=Virchows Arch | year= 2002 | volume= 440 | issue= 5 | pages= 461-75 | pmid=12021920 | doi=10.1007/s00428-002-0609-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12021920 }}</ref>

* It is less sensitive than Ga DOTATATE PET scan with gadolinium but still useful if Ga DOTATATE is not available.
<gallery>
Image:Scintigraphy.jpg|Somatostatin receptor scintigraphy with SPECT acquisition. Area of increased uptake anterior to the left kidney and medial to the spleen, consistent with the pancreatic mass<ref name="pmid23259638">{{cite journal| author=Erdas E, Aste N, Pilloni L, Nicolosi A, Licheri S, Cappai A et al.| title=Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? | journal=BMC Cancer | year= 2012 | volume= 12 | issue= | pages= 614 | pmid=23259638 | doi=10.1186/1471-2407-12-614 | pmc=PMC3543729 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23259638 }} </ref>

Image:Neuroendocrine-tumour-pancreas.jpg|Octreoscan.Case courtesy of Radswiki rID: 11670

</gallery>

==References==
{{reflist|2}}
{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma ultrasound

2017-11-20T16:35:08Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}

==Overview==
The abdominal ultrasound scan may be helpful in the diagnosis of glucagonoma. Finding on ultrasound scan suggestive of glucagonoma is a hypoechoic tumor in the distal [[pancreas]]. US-guided [[fine-needle aspiration]] biopsy is a non-operative histologic diagnosis. Intraoperative ultrasonography is used as an adjunct to intraoperative palpation.

==Ultrasound==
The ultrasound findings associated with glucagonoma are:<ref name="pmid21964743">{{cite journal| author=Atiq M, Bhutani MS, Bektas M, Lee JE, Gong Y, Tamm EP et al.| title=EUS-FNA for pancreatic neuroendocrine tumors: a tertiary cancer center experience. | journal=Dig Dis Sci | year= 2012 | volume= 57 | issue= 3 | pages= 791-800 | pmid=21964743 | doi=10.1007/s10620-011-1912-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21964743 }}</ref><ref name="pmid12918465">{{cite journal| author=Koike N, Hatori T, Imaizumi T, Harada N, Fukuda A, Takasaki K et al.| title=Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus. | journal=J Hepatobiliary Pancreat Surg | year= 2003 | volume= 10 | issue= 1 | pages= 101-5 | pmid=12918465 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12918465 }} </ref><ref name="pmid16231278">{{cite journal| author=Hellman P, Hennings J, Akerström G, Skogseid B| title=Endoscopic ultrasonography for evaluation of pancreatic tumours in multiple endocrine neoplasia type 1. | journal=Br J Surg | year= 2005 | volume= 92 | issue= 12 | pages= 1508-12 | pmid=16231278 | doi=10.1002/bjs.5149 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16231278 }}</ref>
* Endoscopic ultrasonography can detect lesions as small as 2 mm.
* It is more sensitive than CT or transabdominal ultrasonography for detection of glucagonoma.
* US-guided [[fine-needle aspiration]] biopsy is a good non-operative way to histologically diagnose glucagonoma.
* Finding of a hypoechoic tumor in the distal pancreas on ultrasound is suggestive of glucagonoma.
* Intraoperative ultrasonography is used as an adjunct to intraoperative palpation.
[[File:O0201af1.jpg|center|500px]]

==References==
{{reflist|2}}
{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma MRI

2017-11-20T16:03:19Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}

==Overview==
Abdominal MRI is helpful in the diagnosis of glucagonoma. On abdominal MRI, glucagonoma is characterized by a mass which is hypointense on T1-weighted MRI and hyper-intense on T2-weighted MRI. MRI is preferred over contrast-enhanced CT or [[somatostatin receptor]] scintigraphy for assessing metastasis.

==MRI==
*Findings on abdominal MRI suggestive of glucagonoma include:<ref name="pmid25789004">{{cite journal| author=Lv WF, Han JK, Liu X, Wang SC, Pan BO, Xu AO| title=Imaging features of glucagonoma syndrome: A case report and review of the literature. | journal=Oncol Lett | year= 2015 | volume= 9 | issue= 4 | pages= 1579-1582 | pmid=25789004 | doi=10.3892/ol.2015.2930 | pmc=PMC4356379 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25789004 }} </ref>
**T1: low signal intensity
**T2: high signal intensity
*MRI is preferred over contrast-enhanced CT or [[somatostatin receptor]] scintigraphy hy for assessing metastasis.

==References==
{{reflist|2}}
{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma CT

2017-11-20T15:59:28Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}

==Overview==
Findings on abdominal CT scan suggestive of glucagonoma include a reinforced mass in the arterial phase of the enhanced CT scan. Symptomatic but nonfunctioning glucagonomas are usually large (>3 cm) at the time of diagnosis.

==CT==
The CT scan findings associated with glucagonoma include:<ref name="pmid25789004">{{cite journal| author=Lv WF, Han JK, Liu X, Wang SC, Pan BO, Xu AO| title=Imaging features of glucagonoma syndrome: A case report and review of the literature. | journal=Oncol Lett | year= 2015 | volume= 9 | issue= 4 | pages= 1579-1582 | pmid=25789004 | doi=10.3892/ol.2015.2930 | pmc=PMC4356379 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25789004 }} </ref><ref name="pmid21067742">{{cite journal| author=Khashab MA, Yong E, Lennon AM, Shin EJ, Amateau S, Hruban RH et al.| title=EUS is still superior to multidetector computerized tomography for detection of pancreatic neuroendocrine tumors. | journal=Gastrointest Endosc | year= 2011 | volume= 73 | issue= 4 | pages= 691-6 | pmid=21067742 | doi=10.1016/j.gie.2010.08.030 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21067742 }}</ref><ref name="pmid9423664">{{cite journal| author=Paulson EK, McDermott VG, Keogan MT, DeLong DM, Frederick MG, Nelson RC| title=Carcinoid metastases to the liver: role of triple-phase helical CT. | journal=Radiology | year= 1998 | volume= 206 | issue= 1 | pages= 143-50 | pmid=9423664 | doi=10.1148/radiology.206.1.9423664 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9423664 }}</ref><ref name="pmid9574609">{{cite journal| author=Legmann P, Vignaux O, Dousset B, Baraza AJ, Palazzo L, Dumontier I et al.| title=Pancreatic tumors: comparison of dual-phase helical CT and endoscopic sonography. | journal=AJR Am J Roentgenol | year= 1998 | volume= 170 | issue= 5 | pages= 1315-22 | pmid=9574609 | doi=10.2214/ajr.170.5.9574609 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9574609 }}</ref><ref name="pmid20231629">{{cite journal| author=Wang SC, Parekh JR, Zuraek MB, Venook AP, Bergsland EK, Warren RS et al.| title=Identification of unknown primary tumors in patients with neuroendocrine liver metastases. | journal=Arch Surg | year= 2010 | volume= 145 | issue= 3 | pages= 276-80 | pmid=20231629 | doi=10.1001/archsurg.2010.10 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20231629 }}</ref>
* CT scans are used to determine the location of the tumor, show the organs nearby, determining the stage of cancer and in determining whether surgery is a good treatment option.
* Spiral multi phasic contrast-enhanced CT is recommended.
* Sensitivity is greater than 80 percent but it is decreased for tumors smaller than 2 cm.
* With contrast, glucagonomas often enhance with iodinated contrast during the early arterial phase with washout during the [[portal vein|portal venous]] imaging phase.
* Liver metastases may appear isodense with the liver on a non-contrasted study.
* Symptomatic but nonfunctioning glucagonomas are usually large (>3 cm) at the time of diagnosis.
<gallery>Image:Contrast-enhanced computed tomography scan of the abdomen. A 3-cm nodular mass in the head of the [[pancreas]] was seen. There was no evidence of metastases..jpg|A 3-cm nodular mass in the head of the pancreas<ref name="pmid25029913">{{cite journal| author=Wu SL, Bai JG, Xu J, Ma QY, Wu Z| title=Necrolytic migratory erythema as the first manifestation of pancreatic neuroendocrine tumor. | journal=World J Surg Oncol | year= 2014 | volume= 12 | issue= | pages= 220 | pmid=25029913 | doi=10.1186/1477-7819-12-220 | pmc=PMC4105234 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25029913 }} </ref>
Image:NEM23.jpg|Computed tomography scan of the abdomen showing a large tumor in the tail of the pancreas<ref name="pmid25152626">{{cite journal| author=Fang S, Li S, Cai T| title=Glucagonoma syndrome: a case report with focus on skin disorders. | journal=Onco Targets Ther | year= 2014 | volume= 7 | issue= | pages= 1449-53 | pmid=25152626 | doi=10.2147/OTT.S66285 | pmc=PMC4140234 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25152626 }} </ref>
</gallery>

==References==
{{reflist|2}}

{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma laboratory tests

2017-11-20T15:58:25Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}

==Overview==
Laboratory findings consistent with the diagnosis of glucagonoma include a serum [[glucagon]] concentration of 1000 pg/ml or greater.<ref name="pmid15313692">{{cite journal| author=Zhang M, Xu X, Shen Y, Hu ZH, Wu LM, Zheng SS| title=Clinical experience in diagnosis and treatment of glucagonoma syndrome. | journal=Hepatobiliary Pancreat Dis Int | year= 2004 | volume= 3 | issue= 3 | pages= 473-5 | pmid=15313692 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15313692 }} </ref><ref name="lab">Glucagonoma. Wikipedia. https://en.wikipedia.org/wiki/Glucagonoma. Accessed on October 15,2015</ref>

==Laboratory Findings==
The laboratory findings associated with glucagonoma are:<ref name="pmid15313692" /><ref name="pmid8606627" /><ref name="pmid17873310">{{cite journal| author=Kindmark H, Sundin A, Granberg D, Dunder K, Skogseid B, Janson ET et al.| title=Endocrine [[pancreatic tumors]] with [[glucagon]] hypersecretion: a retrospective study of 23 cases during 20 years. | journal=Med Oncol | year= 2007 | volume= 24 | issue= 3 | pages= 330-7 | pmid=17873310 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17873310 }}</ref>

'''Serum glucagon'''
*Increased plasma [[glucagon]] levels >500 pg/mL (Normal [[Glucagon|glucagon level]] is less than 50 pg/mL).
*Concentrations above 1000 pg/mL are diagnostic of glucagonoma.
*Some conditions can increase [[glucagon]] level to a level less than 500 pg/mL such as fasting and [[hypoglycemia]].
*Some symptomatic glucagonomas are associated with normal glucagon serum level. So, a serum glucagon concentration below 500 pg/mL does not exclude a glucagonoma.<ref name="pmid17873310" />
'''Other laboratory findings'''
* Blood tests may also reveal low concentrations of [[amino acids]], [[zinc]], and [[essential fatty acid]]s.
* A [[complete blood count|complete blood count]] ([[complete blood count|CBC]]) to diagnose [[anemia]].

==References==
{{reflist|2}}
{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma physical examination

2017-11-20T15:57:04Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}

==Overview==
Common physical examination findings of glucagonoma include [[tachycardia]], [[fever]], [[rash]], [[muscle atrophy|muscle atrophy.]] [[Cotton wool spots]], [[Flame hemorrhages|flame hemorrhage]], and [[dot-blot hemorrhages]] on [[fundoscopic]] examination of the eye may be present.

==Physical Examination==
Common physical examination findings of glucagonoma include:
===General Appearance===
*Patients with glucagonoma are generally well-appearing
*Patient may appear thin and [[Cachexia|cachectic]] in advanced cases
===Vital Signs===
*[[Tachycardia]] may be present
*[[Fever]] may be present
===Skin===
*[[Rash|Rash:]] Erythematous, ring shaped [[rash]] that blisters, erodes, and crusts over suggesting [[necrolytic migratory erythema]]
===HEENT===
* The ophthalmoscopic exam may be abnormal with findings of [[cotton wool spots]], [[Flame hemorrhages|flame hemorrhage]], and dot and blot hemorrhage.

===Heart===
* [[Tachycardia]] may be present

===Extremities===
*Muscle [[atrophy]] may be present
*Unilateral calf or thigh tenderness
*Unilateral calf or thigh swelling
*Unilateral calf or thigh warmth
*Unilateral calf or thigh erythema
*Palpable cord (a thickened palpable vein suggestive of thrombosed vein)
*Dilatation of unilateral collateral superficial veins
===Neuromuscular===
*Patient is oriented to persons, place, and time
*[[Hyporeflexia]] may be present
*Unilateral/bilateral [[sensory loss]] in the upper/lower extremity may be present
*Muscle [[atrophy]] may be present
*Vibration and Joint position sense may be decreased

==References==
{{reflist|2}}

{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma history and symptoms

2017-11-20T15:55:30Z

Anthony Gallo: /* Less common symptoms include: */

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}

==Overview==
Symptoms of glucagonoma include [[necrolytic migratory erythema]], [[weight loss]], [[glucose intolerance]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]]. A positive family history of [[multiple endocrine neoplasia type 1]] may be present.

==History==
* When evaluating a patient for glucagonoma, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough review of past medical history.
* Other specific areas of focus when obtaining the history include the family history of predisposing genetic disorders such as [[multiple endocrine neoplasia type 1|multiple endocrine neoplasia type 1.]]

==Symptoms==

===Common symptoms of glucagonoma===
Common symptoms of glucagonoma include:<ref name="pmid8606627">{{cite journal| author=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV| title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients. | journal=Medicine (Baltimore) | year= 1996 | volume= 75 | issue= 2 | pages= 53-63 | pmid=8606627 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8606627 }} </ref><ref name="pmid4793623">{{cite journal| author=Wilkinson DS| title=Necrolytic migratory erythema with carcinoma of the pancreas. | journal=Trans St Johns Hosp Dermatol Soc | year= 1973 | volume= 59 | issue= 2 | pages= 244-50 | pmid=4793623 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4793623 }}</ref><ref name="pmid86066272">{{cite journal| author=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV| title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients. | journal=Medicine (Baltimore) | year= 1996 | volume= 75 | issue= 2 | pages= 53-63 | pmid=8606627 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8606627 }}</ref><ref name="pmid6268399">{{cite journal| author=Stacpoole PW| title=The glucagonoma syndrome: clinical features, diagnosis, and treatment. | journal=Endocr Rev | year= 1981 | volume= 2 | issue= 3 | pages= 347-61 | pmid=6268399 | doi=10.1210/edrv-2-3-347 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6268399 }}</ref>
*[[Necrolytic migratory erythema]] (NME) is a classical symptom observed in patients with glucagonoma and is present in 80% of cases. Associated NME is characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure, including the lower [[abdomen]], [[buttock]]s, [[perineum]], and [[groin]].
*[[Weight loss]]
*[[Glucose intolerance|Glucose intolerance]]

=== Less common symptoms include: ===
Less common symptoms of glucagonoma include:<ref name="pmid62683992">{{cite journal| author=Stacpoole PW| title=The glucagonoma syndrome: clinical features, diagnosis, and treatment. | journal=Endocr Rev | year= 1981 | volume= 2 | issue= 3 | pages= 347-61 | pmid=6268399 | doi=10.1210/edrv-2-3-347 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6268399 }}</ref><ref name="pmid15201270">{{cite journal| author=Chang-Chretien K, Chew JT, Judge DP| title=Reversible dilated cardiomyopathy associated with glucagonoma. | journal=Heart | year= 2004 | volume= 90 | issue= 7 | pages= e44 | pmid=15201270 | doi=10.1136/hrt.2004.036905 | pmc=1768315 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15201270 }}</ref><ref name="pmid22350605">{{cite journal| author=Cheema A, Weber J, Strosberg JR| title=Incidental detection of pancreatic neuroendocrine tumors: an analysis of incidence and outcomes. | journal=Ann Surg Oncol | year= 2012 | volume= 19 | issue= 9 | pages= 2932-6 | pmid=22350605 | doi=10.1245/s10434-012-2285-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22350605 }}</ref>
*[[Cheilosis]]
*[[Hemorrhage retroperitoneal|intraabdominal hemorrhage]]
*[[Venous thrombosis]]
*[[Stomatitis]]
*[[Diarrhea]]
*[[Polyuria]]
*[[Polydipsia]]
*[[Polyphagia]]
*[[Blurred vision]]
*Neuropsychiatric manifestations include [[depression]], [[dementia]], [[psychosis]], and [[agitation]]
*[[Dilated cardiomyopathy]]

* Some patients do not present with hormonal symptoms but present with metastatic symptoms in other organs and mainly the liver.
**These symptoms include [[jaundice]], [[edema]], and [[abdominal pain]].
**There is usually high level of substances such as chromogranins, [[enolase]], [[pancreatic polypeptide]], and [[ghrelin]] in patients with metastses.

* Some tumors can develop additional syndromes subsequently due to secretion of more than one hormone.<gallery>Image:Close-up view of well demarcated erythematous plaques, with fragile vesicles on gluteal area..jpg|Close-up view of well demarcated erythematous plaques, with fragile vesicles on gluteal area<ref name="pmid25029913">{{cite journal| author=Wu SL, Bai JG, Xu J, Ma QY, Wu Z| title=Necrolytic migratory erythema as the first manifestation of pancreatic neuroendocrine tumor. | journal=World J Surg Oncol | year= 2014 | volume= 12 | issue= | pages= 220 | pmid=25029913 | doi=10.1186/1477-7819-12-220 | pmc=PMC4105234 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25029913 }} </ref>
Image:NEM1.jpg|Necrolytic migratory erythema with erosion and crust formation<ref name="pmid25152626">{{cite journal| author=Fang S, Li S, Cai T| title=Glucagonoma syndrome: a case report with focus on skin disorders. | journal=Onco Targets Ther | year= 2014 | volume= 7 | issue= | pages= 1449-53 | pmid=25152626 | doi=10.2147/OTT.S66285 | pmc=PMC4140234 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25152626 }} </ref>
Image:Glucagonoma1.jpg|Skin eruptions. A) Erythema, scaling, erosions and crusts on the face. B) Intense erythema with crusted erosions at perineum. C) Polycyclic migratory lesions with scaling advancing borders at groin folds. D) Glossitis<ref name="pmid23259638">{{cite journal| author=Erdas E, Aste N, Pilloni L, Nicolosi A, Licheri S, Cappai A et al.| title=Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? | journal=BMC Cancer | year= 2012 | volume= 12 | issue= | pages= 614 | pmid=23259638 | doi=10.1186/1471-2407-12-614 | pmc=PMC3543729 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23259638 }} </ref>
</gallery>

==References==
{{reflist|2}}
{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma staging

2017-11-20T15:53:34Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}

==Overview==
According to The American Joint Committee on Cancer (AJCC), there are four stages of glucagonoma based on the [[TNM staging system]].
==Staging==
According to The American Joint Committee on Cancer (AJCC), there are four stages of glucagonoma based on the [[TNM staging system]].<ref name="stage">Stage Information for Pancreatic Neuroendocrine Tumors (Islet Cell Tumors). http://www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq#section/_199</ref><ref name="pmid20180029">{{cite journal| author=Edge SB, Compton CC| title=The American Joint Committee on Cancer: the 7th edition of the AJCC cancer staging manual and the future of TNM. | journal=Ann Surg Oncol | year= 2010 | volume= 17 | issue= 6 | pages= 1471-4 | pmid=20180029 | doi=10.1245/s10434-010-0985-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20180029 }}</ref>
===TNM Classification===

{| class="wikitable"
! style="font-weight: bold;" | T
! style="text-align: center; font-weight: bold;" | [[Primary Tumor]]
|-
| style="font-weight: bold;" | Tx
| style="text-align: center;" | Primary tumor cannot be assessed
|-
| style="font-weight: bold;" | T0
| style="text-align: center;" | No evidence of primary tumor
|-
| style="font-weight: bold;" | Tis
| style="text-align: center;" | [[Carcinoma in situ]]
|-
| style="font-weight: bold;" | T1
| style="text-align: center;" | Tumor limited to the [[pancreas]], ≤2 cm in greatest dimension
|-
| style="font-weight: bold;" | T2
| style="text-align: center;" | Tumor limited to the [[pancreas]], >2 cm in greatest dimension
|-
| style="font-weight: bold;" | T3
| style="text-align: center;" | Tumor extends beyond the pancreas but without involvement of the celiac axis or the [[superior mesenteric artery]]
|-
| style="font-weight: bold;" | T4
| style="text-align: center;" | Tumor involves the celiac axis or the [[superior mesenteric artery]] (unresectable primary tumor)
|}

{| class="wikitable"
! N
! Regional Lymph Nodes
|-
| '''NX'''
| Regional lymph nodes cannot be assessed
|-
| '''N0'''
| No regional lymph node metastasis
|-
| '''N1'''
| Regional lymph node metastasis
|}

{| class="wikitable"
! style="font-weight: bold;" | M
! Distant Metastases
|-
| style="font-weight: bold;" | M0
| No distant metastasis
|-
| style="font-weight: bold;" | M1
| Distant metastasis
|}

===Anatomic Stage/Prognostic Groups===
{| class="wikitable"
! style="font-weight: bold;" | Stage
! style="font-weight: bold;" | T
! style="font-weight: bold;" | N
! style="font-weight: bold;" | M
|-
| style="font-weight: bold;" | 0
| Tis
| N0
| M0
|-
| style="font-weight: bold;" | IA
| T1
| N0
| M0
|-
| style="font-weight: bold;" | IB
| T2
| N0
| M0
|-
| style="font-weight: bold;" | IIA
| T3
| N0
| M0
|-
| rowspan="3" style="font-weight: bold;" | IIB
| T1
| N1
| M0
|-
| T2
| N1
| M0
|-
| T3
| N1
| M0
|-
| style="font-weight: bold;" | III
| T4
| Any N
| M0
|-
| style="font-weight: bold;" | IV
| Any T
| Any N
| M1
|}

==References==
{{reflist|2}}
{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma natural history, complications and prognosis

2017-11-17T14:41:14Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}

==Overview==
If left untreated, patients with glucagonoma may progress to develop [[necrolytic migratory erythema]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]]. The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma. The 10-year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis. Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis. Age, grade, and distant metastases are the most significant predictors of survival.

==Natural History==
*If left untreated, patients with glucagonoma may progress to develop [[necrolytic migratory erythema]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]].
*Glucagonoma has a very slow growth rate compared to most malignant tumors.

== Complications ==
Complications of glucagonoma include:
* Metastasis:
**Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis.
**Metastasis occurs mainly in the [[liver]] but in few cases, it can occur in [[lymph nodes]], [[peritoneum]], [[lung]], and [[Adrenal gland|adrenals]].
* Weight loss: due to the catabolic effect of [[glucagon]], most patients lose weight.
* [[Anemia]]
* Neuropsychiatric manifestations include [[depression]], [[dementia]], [[psychosis]], and [[agitation]]
* Reversible [[dilated cardiomyopathy]]<ref>{{Cite journal
| author = [[K. Chang-Chretien]], [[J. T. Chew]] & [[D. P. Judge]]
| title = Reversible dilated cardiomyopathy associated with glucagonoma
| journal = [[Heart (British Cardiac Society)]]
| volume = 90
| issue = 7
| pages = e44
| doi = 10.1136/hrt.2004.036905
| pmid = 15201270
}}</ref>

==Prognosis==
Prognosis of glucagonoma depends on the following:
*Whether or not the tumor can be removed by surgery.
*The [[stage of the tumor]], the size of the tumor, whether cancer has [[spread]] outside [[the pancreas]].
*The patient’s general health.
*Whether the tumor has just been diagnosed or has recurred.
*The presence of [[metastasis]] is associated with a particularly poor prognosis among patients with glucagonoma.
*The 10-year event free [[survival rate]] is less than 51.6% with [[metastasis]] and 64.3% without metastasis.

Additionally:
* [[Age]], [[grade]], and distant [[metastases]] are the most significant predictors of survival.
* Five and 10-year survival rates for patients undergoing resection of gastroenteropancreatic [[neuroendocrine tumors]].<ref name="pmid8606627">{{cite journal| author=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV| title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients. | journal=Medicine (Baltimore) | year= 1996 | volume= 75 | issue= 2 | pages= 53-63 | pmid=8606627 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8606627 }}</ref>
* Sixty percent of glucagonomas are [[malignant]]. Once the tumor is [[Metastasis|metastatic]], the cure is rare.<ref>{{Cite journal

| author = [[M. A. Chastain]]
| title = The glucagonoma syndrome: a review of its features and discussion of new perspectives
| journal = [[The American journal of the medical sciences]]
| volume = 321
| issue = 5
| pages = 306–320
| year = 2001
| month = May
| pmid = 11370794
}}</ref>

==References==
{{reflist|2}}

{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma screening

2017-11-17T14:29:07Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}

==Overview==
Screening of [[multiple endocrine neoplasia type 1]] associated glucagonoma improves [[morbidity]] and [[survival rates]] of patients. Biochemical screening depends on measuring gastrointestinal hormones such as [[gastrin]], [[insulin]], [[glucagon]], [[VIP]], [[pancreatic polypeptide]], [[chromogranin A]], [[prolactin]], and [[IGF-1]] in all patients. Radiological screening should include an [[MRI]] of [[Pancreas|the pancreas]], [[adrenal glands]], and [[Pituitary gland|pituitary]] and repeated every 2 years. All high-risk patients should be offered [[genetic counseling]] and [[MEN1]] mutation testing. If the genetic tests result patients should have a periodic clinical, biochemical, and radiological screening program.

==Screening==
* Screening of [[multiple endocrine neoplasia type 1]] associated glucagonoma improves [[morbidity]] and [[survival rates]] of patients.
* The [[survival rate]] of early diagnosed and treated glucagonoma is 85% while this rate falls to 60% in patients with malignant disease.
* Sporadic cases present in their fifth decade whereas patients with MEN I present at the younger age of 33 y. So, familiar cases of glucagonoma should be diagnosed and treated as fast as possible.<ref name="pmid86066272">{{cite journal| author=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV| title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients. | journal=Medicine (Baltimore) | year= 1996 | volume= 75 | issue= 2 | pages= 53-63 | pmid=8606627 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8606627 }}</ref><ref name="pmid8606627">{{cite journal| author=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV| title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients. | journal=Medicine (Baltimore) | year= 1996 | volume= 75 | issue= 2 | pages= 53-63 | pmid=8606627 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8606627 }}</ref>
* Criteria of screening:<ref name="pmid21454234">{{cite journal| author=Newey PJ, Thakker RV| title=Role of multiple endocrine neoplasia type 1 mutational analysis in clinical practice. | journal=Endocr Pract | year= 2011 | volume= 17 Suppl 3 | issue= | pages= 8-17 | pmid=21454234 | doi=10.4158/EP10379.RA | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21454234 }}</ref>
** A first-degree relative of family member with known [[MEN1]] mutation.
** Asymptomatic first-degree relative.
** First-degree relative with familial [[MEN1|MEN1,]] for example, one MEN1-associated tumor.
** Suspicious multiple [[Parathyroid adenoma|parathyroid adenomas]] before the age of 40 yr; recurrent [[hyperparathyroidism]]; [[gastrinoma]] or [[Neuroendocrine tumors|multiple pancreatic NET]] at any age) or atypical for [[MEN1]] (i.e. development of two nonclassical [[MEN1]]-associated tumors, e.g. [[Parathyroid adenoma|parathyroid]] and [[adrenal tumor]]).

* Patients should be screened as early as possible; Before 5 yr of age for asymptomatic patients.
* Biochemical screening for the development of [[MEN1]] tumors in asymptomatic members of families with MEN1 is likely to be of benefit in as much as earlier diagnosis and treatment of these tumors may help reduce morbidity and mortality. Screening for MEN1 tumors is difficult because clinical and biochemical manifestations in members of any one family are not uniformly similar.

==== [[Biochemical screening]] ====
* Biochemical screening depended on measuring:<ref name="pmid19622622">{{cite journal| author=Newey PJ, Jeyabalan J, Walls GV, Christie PT, Gleeson FV, Gould S et al.| title=Asymptomatic children with multiple endocrine neoplasia type 1 mutations may harbor nonfunctioning pancreatic neuroendocrine tumors. | journal=J Clin Endocrinol Metab | year= 2009 | volume= 94 | issue= 10 | pages= 3640-6 | pmid=19622622 | doi=10.1210/jc.2009-0564 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19622622 }}</ref>
** Serum concentrations of [[calcium]] in all patients as the earliest manifestation for [[MEN1]] is [[hyperparathyroidism]].
** Gastrointestinal hormones: [[Gastrin]], [[insulin]], [[glucagon]], [[pancreatic polypeptide]], [[chromogranin A]], [[Prolactin|prolactin,]] and [[IGF-1]] in all patients.

* Pancreatic involvement in asymptomatic patients has been detected by measuring fasting plasma concentrations of [[gastrin]], [[pancreatic polypeptide]], [[glucagon]], and [[chromogranin A]] and by abdominal imaging.<ref name="pmid20833329">{{cite journal| author=Thakker RV| title=Multiple endocrine neoplasia type 1 (MEN1). | journal=Best Pract Res Clin Endocrinol Metab | year= 2010 | volume= 24 | issue= 3 | pages= 355-70 | pmid=20833329 | doi=10.1016/j.beem.2010.07.003 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20833329 }}</ref>
* Screening should be done at least once annually and also have baseline [[Pituitary gland|pituitary]] and [[abdominal imaging]] which should then be repeated at 1- to 3-yr intervals and it should be repeated throughout life because the disease may not manifest in some patients until the eighth decade.

==== [[Radiological screening]] ====
* Radiological screening should include an [[MRI]] of the [[pancreas]], [[Adrenal gland|adrenal glands]], and [[pituitary]] and repeated every 2 years.<ref name="pmid1677362">{{cite journal| author=Skogseid B, Eriksson B, Lundqvist G, Lörelius LE, Rastad J, Wide L et al.| title=Multiple endocrine neoplasia type 1: a 10-year prospective screening study in four kindreds. | journal=J Clin Endocrinol Metab | year= 1991 | volume= 73 | issue= 2 | pages= 281-7 | pmid=1677362 | doi=10.1210/jcem-73-2-281 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1677362 }}</ref>

==== Genetic counselling ====
* All high-risk patients should be offered [[genetic counseling]] and [[MEN1]] mutation testing. If the genetic tests result patients should have a periodic clinical, biochemical, and radiological screening program.<ref name="pmid21454234" />
** This may include examination for mutations in genes associated with familial [[hyperparathyroidism]] including [[CDC73]] associated with the [[HPT|HPT-JT]] and the [[Calcium-sensing receptor|calcium sensing receptor]] ([[CASR]]), or [[cyclin-dependent kinase]] 1B (CDKN1B) and [[AIP]] which are rarely identified in those with clinical [[MEN1]].

== References ==
{{reflist|2}}
{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma risk factors

2017-11-17T14:27:28Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}
==Overview==
The most common risk factor in the development of glucagonoma is a positive family history of [[multiple endocrine neoplasia type 1|multiple endocrine neoplasia type1]] which is characterized by the presence of [[Pituitary adenoma|pituitary adenomas]], [[Islet cell tumor|islet cell tumors]] of the [[pancreas]], and [[hyperparathyroidism]].
==Risk Factors==
* The most common risk factor in the development of glucagonoma is a positive family history of [[multiple endocrine neoplasia type 1]].<ref name="pmid22970401">{{cite journal| author=Afsharfard A, Atqiaee K, Lotfollahzadeh S, Alborzi M, Derakhshanfar A| title=Necrolytic migratory erythema as the first manifestation of glucagonoma. | journal=Case Rep Surg | year= 2012 | volume= 2012 | issue= | pages= 974210 | pmid=22970401 | doi=10.1155/2012/974210 | pmc=PMC3434377 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22970401 }} </ref><ref name="causes">Glucagonoma. U.S. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/000326.htm</ref>
** It is an [[Autosomal dominant inheritance|autosomal dominant]] syndrome that is usually caused by mutations in the [[MEN1 syndrome|''MEN1'' gene]].
** It is characterized by the development of the following tumors:[[Multiple endocrine neoplasia type 1 pathophysiology#cite note-wikipedia-1|[1]]]
*** [[Pituitary adenoma|Pituitary adenomas]]
*** [[Islet cell tumor|Islet cell tumors]] of the [[pancreas]] (commonly [[gastrinoma]] and glucagonoma)
*** [[Parathyroid]] [[hyperplasia]] with resulting [[hyperparathyroidism]]

==References==
{{reflist|2}}
{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma epidemiology and demographics

2017-11-17T14:26:50Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}

==Overview==
The [[incidence]] of glucagonoma is approximately 0.0005 per 100,000 individuals worldwide. Glucagonoma affects men and women equally. The median age of diagnosis is the fifth decade.

==Epidemiology and Demographics==
=== Incidence ===
* Annual [[incidence]] is 0.01 to 0.1 new cases per 100,000.<ref name="pmid22261919">{{cite journal| author=Jensen RT, Cadiot G, Brandi ML, de Herder WW, Kaltsas G, Komminoth P et al.| title=ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. | journal=Neuroendocrinology | year= 2012 | volume= 95 | issue= 2 | pages= 98-119 | pmid=22261919 | doi=10.1159/000335591 | pmc=3701449 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22261919 }}</ref>
* Most glucagonomas are [[sporadic]] but up to 20 percent may be associated with the [[Multiple endocrine neoplasia syndrome type 1|multiple endocrine neoplasia syndrome type 1.]] Glucagonomas occur in only 3 percent of [[MEN1]] patients.<ref name="pmid15657529">{{cite journal| author=Lévy-Bohbot N, Merle C, Goudet P, Delemer B, Calender A, Jolly D et al.| title=Prevalence, characteristics and prognosis of MEN 1-associated glucagonomas, VIPomas, and somatostatinomas: study from the GTE (Groupe des Tumeurs Endocrines) registry. | journal=Gastroenterol Clin Biol | year= 2004 | volume= 28 | issue= 11 | pages= 1075-81 | pmid=15657529 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15657529 }}</ref><ref>{{Cite journal
| author = [[Rajesh V. Thakker]], [[Paul J. Newey]], [[Gerard V. Walls]], [[John Bilezikian]], [[Henning Dralle]], [[Peter R. Ebeling]], [[Shlomo Melmed]], [[Akihiro Sakurai]], [[Francesco Tonelli]] & [[Maria Luisa Brandi]]
| title = Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1)
| journal = [[The Journal of clinical endocrinology and metabolism]]
| volume = 97
| issue = 9
| pages = 2990–3011
| year = 2012
| month = September
| doi = 10.1210/jc.2012-1230
| pmid = 22723327
}}</ref>

=== Age ===
* Patients typically present in their fifth decade with lesions mainly located in the tail of [[the pancreas]], whereas patients with MEN I present at the younger age of 33 y.<ref name="pmid86066272">{{cite journal| author=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV| title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients. | journal=Medicine (Baltimore) | year= 1996 | volume= 75 | issue= 2 | pages= 53-63 | pmid=8606627 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8606627 }}</ref><ref name="pmid8606627">{{cite journal| author=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV| title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients. | journal=Medicine (Baltimore) | year= 1996 | volume= 75 | issue= 2 | pages= 53-63 | pmid=8606627 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8606627 }}</ref>

=== Gender ===
*Glucagonoma affects men and women equally.

==References==
{{reflist|2}}

{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma differential diagnosis

2017-11-17T14:21:24Z

Anthony Gallo: /* Differentiating glucagonoma from other causes of hyperglycemia: */

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}} {{MAD}}
==Overview==
Glucagonoma must be differentiated from certain skin lesions in which necrolytic migratory erythema can be found such as [[acrodermatitis enteropathica]], [[psoriasis]], [[pellagra]], and [[eczema]]. Glucagonoma should be differentiated from other causes of hyperglycemia include [[infection]], [[diabetes mellitus]], [[Cushing syndrome]], [[renal failure]], [[acute pancreatitis]], severe stress, and prolonged fasting.

==Differentiating Glucagonoma from other Diseases==
Glucagonoma must be differentiated from certain skin lesions in which necrolytic migratory erythema can be found such as:<ref name="pmid25152626">{{cite journal| author=Fang S, Li S, Cai T| title=Glucagonoma syndrome: a case report with focus on skin disorders. | journal=Onco Targets Ther | year= 2014 | volume= 7 | issue= | pages= 1449-53 | pmid=25152626 | doi=10.2147/OTT.S66285 | pmc=PMC4140234 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25152626 }} </ref>
* [[Pemphigus foliaceus]]
* [[Pustular psoriasis]]
* [[Atopic dermatitis|Chronic eczema]]
* [[Acrodermatitis enteropathica]]
* [[Pellagra]]

{| class="wikitable"
! rowspan="2" |Disease
! colspan="3" |Clinical Picture
! rowspan="2" |Investigations
! rowspan="2" |Pictures
|-
!History
!Symptoms
!Signs
|-
|Glucagonoma<ref name="pmid4793623">{{cite journal |vauthors=Wilkinson DS |title=Necrolytic migratory erythema with carcinoma of the pancreas |journal=Trans St Johns Hosp Dermatol Soc |volume=59 |issue=2 |pages=244–50 |year=1973 |pmid=4793623 |doi= |url=}}</ref><ref name="pmid8606627">{{cite journal |vauthors=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV |title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients |journal=Medicine (Baltimore) |volume=75 |issue=2 |pages=53–63 |year=1996 |pmid=8606627 |doi= |url=}}</ref><ref name="pmid15313692">{{cite journal |vauthors=Zhang M, Xu X, Shen Y, Hu ZH, Wu LM, Zheng SS |title=Clinical experience in diagnosis and treatment of glucagonoma syndrome |journal=HBPD INT |volume=3 |issue=3 |pages=473–5 |year=2004 |pmid=15313692 |doi= |url=}}</ref><ref name="pmid17873310">{{cite journal |vauthors=Kindmark H, Sundin A, Granberg D, Dunder K, Skogseid B, Janson ET, Welin S, Oberg K, Eriksson B |title=Endocrine pancreatic tumors with glucagon hypersecretion: a retrospective study of 23 cases during 20 years |journal=Med. Oncol. |volume=24 |issue=3 |pages=330–7 |year=2007 |pmid=17873310 |doi= |url=}}</ref>
|A family history of [[multiple endocrine neoplasia type 1]]
|
* [[Necrolytic migratory erythema]] characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure
*Skin lesions involving the lower [[abdomen]], [[Buttock|buttocks]], [[perineum]], and [[groin]]
* [[Weight loss]]
* [[Glucose intolerance]]
|
* [[Rash|Rash:]] Erythematous, ring shaped [[rash]] that blisters, erodes, and crusts over suggesting [[necrolytic migratory erythema]]
* Muscle [[atrophy]]
* Unilateral [[calf]] or [[thigh]] [[erythema]] and [[swelling]]
* Hyporeflexia
* Unilateral/bilateral [[sensory loss]] in the upper/lower extremity
|
* '''Serum glucagon'''
** Increased plasma glucagon levels (>500 pg/mL)
** Concentrations above 1000 pg/mL are diagnostic of glucagonoma

* CT scan is used to determine:
**The location of the tumor
**Metastasis (usually liver metastasis)
***Appear isodense with the liver on a non-contrasted study
|[[File:NEM1.jpg|center|250px]]
|-
|[[Pemphigus foliaceus]].<ref name="pmid15993235">{{cite journal| author=Bystryn JC, Rudolph JL| title=Pemphigus. | journal=Lancet | year= 2005 | volume= 366 | issue= 9479 | pages= 61-73 | pmid=15993235 | doi=10.1016/S0140-6736(05)66829-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15993235 }}</ref>[[Pemphigus foliaceus#cite note-Bolognia-2|[2]]]<ref name="pmid159414332">{{cite journal| author=Chams-Davatchi C, Valikhani M, Daneshpazhooh M, Esmaili N, Balighi K, Hallaji Z et al.| title=Pemphigus: analysis of 1209 cases. | journal=Int J Dermatol | year= 2005 | volume= 44 | issue= 6 | pages= 470-6 | pmid=15941433 | doi=10.1111/j.1365-4632.2004.02501.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15941433 }}</ref><ref name="pmid21353333">{{cite journal| author=Martin LK, Werth VP, Villaneuva EV, Murrell DF| title=A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. | journal=J Am Acad Dermatol | year= 2011 | volume= 64 | issue= 5 | pages= 903-8 | pmid=21353333 | doi=10.1016/j.jaad.2010.04.039 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21353333 }}</ref>
|Autoimmune blistering disease of the skin with characteristic lesions that are scaly, crusted erosions, often on an erythematous base.[[Pemphigus foliaceus#cite note-Fitz2-1|[1]]]

Mucosal involvement is absent even with widespread disease

|
* Cutaneous lesion that usually develops in a seborrheic distribution
* The scalp, face, and trunk are common sites of involvement

* Skin lesions may remain localized or may coalesce to cover large areas of skin
* Pain or burning sensations frequently accompany the cutaneous lesions
* Systemic symptoms are usually absent
|
* The skin lesions usually consist of small, scattered superficial blisters
** Lesions rapidly evolve into scaly, crusted erosions
** Positive Nikolsky sign

* Occasionally, pemphigus foliaceus progresses to involve the entire skin surface as an exfoliative erythroderma
|Autoimmune [[IgG]] build up in the [[Epidermis (skin)|epidermis]], then nearly almost all of the antibodies are aimed against [[desmoglein 1]]
|[[File:Pemphigus foliaceus08.jpg|center|250px|thumb|Courtesy:http://www.atlasdermatologico.com.br/index.jsf]]
|-
|[[Psoriasis|Pustular psoriasis]][[Psoriasis history and symptoms#cite note-pmid1390163-2|[2]]][[Psoriasis history and symptoms#cite note-pmid24790463-3|[3]]]
|
* Positive family history of psoriasis
*Frequent association with [[histocompatibility]] [[antigen]] (HLA)- Cw6
* A history of a long-term [[erythematous]] scaly area with [[ocular]] and [[joint]] involvement
* Past medical history of the patient may include
**[[Viral]] or [[bacterial]] infection
** [[Diabetes mellitus|diabetes]]
** [[Hypertension]]
** [[Chronic kidney disease]]
**[[Obesity]].
|
* [[Pain]](unpleasant, superficial, sensitive, itchy, hot or burning)
* [[Pruritus]]
* High [[fever]]
* Dystrophic nails
* Recent presentation of [[arthralgia]]
|
* [[Papulosquamous disorder|Papulosquamous]] [[disease]] with variable morphology, distribution, severity, and course
* Scaling [[Papule|papules]] and [[Plaque|plaques]]
* [[Koebner phenomenon]]: appearance of new psoriatic lesions at the site of skin injury
* [[Woronoff|Woronoff’s ring]]: ring of peripheral blanching skin around a psoriatic [[plaque]]
* Auspitz’s sign:
** Small [[bleeding]] points are seen upon disruption of a psoriatic scale
|
* '''Skin biopsy'''
**Perivascular and [[dermal]] [[Inflammatory cells|inflammatory cell]] infiltration
**[[Vascular]] dilation
**Absent [[granular layer]]
**Elongation of [[dermal]] [[Papilla|papillae]]
**Parakeratosis
**Spongiform [[pustules]] of Kogoj (pathognomic of psoriasis)
**Munro's micro abscesses (pathognomic of psoriasis)
**[[Edema]] of [[dermal]] [[papillae]]
**[[Basal cell layer]] is expanded
* Leukocytosis
|[[File:Pus.png|center|250px|thumb|Courtesy:http://www.atlasdermatologico.com.br/index.jsf]]
|-
|[[Acrodermatitis enteropathica]]<ref name="pmid6696358">{{cite journal| author=Prasad AS, Cossack ZT| title=Zinc supplementation and growth in sickle cell disease. | journal=Ann Intern Med | year= 1984 | volume= 100 | issue= 3 | pages= 367-71 | pmid=6696358 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6696358 }}</ref><ref name="pmid1940572">{{cite journal| author=Meftah S, Prasad AS, Lee DY, Brewer GJ| title=Ecto 5' nucleotidase (5'NT) as a sensitive indicator of human zinc deficiency. | journal=J Lab Clin Med | year= 1991 | volume= 118 | issue= 4 | pages= 309-16 | pmid=1940572 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1940572 }}</ref><ref name="pmid9481631">{{cite journal| author=Kiliç I, Ozalp I, Coŝkun T, Tokatli A, Emre S, Saldamli I et al.| title=The effect of zinc-supplemented bread consumption on school children with asymptomatic zinc deficiency. | journal=J Pediatr Gastroenterol Nutr | year= 1998 | volume= 26 | issue= 2 | pages= 167-71 | pmid=9481631 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9481631 }}</ref>
|
* An [[autosomal]] [[recessive]] disorder characterized by:
**Periorificial and acral [[dermatitis]], [[alopecia]], and [[diarrhea]]
* The genetic base is a [[mutation]] of [[SLC39A4]] which encodes a [[transmembrane protein]] that serves as a zinc uptake protein
|
* Symptoms appear in infants after breast milk weaning
* The appearance of erythematous patches and [[plaques]] of dry, [[Scal|scaly skin]]
* [[Diarrhea]]
|
* [[Erythematous]] [[Patch|patches]/plaques of dry and [[Scale|scaly]] skin

* The lesions may appear [[Eczematous Scaling|eczematous]] or may evolve into [[Crust|crusted]] [[vesicles]], [[Bulla|bullae]] or [[pustules]]
* The lesions are frequent in:
**Mouth/ perioral
** [[Anus]]/ Peri-anal
**Also involves hands, feet, and [[scalp]]
* [[Paronychia]]
* [[Alopecia]] of the scalp, eyebrows, and eyelashes
|
* Measurement of [[zinc]] in plasma, [[erythrocytes]], [[Neutrophil|neutrophils]], [[lymphocytes]], and hair

* A low plasma [[zinc]] usually is defined as a value less than 60 mcg/dL
* The criteria for [[zinc]] deficiency are decreased [[zinc]] level in [[lymphocyte]]
* Depressed serum [[alkaline phosphatase]] levels
|[[File:Acrodermatitis enteropathica 05.png|center|250px|thumb|Courtesy:http://www.atlasdermatologico.com.br/index.jsf]]
|-
|[[Pellagra]]<ref name="pmid12777163">{{cite journal| author=Prousky JE| title=Pellagra may be a rare secondary complication of anorexia nervosa: a systematic review of the literature. | journal=Altern Med Rev | year= 2003 | volume= 8 | issue= 2 | pages= 180-5 | pmid=12777163 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12777163 }}</ref><ref name="pmid21128910">{{cite journal| author=Wan P, Moat S, Anstey A| title=Pellagra: a review with emphasis on photosensitivity. | journal=Br J Dermatol | year= 2011 | volume= 164 | issue= 6 | pages= 1188-200 | pmid=21128910 | doi=10.1111/j.1365-2133.2010.10163.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21128910 }}</ref>
|
* It is a niacin deficiency disease characterized by
**[[Photosensitivity|photosensitive]]
**Pigmented [[dermatitis]]
** [[Diarrhea]]
** [[Dementia]].
* Hisotry of:
**[[Alcoholic|alcoholics]]
** [[Bariatric surgery]]
** [[Anorexia nervosa]]
** [[Malabsorption|Malabsorptive disease]]
* Dietary deficiency especially in infants
* Past history of [[Carcinoid syndrome]]
* Prolonged use of [[isoniazide]]
* A family history of [[Hartnup disease]]
|
* [[Photosensitivity]]
* [[Pigmented dermatitis]] in [[sun-exposed areas]]
* [[Diarrhea]]
* [[Dementia]]
|Symmetric hyper pigmented [[rash]], similar in color and distribution to a sunburn, which is present in the exposed areas of skin
|[[Niacin]] status can be assessed by measuring urinary [[N-methylnicotinamide]] or by measuring the erythrocyte [[NAD|NAD/]][[NADP]] ratio
|[[File:Pellagra24.jpg|center|250px|thumb|Courtesy:http://www.atlasdermatologico.com.br/index.jsf]]
|-
|[[eczema|Chronic eczema]] ([[atopic dermatitis]])
|
* Chronic pruritic inflammatory skin disease
*Occurs most frequently in children but also affects adults
* Family history of:
** [[Atopy]]
**([[Eczema]]
**[[Asthma]]
**[[Allergic rhinitis]])
* History of [[dermatitis]] involving the skin creases
* Personal or family history of [[asthma]] or [[hay fever]]
|
* Symptoms beginning in a child before the age of 2 years or, in children <4 years
* Dermatitis affecting the cheeks or dorsal aspect of extremities
* Dry skin and severe [[pruritus]] that is associated with cutaneous [[hyperreactivity]] to various environmental stimuli
* Exposure to:
**Food and inhalant allergens
**Irritants
**Infection
|
* Visible [[dermatitis]] involving flexural surfaces
* Presence of generally dry skin within the past year
* [[Erythema]], [[papulation]], oozing and [[crusting]], [[excoriation]]
|
* Raised [[IgE]] or an [[eosinophilia]]
* [[RAST test|Radioallergosorbent Test]]:
**Blood is mixed separately with many different allergens and the antibody levels measured
* [[Skin biopsy]]:
** A procedure that removes a small piece of the affected skin and sent for [[microscopic examination]] in a pathology laboratory
|[[File:Atopic Dermatitis27.jpg|center|250px|thumb|Courtesy:http://www.atlasdermatologico.com.br/index.jsf]]
|}

== Differentiating glucagonoma from other causes of hyperglycemia:==
Glucagonoma can be differentiated from other causes of hyperglycemia which include:<ref name="pmid17727381">{{cite journal| author=Barrett TG| title=Differential diagnosis of type 1 diabetes: which genetic syndromes need to be considered? | journal=Pediatr Diabetes | year= 2007 | volume= 8 Suppl 6 | issue= | pages= 15-23 | pmid=17727381 | doi=10.1111/j.1399-5448.2007.00278.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17727381 }}</ref><ref>Type 1 Diabetes mellitus "Dennis Kasper, Anthony Fauci, Stephen Hauser, Dan Longo, J. Larry Jameson, Joseph Loscalzo"Harrison's Principles of Internal Medicine, 19e Accessed on December 27th,2016</ref><ref>{{Cite web|url=http://www.namrata.co/diabetes-mellitus-differential-diagnosis-and-management/|title=namrata|last=|first=|date=|website=|publisher=|access-date=}}</ref>
* [[DM1|Type1 DM]]

* [[Type 2 diabetes mellitus|Type 2 DM]]
* [[Maturity onset diabetes of the young|MODY-DM]]
* [[Psychogenic polydipsia]]
* [[Diabetes insipidus]]
* Transient hyperglycemia
* Steroid therapy
* [[Renal tubular acidosis|Renal tubular acidosis type-1]]
* [[Glucagonoma]]
* [[Cushing's syndrome]]
* [[Hypothyroidism]]
* [[Wolfram syndrome]]
* [[Alstrom syndrome]]

{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Disease
! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" |History and symptoms
! colspan="8" align="center" style="background:#4479BA; color: #FFFFFF;" |Laboratory findings
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Additional findings
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" |Polyuria
! align="center" style="background:#4479BA; color: #FFFFFF;" |Polydipsia
! align="center" style="background:#4479BA; color: #FFFFFF;" |Polyphagia
! align="center" style="background:#4479BA; color: #FFFFFF;" |Weight loss
! align="center" style="background:#4479BA; color: #FFFFFF;" |Weight gain
! align="center" style="background:#4479BA; color: #FFFFFF;" |Serum glucose
! align="center" style="background:#4479BA; color: #FFFFFF;" |Urinary Glucose
! align="center" style="background:#4479BA; color: #FFFFFF;" |Urine PH
! align="center" style="background:#4479BA; color: #FFFFFF;" |Serum Sodium
! align="center" style="background:#4479BA; color: #FFFFFF;" |Urinary Glucose
! align="center" style="background:#4479BA; color: #FFFFFF;" |24 hrs cortisol level
! align="center" style="background:#4479BA; color: #FFFFFF;" |C-peptide level
! align="center" style="background:#4479BA; color: #FFFFFF;" |Serum glucagon
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Diabetes mellitus type 1|Type 1 Diabetes mellitus]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''''↑'''''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↓'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Auto antibodies present ([[GAD65|Anti GAD-65]] and anti insulin anti bodies)
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Diabetes mellitus type 2|Type 2 Diabetes mellitus]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Acanthosis nigricans]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[MODY]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Psychogenic polydipsia]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↓'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Diabetes insipidus]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |Transient [[hyperglycemia]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |In hospitalized patients especially in [[ICU]] and [[CCU]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Steroid]] therapy
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Acanthosis nigricans|Acanthosis nigricans,]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[RTA|RTA 1]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Hypokalemia]], [[nephrolithiasis]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Glucagonoma]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Necrolytic migratory erythema]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Cushing's syndrome|Cushing syndrome]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↓'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Moon face]], [[obesity]], [[buffalo hump]], easy [[Bruising|bruisibility]]
|}

==References==
{{reflist|2}}
{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma differential diagnosis

2017-11-17T14:21:02Z

Anthony Gallo: /* Differentiating glucagonoma from other causes of hyperglycemia: */

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}} {{MAD}}
==Overview==
Glucagonoma must be differentiated from certain skin lesions in which necrolytic migratory erythema can be found such as [[acrodermatitis enteropathica]], [[psoriasis]], [[pellagra]], and [[eczema]]. Glucagonoma should be differentiated from other causes of hyperglycemia include [[infection]], [[diabetes mellitus]], [[Cushing syndrome]], [[renal failure]], [[acute pancreatitis]], severe stress, and prolonged fasting.

==Differentiating Glucagonoma from other Diseases==
Glucagonoma must be differentiated from certain skin lesions in which necrolytic migratory erythema can be found such as:<ref name="pmid25152626">{{cite journal| author=Fang S, Li S, Cai T| title=Glucagonoma syndrome: a case report with focus on skin disorders. | journal=Onco Targets Ther | year= 2014 | volume= 7 | issue= | pages= 1449-53 | pmid=25152626 | doi=10.2147/OTT.S66285 | pmc=PMC4140234 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25152626 }} </ref>
* [[Pemphigus foliaceus]]
* [[Pustular psoriasis]]
* [[Atopic dermatitis|Chronic eczema]]
* [[Acrodermatitis enteropathica]]
* [[Pellagra]]

{| class="wikitable"
! rowspan="2" |Disease
! colspan="3" |Clinical Picture
! rowspan="2" |Investigations
! rowspan="2" |Pictures
|-
!History
!Symptoms
!Signs
|-
|Glucagonoma<ref name="pmid4793623">{{cite journal |vauthors=Wilkinson DS |title=Necrolytic migratory erythema with carcinoma of the pancreas |journal=Trans St Johns Hosp Dermatol Soc |volume=59 |issue=2 |pages=244–50 |year=1973 |pmid=4793623 |doi= |url=}}</ref><ref name="pmid8606627">{{cite journal |vauthors=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV |title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients |journal=Medicine (Baltimore) |volume=75 |issue=2 |pages=53–63 |year=1996 |pmid=8606627 |doi= |url=}}</ref><ref name="pmid15313692">{{cite journal |vauthors=Zhang M, Xu X, Shen Y, Hu ZH, Wu LM, Zheng SS |title=Clinical experience in diagnosis and treatment of glucagonoma syndrome |journal=HBPD INT |volume=3 |issue=3 |pages=473–5 |year=2004 |pmid=15313692 |doi= |url=}}</ref><ref name="pmid17873310">{{cite journal |vauthors=Kindmark H, Sundin A, Granberg D, Dunder K, Skogseid B, Janson ET, Welin S, Oberg K, Eriksson B |title=Endocrine pancreatic tumors with glucagon hypersecretion: a retrospective study of 23 cases during 20 years |journal=Med. Oncol. |volume=24 |issue=3 |pages=330–7 |year=2007 |pmid=17873310 |doi= |url=}}</ref>
|A family history of [[multiple endocrine neoplasia type 1]]
|
* [[Necrolytic migratory erythema]] characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure
*Skin lesions involving the lower [[abdomen]], [[Buttock|buttocks]], [[perineum]], and [[groin]]
* [[Weight loss]]
* [[Glucose intolerance]]
|
* [[Rash|Rash:]] Erythematous, ring shaped [[rash]] that blisters, erodes, and crusts over suggesting [[necrolytic migratory erythema]]
* Muscle [[atrophy]]
* Unilateral [[calf]] or [[thigh]] [[erythema]] and [[swelling]]
* Hyporeflexia
* Unilateral/bilateral [[sensory loss]] in the upper/lower extremity
|
* '''Serum glucagon'''
** Increased plasma glucagon levels (>500 pg/mL)
** Concentrations above 1000 pg/mL are diagnostic of glucagonoma

* CT scan is used to determine:
**The location of the tumor
**Metastasis (usually liver metastasis)
***Appear isodense with the liver on a non-contrasted study
|[[File:NEM1.jpg|center|250px]]
|-
|[[Pemphigus foliaceus]].<ref name="pmid15993235">{{cite journal| author=Bystryn JC, Rudolph JL| title=Pemphigus. | journal=Lancet | year= 2005 | volume= 366 | issue= 9479 | pages= 61-73 | pmid=15993235 | doi=10.1016/S0140-6736(05)66829-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15993235 }}</ref>[[Pemphigus foliaceus#cite note-Bolognia-2|[2]]]<ref name="pmid159414332">{{cite journal| author=Chams-Davatchi C, Valikhani M, Daneshpazhooh M, Esmaili N, Balighi K, Hallaji Z et al.| title=Pemphigus: analysis of 1209 cases. | journal=Int J Dermatol | year= 2005 | volume= 44 | issue= 6 | pages= 470-6 | pmid=15941433 | doi=10.1111/j.1365-4632.2004.02501.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15941433 }}</ref><ref name="pmid21353333">{{cite journal| author=Martin LK, Werth VP, Villaneuva EV, Murrell DF| title=A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. | journal=J Am Acad Dermatol | year= 2011 | volume= 64 | issue= 5 | pages= 903-8 | pmid=21353333 | doi=10.1016/j.jaad.2010.04.039 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21353333 }}</ref>
|Autoimmune blistering disease of the skin with characteristic lesions that are scaly, crusted erosions, often on an erythematous base.[[Pemphigus foliaceus#cite note-Fitz2-1|[1]]]

Mucosal involvement is absent even with widespread disease

|
* Cutaneous lesion that usually develops in a seborrheic distribution
* The scalp, face, and trunk are common sites of involvement

* Skin lesions may remain localized or may coalesce to cover large areas of skin
* Pain or burning sensations frequently accompany the cutaneous lesions
* Systemic symptoms are usually absent
|
* The skin lesions usually consist of small, scattered superficial blisters
** Lesions rapidly evolve into scaly, crusted erosions
** Positive Nikolsky sign

* Occasionally, pemphigus foliaceus progresses to involve the entire skin surface as an exfoliative erythroderma
|Autoimmune [[IgG]] build up in the [[Epidermis (skin)|epidermis]], then nearly almost all of the antibodies are aimed against [[desmoglein 1]]
|[[File:Pemphigus foliaceus08.jpg|center|250px|thumb|Courtesy:http://www.atlasdermatologico.com.br/index.jsf]]
|-
|[[Psoriasis|Pustular psoriasis]][[Psoriasis history and symptoms#cite note-pmid1390163-2|[2]]][[Psoriasis history and symptoms#cite note-pmid24790463-3|[3]]]
|
* Positive family history of psoriasis
*Frequent association with [[histocompatibility]] [[antigen]] (HLA)- Cw6
* A history of a long-term [[erythematous]] scaly area with [[ocular]] and [[joint]] involvement
* Past medical history of the patient may include
**[[Viral]] or [[bacterial]] infection
** [[Diabetes mellitus|diabetes]]
** [[Hypertension]]
** [[Chronic kidney disease]]
**[[Obesity]].
|
* [[Pain]](unpleasant, superficial, sensitive, itchy, hot or burning)
* [[Pruritus]]
* High [[fever]]
* Dystrophic nails
* Recent presentation of [[arthralgia]]
|
* [[Papulosquamous disorder|Papulosquamous]] [[disease]] with variable morphology, distribution, severity, and course
* Scaling [[Papule|papules]] and [[Plaque|plaques]]
* [[Koebner phenomenon]]: appearance of new psoriatic lesions at the site of skin injury
* [[Woronoff|Woronoff’s ring]]: ring of peripheral blanching skin around a psoriatic [[plaque]]
* Auspitz’s sign:
** Small [[bleeding]] points are seen upon disruption of a psoriatic scale
|
* '''Skin biopsy'''
**Perivascular and [[dermal]] [[Inflammatory cells|inflammatory cell]] infiltration
**[[Vascular]] dilation
**Absent [[granular layer]]
**Elongation of [[dermal]] [[Papilla|papillae]]
**Parakeratosis
**Spongiform [[pustules]] of Kogoj (pathognomic of psoriasis)
**Munro's micro abscesses (pathognomic of psoriasis)
**[[Edema]] of [[dermal]] [[papillae]]
**[[Basal cell layer]] is expanded
* Leukocytosis
|[[File:Pus.png|center|250px|thumb|Courtesy:http://www.atlasdermatologico.com.br/index.jsf]]
|-
|[[Acrodermatitis enteropathica]]<ref name="pmid6696358">{{cite journal| author=Prasad AS, Cossack ZT| title=Zinc supplementation and growth in sickle cell disease. | journal=Ann Intern Med | year= 1984 | volume= 100 | issue= 3 | pages= 367-71 | pmid=6696358 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6696358 }}</ref><ref name="pmid1940572">{{cite journal| author=Meftah S, Prasad AS, Lee DY, Brewer GJ| title=Ecto 5' nucleotidase (5'NT) as a sensitive indicator of human zinc deficiency. | journal=J Lab Clin Med | year= 1991 | volume= 118 | issue= 4 | pages= 309-16 | pmid=1940572 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1940572 }}</ref><ref name="pmid9481631">{{cite journal| author=Kiliç I, Ozalp I, Coŝkun T, Tokatli A, Emre S, Saldamli I et al.| title=The effect of zinc-supplemented bread consumption on school children with asymptomatic zinc deficiency. | journal=J Pediatr Gastroenterol Nutr | year= 1998 | volume= 26 | issue= 2 | pages= 167-71 | pmid=9481631 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9481631 }}</ref>
|
* An [[autosomal]] [[recessive]] disorder characterized by:
**Periorificial and acral [[dermatitis]], [[alopecia]], and [[diarrhea]]
* The genetic base is a [[mutation]] of [[SLC39A4]] which encodes a [[transmembrane protein]] that serves as a zinc uptake protein
|
* Symptoms appear in infants after breast milk weaning
* The appearance of erythematous patches and [[plaques]] of dry, [[Scal|scaly skin]]
* [[Diarrhea]]
|
* [[Erythematous]] [[Patch|patches]/plaques of dry and [[Scale|scaly]] skin

* The lesions may appear [[Eczematous Scaling|eczematous]] or may evolve into [[Crust|crusted]] [[vesicles]], [[Bulla|bullae]] or [[pustules]]
* The lesions are frequent in:
**Mouth/ perioral
** [[Anus]]/ Peri-anal
**Also involves hands, feet, and [[scalp]]
* [[Paronychia]]
* [[Alopecia]] of the scalp, eyebrows, and eyelashes
|
* Measurement of [[zinc]] in plasma, [[erythrocytes]], [[Neutrophil|neutrophils]], [[lymphocytes]], and hair

* A low plasma [[zinc]] usually is defined as a value less than 60 mcg/dL
* The criteria for [[zinc]] deficiency are decreased [[zinc]] level in [[lymphocyte]]
* Depressed serum [[alkaline phosphatase]] levels
|[[File:Acrodermatitis enteropathica 05.png|center|250px|thumb|Courtesy:http://www.atlasdermatologico.com.br/index.jsf]]
|-
|[[Pellagra]]<ref name="pmid12777163">{{cite journal| author=Prousky JE| title=Pellagra may be a rare secondary complication of anorexia nervosa: a systematic review of the literature. | journal=Altern Med Rev | year= 2003 | volume= 8 | issue= 2 | pages= 180-5 | pmid=12777163 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12777163 }}</ref><ref name="pmid21128910">{{cite journal| author=Wan P, Moat S, Anstey A| title=Pellagra: a review with emphasis on photosensitivity. | journal=Br J Dermatol | year= 2011 | volume= 164 | issue= 6 | pages= 1188-200 | pmid=21128910 | doi=10.1111/j.1365-2133.2010.10163.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21128910 }}</ref>
|
* It is a niacin deficiency disease characterized by
**[[Photosensitivity|photosensitive]]
**Pigmented [[dermatitis]]
** [[Diarrhea]]
** [[Dementia]].
* Hisotry of:
**[[Alcoholic|alcoholics]]
** [[Bariatric surgery]]
** [[Anorexia nervosa]]
** [[Malabsorption|Malabsorptive disease]]
* Dietary deficiency especially in infants
* Past history of [[Carcinoid syndrome]]
* Prolonged use of [[isoniazide]]
* A family history of [[Hartnup disease]]
|
* [[Photosensitivity]]
* [[Pigmented dermatitis]] in [[sun-exposed areas]]
* [[Diarrhea]]
* [[Dementia]]
|Symmetric hyper pigmented [[rash]], similar in color and distribution to a sunburn, which is present in the exposed areas of skin
|[[Niacin]] status can be assessed by measuring urinary [[N-methylnicotinamide]] or by measuring the erythrocyte [[NAD|NAD/]][[NADP]] ratio
|[[File:Pellagra24.jpg|center|250px|thumb|Courtesy:http://www.atlasdermatologico.com.br/index.jsf]]
|-
|[[eczema|Chronic eczema]] ([[atopic dermatitis]])
|
* Chronic pruritic inflammatory skin disease
*Occurs most frequently in children but also affects adults
* Family history of:
** [[Atopy]]
**([[Eczema]]
**[[Asthma]]
**[[Allergic rhinitis]])
* History of [[dermatitis]] involving the skin creases
* Personal or family history of [[asthma]] or [[hay fever]]
|
* Symptoms beginning in a child before the age of 2 years or, in children <4 years
* Dermatitis affecting the cheeks or dorsal aspect of extremities
* Dry skin and severe [[pruritus]] that is associated with cutaneous [[hyperreactivity]] to various environmental stimuli
* Exposure to:
**Food and inhalant allergens
**Irritants
**Infection
|
* Visible [[dermatitis]] involving flexural surfaces
* Presence of generally dry skin within the past year
* [[Erythema]], [[papulation]], oozing and [[crusting]], [[excoriation]]
|
* Raised [[IgE]] or an [[eosinophilia]]
* [[RAST test|Radioallergosorbent Test]]:
**Blood is mixed separately with many different allergens and the antibody levels measured
* [[Skin biopsy]]:
** A procedure that removes a small piece of the affected skin and sent for [[microscopic examination]] in a pathology laboratory
|[[File:Atopic Dermatitis27.jpg|center|250px|thumb|Courtesy:http://www.atlasdermatologico.com.br/index.jsf]]
|}

== Differentiating glucagonoma from other causes of hyperglycemia:==
Glucagonoma can be differentiated from other causes of hyperglycemia which include:<ref name="pmid17727381">{{cite journal| author=Barrett TG| title=Differential diagnosis of type 1 diabetes: which genetic syndromes need to be considered? | journal=Pediatr Diabetes | year= 2007 | volume= 8 Suppl 6 | issue= | pages= 15-23 | pmid=17727381 | doi=10.1111/j.1399-5448.2007.00278.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17727381 }}</ref><ref>Type 1 Diabetes mellitus "Dennis Kasper, Anthony Fauci, Stephen Hauser, Dan Longo, J. Larry Jameson, Joseph Loscalzo"Harrison's Principles of Internal Medicine, 19e Accessed on December 27th,2016</ref><ref>{{Cite web|url=http://www.namrata.co/diabetes-mellitus-differential-diagnosis-and-management/|title=namrata|last=|first=|date=|website=|publisher=|access-date=}}</ref>
* [[DM1|Type1 DM]]

* [[Type 2 diabetes mellitus|Type 2 DM]]
* [[Maturity onset diabetes of the young|MODY-DM]]
* [[Psychogenic polydipsia]]
* [[Diabetes insipidus]]
* [[Transient hyperglycemia]]
* Steroid therapy
* [[Renal tubular acidosis|Renal tubular acidosis type-1]]
* [[Glucagonoma]]
* [[Cushing's syndrome]]
* [[Hypothyroidism]]
* [[Wolfram syndrome]]
* [[Alstrom syndrome]]

{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Disease
! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" |History and symptoms
! colspan="8" align="center" style="background:#4479BA; color: #FFFFFF;" |Laboratory findings
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Additional findings
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" |Polyuria
! align="center" style="background:#4479BA; color: #FFFFFF;" |Polydipsia
! align="center" style="background:#4479BA; color: #FFFFFF;" |Polyphagia
! align="center" style="background:#4479BA; color: #FFFFFF;" |Weight loss
! align="center" style="background:#4479BA; color: #FFFFFF;" |Weight gain
! align="center" style="background:#4479BA; color: #FFFFFF;" |Serum glucose
! align="center" style="background:#4479BA; color: #FFFFFF;" |Urinary Glucose
! align="center" style="background:#4479BA; color: #FFFFFF;" |Urine PH
! align="center" style="background:#4479BA; color: #FFFFFF;" |Serum Sodium
! align="center" style="background:#4479BA; color: #FFFFFF;" |Urinary Glucose
! align="center" style="background:#4479BA; color: #FFFFFF;" |24 hrs cortisol level
! align="center" style="background:#4479BA; color: #FFFFFF;" |C-peptide level
! align="center" style="background:#4479BA; color: #FFFFFF;" |Serum glucagon
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Diabetes mellitus type 1|Type 1 Diabetes mellitus]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''''↑'''''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↓'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Auto antibodies present ([[GAD65|Anti GAD-65]] and anti insulin anti bodies)
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Diabetes mellitus type 2|Type 2 Diabetes mellitus]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Acanthosis nigricans]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[MODY]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Psychogenic polydipsia]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↓'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Diabetes insipidus]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |Transient [[hyperglycemia]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |In hospitalized patients especially in [[ICU]] and [[CCU]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Steroid]] therapy
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Acanthosis nigricans|Acanthosis nigricans,]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[RTA|RTA 1]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Hypokalemia]], [[nephrolithiasis]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Glucagonoma]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Necrolytic migratory erythema]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Cushing's syndrome|Cushing syndrome]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |✔
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↓'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |'''↑'''
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Normal
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Moon face]], [[obesity]], [[buffalo hump]], easy [[Bruising|bruisibility]]
|}

==References==
{{reflist|2}}
{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma pathophysiology

2017-11-17T14:00:03Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}} {{MAD}}

==Overview==
''Glucagonoma'' is a tumor of the [[alpha cells]] of the [[pancreas]] characterized by the excessive secretion of [[glucagon]] and [[necrolytic migratory erythema]]. Glucagonoma causes hyperglucagonemia, [[zinc deficiency]], [[fatty acid]] deficiency, [[Aminoacid|hypoaminoacidemia]] that may cause [[necrolytic migratory erythema]]. Glucagonoma may be a part of [[MEN1 syndrome|type 1 multiple endocrine neoplasia]]. It is an autosomal dominant syndrome that is usually caused by mutations in the [[MEN1 syndrome|''MEN1'' gene]]. [[MEN1 syndrome|''MEN1'' gene]] is a [[tumor suppressor gene]] and causes [[MEN1 syndrome|type 1 multiple endocrine neoplasia]] by [[Knudson hypothesis|Knudson's "two hits" model]] for [[tumor]] development. All glucagonomas are located in the pancreas, 50–80% occur in the pancreatic tail, 32.2% in the body and 21.9% in the head. Glucagonoma can metastasize mainly to the liver. Glucagonomas consist of [[Pleomorphism|pleomorphic]] cells containing granules that stain for other peptides, most frequently [[pancreatic polypeptide]]. [[Immunoperoxidase|Immunoperoxidase staining]] can detect glucagon within the tumor cells and [[Glucagon|glucagon.]]

==Pathogenesis==
* Glucagonoma is a rare tumor of the [[alpha cells]] of the [[pancreas]] that results in the overproduction of the hormone [[glucagon]]. Glucagonomas are [[neuroendocrine tumors]] derived from [[Stem cells|multipotential stem cells]].<ref name="pmid9113318">{{cite journal| author=Frankton S, Bloom SR| title=Gastrointestinal endocrine tumours. Glucagonomas. | journal=Baillieres Clin Gastroenterol | year= 1996 | volume= 10 | issue= 4 | pages= 697-705 | pmid=9113318 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9113318 }}</ref><ref name="pmid6127984">{{cite journal| author=Braverman IM| title="Cutaneous manifestations of internal malignant tumors" by Becker, Kahn and Rothman, June 1942. Commentary: Migratory necrolytic erythema. | journal=Arch Dermatol | year= 1982 | volume= 118 | issue= 10 | pages= 784-98 | pmid=6127984 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6127984 }}</ref><ref>Necrolytic migratory erythema. Wikipedia. https://en.wikipedia.org/wiki/Necrolytic_migratory_erythema. Accessed on October 13, 2015.</ref><ref name="pmid9591806">{{cite journal| author=Mullans EA, Cohen PR| title=Iatrogenic necrolytic migratory erythema: a case report and review of nonglucagonoma-associated necrolytic migratory erythema. | journal=J Am Acad Dermatol | year= 1998 | volume= 38 | issue= 5 Pt 2 | pages= 866-73 | pmid=9591806 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9591806 }} </ref><ref name="pmid13978995">{{cite journal| author=STURZBECHER M| title=[8 letters of Ferdinand von HEBRAS on his contributin to Virchow's Handbuch der Speziellen Pathologie and Therapie]. | journal=Z Haut Geschlechtskr | year= 1963 | volume= 34 | issue= | pages= 281-6 | pmid=13978995 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13978995 }}</ref><ref name="pmid14356022">{{cite journal| author=Wilson LA, Kuhn JA, Corbisiero RM, Smith M, Beatty JD, Williams LE et al.| title=A technical analysis of an intraoperative radiation detection probe. | journal=Med Phys | year= 1992 | volume= 19 | issue= 5 | pages= 1219-23 | pmid=1435602 | doi=10.1118/1.596754 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1435602 }}</ref>
** [[Glucagon]] increases [[glycogenolysis]], [[gluconeogenesis]] from amino acid substrates and inhibits [[glycolysis]]. This causes weight loss due to the [[Catabolism|catabolic]] action of [[glucagon]].
** When [[glucagon]] is secreted by a tumor, it becomes independent and is no longer influenced by feedback control mechanisms.
** Glucagonoma causes hyperglucagonemia, [[zinc deficiency]], [[fatty acid]] deficiency, [[Aminoacid|hypoaminoacidemia]] that may cause [[necrolytic migratory erythema]].
** The mechanism for [[necrolytic migratory erythema]] involves excessive inflammation in the epidermis in response to trauma and to the necrolysis.
** [[Necrolytic migratory erythema]] (NME) probably results from hyponutrition and [[amino acid]] deficiency. It can be caused by the loss of [[tryptophan]] in cutaneous tissues as a result of the excess circulating [[glucagon]]. [[Tryptophan]] is responsible for [[niacin]] function, which regulates cell turnover and the maturation of the epidermis and mucosal epithelia.
** [[Diarrhea]] may result from the secretion of [[gastrin]] occurs with glucagonoma.

== Genetics ==
Glucagonoma may be part of [[MEN1 syndrome|type 1 multiple endocrine neoplasia]]. It is an autosomal dominant syndrome that is usually caused by mutations in the [[MEN1 syndrome|''MEN1'' gene]].[[Multiple endocrine neoplasia type 1 pathophysiology#cite note-wikipedia-1|[1]]][[Multiple endocrine neoplasia type 1 pathophysiology#cite note-pmid17014705-2|[2]]][[Multiple endocrine neoplasia type 1 pathophysiology#cite note-pmid2894610-3|[3]]][[Multiple endocrine neoplasia type 1 pathophysiology#cite note-pmid2568587-4|[4]]][[Multiple endocrine neoplasia type 1 pathophysiology#cite note-pmid2568586-5|[5]]][[Multiple endocrine neoplasia type 1 pathophysiology#cite note-pmid1968641-6|[6]]][[Multiple endocrine neoplasia type 1 pathophysiology#cite note-pmid7902574-7|[7]]]
* It is characterized by the development of the following tumors:
** [[Pituitary adenoma|Pituitary adenomas]]
** [[Islet cell tumor|Islet cell tumors]] of the [[pancreas]] (commonly [[gastrinoma]] and glucagonoma)
**[[Parathyroid]] [[hyperplasia]] with resulting [[hyperparathyroidism]]
* The [[gene]] [[locus]] causing [[multiple endocrine neoplasia type 1]] has been localized to [[chromosome]] 11q13 by studies of [[loss of heterozygosity]] on [[multiple endocrine neoplasia type 1]]-associated [[Tumor|tumors]] and by linkage analysis in [[multiple endocrine neoplasia type 1]] families. ''MEN1'', spans about 10 Kb and consists of ten exons encoding a 610 [[amino acid]] nuclear protein, named menin.
* ''MEN1'' [[gene]] is a [[tumor suppressor gene]] and causes type 1 multiple endocrine neoplasia by Knudson's "two hits" model for [[tumor]] development.
* Two hits model for [[tumor]] development suggests that there is a [[germline mutation]] present in all [[Cell|cells]] at birth and the second [[mutation]] is a somatic [[mutation]] that occurs in the predisposed [[endocrine]] [[cell]] and leads to loss of the remaining wild type [[allele]]. This "two hits" model gives [[Cell|cells]] the survival advantage needed for [[tumor]] development.

== Gross Pathology ==
The gross pathology of glucagonoma may show:<ref name="pmid21859461">{{cite journal| author=Castro PG, de León AM, Trancón JG, Martínez PA, Alvarez Pérez JA, Fernández Fernández JC et al.| title=Glucagonoma syndrome: a case report. | journal=J Med Case Rep | year= 2011 | volume= 5 | issue= | pages= 402 | pmid=21859461 | doi=10.1186/1752-1947-5-402 | pmc=PMC3171381 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21859461 }} </ref><ref name="pmid9880781">{{cite journal| author=Soga J, Yakuwa Y| title=Glucagonomas/diabetico-dermatogenic syndrome (DDS): a statistical evaluation of 407 reported cases. | journal=J Hepatobiliary Pancreat Surg | year= 1998 | volume= 5 | issue= 3 | pages= 312-9 | pmid=9880781 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9880781 }}</ref><ref name="pmid25152626">{{cite journal| author=Fang S, Li S, Cai T| title=Glucagonoma syndrome: a case report with focus on skin disorders. | journal=Onco Targets Ther | year= 2014 | volume= 7 | issue= | pages= 1449-53 | pmid=25152626 | doi=10.2147/OTT.S66285 | pmc=PMC4140234 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25152626 }} </ref>

* Large tumors at diagnosis with a mean diameter of 5 cm, From 50 to 82% have evidence of [[Metastasis|metastatic]] spread at presentation.

* Nearly all glucagonomas are located in the pancreas, 50–80% occur in the pancreatic tail, 32.2% in the body and 21.9% in the head.
* In few patients, the location was [[extrapancreatic]], such as in [[Kidney|kidney,]] [[duodenum]], [[lung]], [[Accessory pancreas|accessory pancreatic tissue]].

* Metastasis usually occurs to [[Liver|the liver]]. Other sites are [[Lymph node|lymph nodes]], [[bone]], [[lung]], and [[Adrenal gland|adrenals]].

*Tumors smaller than 2 cm in diameter are associated with a very low chance of malignancy.

==Microscopic Pathology==
The microscopic pathology of glucagonoma tumors in pancreas usually show intense staining for [[glucagon]].<ref name="pmid6295622">{{cite journal| author=Warner TF, Block M, Hafez GR, Mack E, Lloyd RV, Bloom SR| title=Glucagonomas. Ultrastructure and immunocytochemistry. | journal=Cancer | year= 1983 | volume= 51 | issue= 6 | pages= 1091-6 | pmid=6295622 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6295622 }}</ref><ref name="pmid1973365">{{cite journal| author=Mozell E, Stenzel P, Woltering EA, Rösch J, O'Dorisio TM| title=Functional endocrine tumors of the pancreas: clinical presentation, diagnosis, and treatment. | journal=Curr Probl Surg | year= 1990 | volume= 27 | issue= 6 | pages= 301-86 | pmid=1973365 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1973365 }}</ref>
* Many glucagonomas are [[Pleomorphism|pleomorphic]] with cells containing granules that stain for other peptides, most frequently [[pancreatic polypeptide]].
* [[Immunoperoxidase|Immunoperoxidase staining]] can detect glucagon within the tumor cells and glucagon [[mRNA]] also may be detected.
* [[Electron|Electron microscopy]] shows secretory granules indicating the origin of glucagonoma from [[alpha cells]].
* Benign tumors are usually fully granulated and malignant cells have fewer granules.
*Skin biopsy may depict epidermal necrosis.

===Images===
<gallery>Image:800px-Confluent epidermal necrosis - high mag.jpg|'''Histology of confluent epidermal necrosis''' '''(high mag)''',Source:By Nephron - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=16874054<ref name=picture>Glucagonoma. Wikimedia Commons. https://commons.wikimedia.org/wiki/File:Confluent_epidermal_necrosis_-_high_mag.jpg</ref>

Image:1024px-Confluent epidermal necrosis - very high mag.jpg|'''Histology of confluent epidermal necrosis (very high mag)'''Source:By Nephron - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=16874054<ref name=picture>Glucagonoma. Wikimedia Commons. https://commons.wikimedia.org/wiki/File:Confluent_epidermal_necrosis_-_high_mag.jpg</ref>

Image:1024px-Confluent epidermal necrosis - intermed mag.jpg|'''Histology of confluent epidermal necrosis''' '''(intermed mag)'''Source:By Nephron - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=16874054<ref name=picture>Glucagonoma. Wikimedia Commons. https://commons.wikimedia.org/wiki/File:Confluent_epidermal_necrosis_-_high_mag.jpg</ref>

Image:800px-Confluent epidermal necrosis - low mag.jpg|'''Histology of confluent epidermal necrosis (low mag)'''Source:By Nephron - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=16874054<ref name=picture>Glucagonoma. Wikimedia Commons. https://commons.wikimedia.org/wiki/File:Confluent_epidermal_necrosis_-_high_mag.jpg</ref>
Image:1752-1947-5-402-1.jpg|(A) Skin lesions affecting pretibial area. (B) Skin biopsy in necrolytic migratory erythema showing a zone of necrolysis and vacuolated keratinocytes<ref name="pmid21859461">{{cite journal| author=Castro PG, de León AM, Trancón JG, Martínez PA, Alvarez Pérez JA, Fernández Fernández JC et al.| title=Glucagonoma syndrome: a case report. | journal=J Med Case Rep | year= 2011 | volume= 5 | issue= | pages= 402 | pmid=21859461 | doi=10.1186/1752-1947-5-402 | pmc=PMC3171381 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21859461 }} </ref>
Image:NEM111.jpg|Skin biopsy in necrolytic migratory erythema showing a large zone of necrolysis in the upper epidermis (arrow)<ref name="pmid25152626">{{cite journal| author=Fang S, Li S, Cai T| title=Glucagonoma syndrome: a case report with focus on skin disorders. | journal=Onco Targets Ther | year= 2014 | volume= 7 | issue= | pages= 1449-53 | pmid=25152626 | doi=10.2147/OTT.S66285 | pmc=PMC4140234 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25152626 }} </ref>

Image:Glucagonoma'.jpg|A) Psoriasiform hyperplasia of the epidermis with overlying parakeratosis and mild perivascular infiltrate of lymphocytes in the upper dermis (HE 5 X). B) Vascular dilatation (HE 20 X).<ref name="pmid23259638">{{cite journal| author=Erdas E, Aste N, Pilloni L, Nicolosi A, Licheri S, Cappai A et al.| title=Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? | journal=BMC Cancer | year= 2012 | volume= 12 | issue= | pages= 614 | pmid=23259638 | doi=10.1186/1471-2407-12-614 | pmc=PMC3543729 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23259638 }} </ref>
Image:Gross1.jpg|Specimen from distal splenopancreatectomy.A) The neoplasia is located in the inferior border of the pancreas (arrow); it shows an exophytic growth but appears well circumscribed. B) The cut surface is whitish-yellow in color with focal areas of hemorrhage.<ref name="pmid23259638">{{cite journal| author=Erdas E, Aste N, Pilloni L, Nicolosi A, Licheri S, Cappai A et al.| title=Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? | journal=BMC Cancer | year= 2012 | volume= 12 | issue= | pages= 614 | pmid=23259638 | doi=10.1186/1471-2407-12-614 | pmc=PMC3543729 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23259638 }} </ref>
Image:Histo.jpg|Histopathological examination of the pancreatic tumor.A) The tumor appears encapsulated and composed of polygonal cells with trabecular or ribbon-like proliferation (HE 5 X). B) At immunohistochemistry, neoplastic cells showed an intense diffuse staining for glucagon (Anti-glucagon antibody 5 X)<ref name="pmid23259638">{{cite journal| author=Erdas E, Aste N, Pilloni L, Nicolosi A, Licheri S, Cappai A et al.| title=Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? | journal=BMC Cancer | year= 2012 | volume= 12 | issue= | pages= 614 | pmid=23259638 | doi=10.1186/1471-2407-12-614 | pmc=PMC3543729 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23259638 }} </ref>

</gallery>

==References==
{{reflist|2}}

{{WH}}
{{WS}}
[[Category:Medicine]]
[[Category:Endocrinology]]

Glucagonoma overview

2017-11-17T13:54:42Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}

==Overview==
A glucagonoma is a [[tumor]] of the [[alpha cell]]s of the [[pancreas]] that results in overproduction of the [[hormone]] [[glucagon]]. Glucagonoma was first described in 1942 by Becker. On microscopic examination, glucagonoma's consist of [[Pleomorphism|pleomorphic]] cells containing granules that stain for other peptides, most frequently [[pancreatic polypeptide]]. [[Immunoperoxidase|Immunoperoxidase staining]] can detect glucagon within the tumor cells and [[Glucagon|glucagon.]] Similar symptoms are present in cases of pseudo-glucagonoma syndrome in the absence of a glucagon-secreting tumor. Further, glucagonoma must be differentiated from certain skin lesions ([[acrodermatitis enteropathica]], [[psoriasis]], [[pellagra]], [[eczema]]) and other causes of hyperglucagonemia ([[infection]], [[diabetes mellitus]], [[Cushing syndrome]], [[renal failure]], [[acute pancreatitis]], severe stress, and prolonged fasting). The incidence of glucagonoma is approximately .0005 per 100,000 individuals worldwide. Glucagonoma affects men and women equally. The median age at diagnosis of glucagonoma is 52.5 years. The most potent risk factor in the development of glucagonoma is a positive family history of [[multiple endocrine neoplasia type 1]]. If left untreated, patients with glucagonoma may progress to develop [[necrolytic migratory erythema]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]].The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma. The 10-year event-free survival rate is less than 51.6% with metastasis and 64.3% without metastasis. According to The American Joint Committee on Cancer (AJCC), there are four stages of glucagonoma based on the [[TNM staging system]]. Symptoms of glucagonoma include [[necrolytic migratory erythema]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]]. A positive family history of [[multiple endocrine neoplasia type 1]] may be present. Common physical examination findings of glucagonoma include [[tachycardia]], [[fever]], [[rash]], [[muscle atrophy]], cotton wool spots, flame hemorrhage, and dot and blot hemorrhage on fundoscopic examination of the eye may be present. Laboratory findings consistent with the diagnosis of glucagonoma include serum glucagon concentration of 1000pg/ml or greater. Findings on abdominal CT scan suggestive of glucagonoma include reinforced mass in the arterial phase of the enhanced CT scan. Abdominal MRI is helpful in the diagnosis of glucagonoma. On abdominal MRI, glucagonoma is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI. The abdominal ultrasound scan may be helpful in the diagnosis of glucagonoma. Findings on ultrasound scan suggestive of glucagonoma is a hypoechoic tumor in the distal pancreas. Other imaging studies for glucagonoma include positron emission tomography scan and somatostatin receptor scintigraphy. Other diagnostic studies for glucagonoma includes biopsy, which demonstrates epidermal necrosis, subcorneal pustules, either isolated or associated with necrosis of the [[epidermis]], confluent parakeratosis, epidermal [[hyperplasia]], and marked papillary dermal angioplasia and suppurative [[folliculitis]]. The predominant therapy for glucagonoma is surgical resection. Adjunctive [[chemotherapy]] may be required. Surgery is the mainstay of treatment for glucagonoma. The feasibility of surgery depends on the stage of glucagonoma at diagnosis. Secondary prevention measures of glucagonoma include routine [[glucagon]] levels and imaging at scheduled intervals after treatment.

==Historical Perspective==
Glucagonoma was first described in 1942 by Becker. In 1966, McGavran was first to report a case of hyperglucagonemia associated with cutaneous changes. In 1970, Wilkinson described the typical skin eruption in glucagonoma as [[necrolytic migratory erythema]].

==Pathophysiology==
Glucagonoma is a tumor of the [[alpha cells]] of the [[pancreas]] characterized by the excessive secretion of [[glucagon]] and [[necrolytic migratory erythema]]. Glucagonoma causes hyperglucagonemia, [[zinc deficiency]], [[fatty acid]] deficiency, [[Aminoacid|hypoaminoacidemia]] that may cause [[necrolytic migratory erythema]]. Glucagonoma may be a part of [[MEN1 syndrome|type 1 multiple endocrine neoplasia]]. It is an autosomal dominant syndrome that is usually caused by mutations in the [[MEN1 syndrome|''MEN1'' gene]]. [[MEN1 syndrome|''MEN1'' gene]] is a [[tumor suppressor gene]] and causes [[MEN1 syndrome|type 1 multiple endocrine neoplasia]] by [[Knudson hypothesis|Knudson's "two hits" model]] for [[tumor]] development. All glucagonomas are located in the pancreas, 50–80% occur in the pancreatic tail, 32.2% in the body and 21.9% in the head. Glucagonoma can metastasize mainly to the liver. Glucagonomas consist of [[Pleomorphism|pleomorphic]] cells containing granules that stain for other peptides, most frequently [[pancreatic polypeptide]]. [[Immunoperoxidase|Immunoperoxidase staining]] can detect glucagon within the tumor cells and [[Glucagon|glucagon.]]

==Causes==
There are no established causes for glucagonoma. Mostly, glucagonomas are sporadic but 20% are associated with the [[MEN1 syndrome|MEN1 syndrome]].

==Differential Diagnosis==
Glucagonoma must be differentiated from certain skin lesions in which necrolytic migratory erythema can be found such as [[acrodermatitis enteropathica]], [[psoriasis]], [[pellagra]], and [[eczema]]. Glucagonoma should be differentiated from other causes of hyperglucagonemia include [[infection]], [[diabetes mellitus]], [[Cushing syndrome]], [[renal failure]], [[acute pancreatitis]], severe stress, and prolonged fasting.

==Epidemiology and Demographics==
The [[incidence]] of glucagonoma is approximately 0.0005 per 100,000 individuals worldwide. Glucagonoma affects men and women equally. The median age of diagnosis is the fifth decade.

==Risk Factors==
The most common risk factor in the development of glucagonoma is a positive family history of [[Multiple endocrine neoplasia type 1|multiple endocrine neoplasia type 1]], which is characterized by the presence of [[Pituitary adenoma|pituitary adenomas]], [[Islet cell tumor|islet cell tumors]] of the [[pancreas]], and [[hyperparathyroidism]].

==Screening==
Screening of [[multiple endocrine neoplasia type 1]] associated glucagonoma improves [[morbidity]] and [[survival rates]] of patients. Biochemical screening depends on measuring gastrointestinal hormones: [[gastrin]], [[insulin]], [[glucagon]], [[VIP]], [[pancreatic polypeptide]], [[chromogranin A]], [[prolactin]], and [[IGF-1]] in all patients. Radiological screening should include an [[MRI]] of [[Pancreas|the pancreas]], [[adrenal glands]], and [[Pituitary gland|pituitary]] and repeated every 2 years. All high-risk patients should be offered [[genetic counseling]] and [[MEN1]] mutation testing. If the genetic tests result patients should have a periodic clinical, biochemical, and radiological screening program.
==Natural History, Complications and Prognosis==
If left untreated, patients with glucagonoma may progress to develop [[necrolytic migratory erythema]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]]. The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma. The 10-year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis. Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis. Age, grade, and distant metastases are the most significant predictors of survival.
==Staging==
According to The American Joint Committee on Cancer (AJCC), there are four stages of glucagonoma based on the [[TNM staging system]].

==History and Symptoms==
Symptoms of glucagonoma include [[necrolytic migratory erythema]], [[weight loss]], [[glucose intolerance]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]]. A positive family history of [[multiple endocrine neoplasia type 1]] may be present.
==Physical Examination==
Common physical examination findings of glucagonoma include [[tachycardia]], [[fever]], [[rash]], [[muscle atrophy]], cotton wool spots, flame hemorrhage, and dot and blot hemorrhage on fundoscopic examination of the eye may be present.
==Laboratory Findings==
Laboratory findings consistent with the diagnosis of glucagonoma include a serum [[glucagon]] concentration of 1000 pg/ml or greater.
==CT==
Findings on abdominal [[Computed tomography|CT scan]] suggestive of glucagonoma include a reinforced mass in the arterial phase of the enhanced CT scan. Symptomatic, but nonfunctioning, glucagonoma is usually large (>3 cm) at the time of diagnosis.
==MRI==
Abdominal [[Magnetic resonance imaging|MRI]] is helpful in the diagnosis of glucagonoma. On abdominal [[Magnetic resonance imaging|MRI]], glucagonoma is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI.
==Ultrasonography==
The abdominal ultrasound scan may be helpful in the diagnosis of glucagonoma. Finding on ultrasound scan suggestive of glucagonoma is a hypoechoic tumor in the [[pancreas]]. US-guided [[Needle aspiration biopsy|fine-needle aspiration biopsy]] is a non-operative histologic diagnosis. Intraoperative ultrasonography is used as an adjunct to intraoperative palpation.
==Other Imaging Findings==
Other imaging studies for glucagonoma include [[Positron emission tomography|positron emission tomography scan]] and [[somatostatin]] receptor [[scintigraphy]]. [[Scintigraphy]] is less sensitive than [[Positron emission tomography|PET]] scan but still useful.
==Other Diagnostic Studies==
Other diagnostic studies for glucagonoma include venous sampling after a selective injection of a stimulating [[secretin]]. Biopsy, which demonstrates epidermal [[necrosis]], subcorneal [[pustules]], either isolated or associated with [[necrosis]] of the epidermis, confluent [[parakeratosis]], epidermal [[hyperplasia]] and marked papillary dermal angioplasia, and suppurative [[folliculitis]].

==Medical Therapy==
The predominant medical therapy for primary glucagonoma is [[Somatostatin|somatostatin analogs]] ([[octreotide]]). Metastatic tumors need [[Therapeutic embolization|h'''epatic''' artery embolization]], [[Radiofrequency ablation]], and molecular therapy.
==Surgery==
Surgery is the mainstay of treatment for glucagonoma. The feasibility of surgery depends on the stage of glucagonoma at diagnosis. Hepatic resection is indicated for the treatment of metastatic liver disease in patients who are candidates for surgery with no extensive extrahepatic metastases.
==Primary Prevention==
There is no established method for prevention of glucagonoma.
==Secondary Prevention==
Secondary prevention measures of glucagonoma include routine [[glucagon]] levels and imaging at scheduled intervals after treatment.

==References==
{{reflist|2}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Endocrinology]]

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{{WikiDoc Sources}}

Glucagonoma overview

2017-11-17T13:53:10Z

Anthony Gallo:

__NOTOC__
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}

==Overview==
A glucagonoma is a [[tumor]] of the [[alpha cell]]s of the [[pancreas]] that results in overproduction of the [[hormone]] [[glucagon]]. Glucagonoma was first described in 1942 by Becker. On microscopic examination, glucagonoma's consist of [[Pleomorphism|pleomorphic]] cells containing granules that stain for other peptides, most frequently [[pancreatic polypeptide]]. [[Immunoperoxidase|Immunoperoxidase staining]] can detect glucagon within the tumor cells and [[Glucagon|glucagon.]] Similar symptoms are present in cases of pseudo-glucagonoma syndrome in the absence of a glucagon-secreting tumor. Further, glucagonoma must be differentiated from certain skin lesions ([[acrodermatitis enteropathica]], [[psoriasis]], [[pellagra]], [[eczema]]) and other causes of hyperglucagonemia ([[infection]], [[diabetes mellitus]], [[Cushing syndrome]], [[renal failure]], [[acute pancreatitis]], severe stress, and prolonged fasting). The incidence of glucagonoma is approximately .0005 per 100,000 individuals worldwide. Glucagonoma affects men and women equally. The median age at diagnosis of glucagonoma is 52.5 years. The most potent risk factor in the development of glucagonoma is a positive family history of [[multiple endocrine neoplasia type 1]]. If left untreated, patients with glucagonoma may progress to develop [[necrolytic migratory erythema]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]].The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma. The 10-year event-free survival rate is less than 51.6% with metastasis and 64.3% without metastasis. According to The American Joint Committee on Cancer (AJCC), there are four stages of glucagonoma based on the [[TNM staging system]]. Symptoms of glucagonoma include [[necrolytic migratory erythema]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]]. A positive family history of [[multiple endocrine neoplasia type 1]] may be present. Common physical examination findings of glucagonoma include [[tachycardia]], [[fever]], [[rash]], [[muscle atrophy]], cotton wool spots, flame hemorrhage, and dot and blot hemorrhage on fundoscopic examination of the eye may be present. Laboratory findings consistent with the diagnosis of glucagonoma include serum glucagon concentration of 1000pg/ml or greater. Findings on abdominal CT scan suggestive of glucagonoma include reinforced mass in the arterial phase of the enhanced CT scan. Abdominal MRI is helpful in the diagnosis of glucagonoma. On abdominal MRI, glucagonoma is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI. The abdominal ultrasound scan may be helpful in the diagnosis of glucagonoma. Findings on ultrasound scan suggestive of glucagonoma is a hypoechoic tumor in the distal pancreas. Other imaging studies for glucagonoma include positron emission tomography scan and somatostatin receptor scintigraphy. Other diagnostic studies for glucagonoma includes biopsy, which demonstrates epidermal necrosis, subcorneal pustules, either isolated or associated with necrosis of the [[epidermis]], confluent parakeratosis, epidermal [[hyperplasia]], and marked papillary dermal angioplasia and suppurative [[folliculitis]]. The predominant therapy for glucagonoma is surgical resection. Adjunctive [[chemotherapy]] may be required. Surgery is the mainstay of treatment for glucagonoma. The feasibility of surgery depends on the stage of glucagonoma at diagnosis. Secondary prevention measures of glucagonoma include routine [[glucagon]] levels and imaging at scheduled intervals after treatment.

==Historical Perspective==
Glucagonoma was first described in 1942 by Becker. In 1966, McGavran was first to report a case of hyperglucagonemia associated with cutaneous changes. In 1970, Wilkinson described the typical skin eruption in glucagonoma as [[necrolytic migratory erythema]].

==Pathophysiology==
Glucagonoma is a tumor of the [[alpha cells]] of the [[pancreas]] characterized by the excessive secretion of [[glucagon]] and [[necrolytic migratory erythema]]. Glucagonoma causes hyperglucagonemia, [[zinc deficiency]], [[fatty acid]] deficiency, [[Aminoacid|hypoaminoacidemia]] that may cause [[necrolytic migratory erythema]]. Glucagonoma may be a part of [[MEN1 syndrome|type 1 multiple endocrine neoplasia]]. It is an autosomal dominant syndrome that is usually caused by mutations in the [[MEN1 syndrome|''MEN1'' gene]]. [[MEN1 syndrome|''MEN1'' gene]] is a [[tumor suppressor gene]] and causes [[MEN1 syndrome|type 1 multiple endocrine neoplasia]] by [[Knudson hypothesis|Knudson's "two hits" model]] for [[tumor]] development. All glucagonomas are located in the pancreas, 50–80% occur in the pancreatic tail, 32.2% in the body and 21.9% in the head. Glucagonoma can metastasize mainly to the liver. Glucagonomas consist of [[Pleomorphism|pleomorphic]] cells containing granules that stain for other peptides, most frequently [[pancreatic polypeptide]]. [[Immunoperoxidase|Immunoperoxidase staining]] can detect glucagon within the tumor cells and [[Glucagon|glucagon.]]

==Causes==
There are no established causes for glucagonoma. Mostly, glucagonomas are sporadic but 20% are associated with the [[MEN1 syndrome|MEN1 syndrome]].

==Differential Diagnosis==
Glucagonoma must be differentiated from certain skin lesions in which necrolytic migratory erythema can be found such as [[acrodermatitis enteropathica]], [[psoriasis]], [[pellagra]], and [[eczema]]. Glucagonoma should be differentiated from other causes of hyperglucagonemia include [[infection]], [[diabetes mellitus]], [[Cushing syndrome]], [[renal failure]], [[acute pancreatitis]], severe stress, and prolonged fasting.

==Epidemiology and Demographics==
The [[incidence]] of glucagonoma is approximately 0.0005 per 100,000 individuals worldwide. Glucagonoma affects men and women equally. The median age of diagnosis is the fifth decade.

==Risk Factors==
The most common risk factor in the development of glucagonoma is a positive family history of [[Multiple endocrine neoplasia type 1|multiple endocrine neoplasia type 1]], which is characterized by the presence of [[Pituitary adenoma|pituitary adenomas]], [[Islet cell tumor|islet cell tumors]] of the [[pancreas]], and [[hyperparathyroidism]].

==Screening==
Screening of [[multiple endocrine neoplasia type 1]] associated glucagonoma improves [[morbidity]] and [[survival rates]] of patients. Biochemical screening depends on measuring gastrointestinal hormones: [[gastrin]], [[insulin]], [[glucagon]], [[VIP]], [[pancreatic polypeptide]], [[chromogranin A]], [[prolactin]], and [[IGF-1]] in all patients. Radiological screening should include an [[MRI]] of [[Pancreas|the pancreas]], [[adrenal glands]], and [[Pituitary gland|pituitary]] and repeated every 2 years. All high-risk patients should be offered [[genetic counseling]] and [[MEN1]] mutation testing. If the genetic tests result patients should have a periodic clinical, biochemical, and radiological screening program.
==Natural History, Complications and Prognosis==
If left untreated, patients with glucagonoma may progress to develop [[necrolytic migratory erythema]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]]. The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma. The 10-year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis. Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis. Age, grade, and distant metastases are the most significant predictors of survival.
==Staging==
According to The American Joint Committee on Cancer (AJCC), there are four stages of glucagonoma based on the TNM staging system.
==History and Symptoms==
Symptoms of glucagonoma include [[necrolytic migratory erythema]], [[weight loss]], [[glucose intolerance]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]]. A positive family history of [[multiple endocrine neoplasia type 1]] may be present.
==Physical Examination==
Common physical examination findings of glucagonoma include [[tachycardia]], [[fever]], [[rash]], [[muscle atrophy]], cotton wool spots, flame hemorrhage, and dot and blot hemorrhage on fundoscopic examination of the eye may be present.
==Laboratory Findings==
Laboratory findings consistent with the diagnosis of glucagonoma include a serum [[glucagon]] concentration of 1000 pg/ml or greater.
==CT==
Findings on abdominal [[Computed tomography|CT scan]] suggestive of glucagonoma include a reinforced mass in the arterial phase of the enhanced CT scan. Symptomatic, but nonfunctioning, glucagonoma is usually large (>3 cm) at the time of diagnosis.
==MRI==
Abdominal [[Magnetic resonance imaging|MRI]] is helpful in the diagnosis of glucagonoma. On abdominal [[Magnetic resonance imaging|MRI]], glucagonoma is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI.
==Ultrasonography==
The abdominal ultrasound scan may be helpful in the diagnosis of glucagonoma. Finding on ultrasound scan suggestive of glucagonoma is a hypoechoic tumor in the [[pancreas]]. US-guided [[Needle aspiration biopsy|fine-needle aspiration biopsy]] is a non-operative histologic diagnosis. Intraoperative ultrasonography is used as an adjunct to intraoperative palpation.
==Other Imaging Findings==
Other imaging studies for glucagonoma include [[Positron emission tomography|positron emission tomography scan]] and [[somatostatin]] receptor [[scintigraphy]]. [[Scintigraphy]] is less sensitive than [[Positron emission tomography|PET]] Scan but still useful.
==Other Diagnostic Studies==
Other diagnostic studies for glucagonoma include venous sampling after a selective injection of a stimulating [[secretin]]. Biopsy, which demonstrates epidermal [[necrosis]], subcorneal [[pustules]], either isolated or associated with [[necrosis]] of the epidermis, confluent [[parakeratosis]], epidermal [[hyperplasia]] and marked papillary dermal angioplasia, and suppurative [[folliculitis]].

==Medical Therapy==
The predominant medical therapy for primary glucagonoma is [[Somatostatin|somatostatin analogs]] ([[octreotide]]). Metastatic tumors need [[Therapeutic embolization|h'''epatic''' artery embolization]], [[Radiofrequency ablation]], and molecular therapy.
==Surgery==
Surgery is the mainstay of treatment for glucagonoma. The feasibility of surgery depends on the stage of glucagonoma at diagnosis. Hepatic resection is indicated for the treatment of metastatic liver disease in patients who are candidates for surgery with no extensive extrahepatic metastases.
==Primary Prevention==
There is no established method for prevention of glucagonoma.
==Secondary Prevention==
Secondary prevention measures of glucagonoma include routine [[glucagon]] levels and imaging at scheduled intervals after treatment.

==References==
{{reflist|2}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Endocrinology]]

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{{WikiDoc Sources}}

Glucagonoma

2017-11-17T13:43:00Z

Anthony Gallo:

__NOTOC__
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
{{Glucagonoma}}
{{CMG}}; {{AE}} {{PSD}}, {{MAD}}

{{SK}} Alpha cell adenoma, Alpha cell tumor
==[[Glucagonoma overview|Overview]]==

==[[Glucagonoma historical perspective|Historical Perspective]]==

==[[Glucagonoma pathophysiology|Pathophysiology]]==

==[[Glucagonoma causes|Causes]]==

==[[Glucagonoma differential diagnosis|Differentiating Glucagonoma from other Diseases]]==

==[[Glucagonoma epidemiology and demographics|Epidemiology and Demographics]]==

==[[Glucagonoma risk factors|Risk Factors]]==

==[[Glucagonoma screening|Screening]]==

==[[Glucagonoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]==

==Diagnosis==
[[Glucagonoma staging|Staging]] | [[Glucagonoma history and symptoms|History and Symptoms]] | [[Glucagonoma physical examination|Physical Examination]] | [[Glucagonoma laboratory tests|Laboratory Findings]] | [[Glucagonoma chest x ray|Chest X Ray]] | [[Glucagonoma CT|CT]] | [[Glucagonoma MRI|MRI]] | [[Glucagonoma ultrasound|Ultrasound]] | [[Glucagonoma other imaging findings|Other Imaging Findings]] | [[Glucagonoma other diagnostic studies|Other Diagnostic Studies]]

==Treatment==
[[Glucagonoma medical therapy|Medical Therapy]] | [[Glucagonoma surgery|Surgery]] | [[Glucagonoma primary prevention|Primary Prevention]] | [[Glucagonoma secondary prevention|Secondary Prevention]] | [[Glucagonoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Glucagonoma future or investigational therapies|Future or Investigational Therapies]]

==Case Studies==
[[Glucagonoma case study one|Case#1]]

{{Epithelial neoplasms}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Endocrinology]]

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Pyloric stenosis risk factors

2017-11-16T16:15:16Z

Anthony Gallo:

__NOTOC__
{{Pyloric stenosis}}
{{CMG}}; {{AE}} {{MMJ}}

==Overview==
The most potent risk factors in the development of infantile pyloric stenosis are bottle-feed infant, [[caesarean section]] delivery, first-born infant, preterm birth, and exposure to [[Macrolide|macrolides]], [[nitrofurantoin]], [[Penicillin|penicillins]] and trimethoprim-sulphamethoxazole during [[pregnancy]].

==Risk Factors==
=== Risk Factors for infantile pyloric stenosis===
Risk factors for infantile pyloric stenosis include:
*Bottle-feed infant
*[[Caesarean section|Cesarean section]] delivery
*First-born infant
*[[Premature birth|Preterm birth]]
*Exposure to [[Macrolide|macrolides]], [[nitrofurantoin]], [[Penicillin|penicillins]] and [[trimethoprim-sulphamethoxazole|trimethoprim-sulphamethoxazole during pregnancy]]<ref name="pmid26905846">{{cite journal| author=Nordeng S, Nordeng H, Høye S| title=[Use of antibiotics during pregnancy]. | journal=Tidsskr Nor Laegeforen | year= 2016 | volume= 136 | issue= 4 | pages= 317-21 | pmid=26905846 | doi=10.4045/tidsskr.15.0451 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26905846 }} </ref><ref name="pmid26905846" />

=== Risk Factors for adult-onset hypertrophic pyloric stenosis (HPS)===
There is no established risk factor for adult-onset hypertrophic pyloric stenosis (HPS).

==References==
{{Reflist|2}}

[[Category:Needs content]]
[[Category:Gastroenterology]]
[[Category:Surgery]]

{{WH}}
{{WS}}

Pyloric stenosis epidemiology and demographics

2017-11-16T16:05:03Z

Anthony Gallo:

__NOTOC__
{{Pyloric stenosis}}
{{CMG}}; {{AE}} {{MMJ}}

==Overview==
Pyloric stenosis occurs in 1 of every 200-300 live births and it is four times more common in males.it is rare in Asians, and it is 2 to 3 times more common in Caucasian compared to African American babies.In one study the prevalence of congenital hypertrophic pyloric stenosis in Donetsk region in the course of 11 years (1989-1999) was 0.73:1000 or 1:1370 newborn infants annual with range of 0.54 to 1.01:1000.<ref name="pmid11944318" /> Studies show the mortality rate of pyloric stenosis is very low and usually results from delays in diagnosis that causes sever dehydration and shock.

==Epidemiology and Demographics==

===Incidence===
Pyloric stenosis occurs in 1 of every 200-300 live births.

===Gender===
Pyloric stenosis is four times more common in males.

===Race===
Pyloric stenosis is rare in Asians, and it is 2 to 3 times more common in Caucasian compared to African American babies.

===Prevalence===
In one study the prevalence of congenital hypertrophic pyloric stenosis in Donetsk region in the course of 11 years (1989-1999) was 0.73:1000 or 1:1370 newborn infants annual with range of 0.54 to 1.01:1000.<ref name="pmid11944318">{{cite journal| author=Mukhin VN, Moskalenko VZ, Grona VN, Sopov GA, Linchevskiĭ GL| title=[Population prevalence of congenital hypertrophic pyloric stenosis in the Donetsk region of Ukraine]. | journal=Tsitol Genet | year= 2001 | volume= 35 | issue= 5 | pages= 60-4 | pmid=11944318 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11944318 }} </ref>

===Case-fatality rate/Mortality rate===
Studies show the mortality rate of pyloric stenosis is very low and usually results from delays in diagnosis that causes sever dehydration and shock.
==References==
{{Reflist|2}}

[[Category:Needs content]]
[[Category:Gastroenterology]]
[[Category:Surgery]]

{{WH}}
{{WS}}

Pyloric stenosis causes

2017-11-16T15:52:13Z

Anthony Gallo:

__NOTOC__
{{Pyloric stenosis}}

{{CMG}}; {{AE}} {{MMJ}}
==Overview==
Common causes of adult-onset hypertrophic pyloric stenosis (HPS) include persisting duodenal hyperacidity, [[Inheritance (genetic algorithm)|inheritance]] of a [[parietal cell]] mass (PCM) at the upper end of the normal range which causes persisting duodenal hyperacidity.

==Causes==
Various causes of pyloric stenosis include:<ref name="pmid16449017">{{cite journal| author=Rogers IM| title=The true cause of pyloric stenosis is hyperacidity. | journal=Acta Paediatr | year= 2006 | volume= 95 | issue= 2 | pages= 132-6 | pmid=16449017 | doi=10.1080/08035250500431385 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16449017 }} </ref>

===Causes of adult-onset hypertrophic pyloric stenosis===
*Persisting duodenal hyperacidity
*Inheritance of a [[parietal cell]] mass (PCM) at the upper end of the normal range which causes persisting duodenal hyperacidity.

=== '''Causes of infantile pyloric stenosis''' ===
Diseases such as gastroenteritis, [[Urinary tract infection|urinary tract infections]], [[pyelonephritis]], [[adrenal insufficiency]], [[acute renal failure]], and [[inborn errors of metabolism]] are the most common causes of infantile pyloric stenosis.

==References==
{{Reflist|2}}

{{WH}}
{{WS}}
[[Category: (name of the system)]]

Pyloric stenosis causes

2017-11-16T15:50:27Z

Anthony Gallo:

__NOTOC__
{{Pyloric stenosis}}

{{CMG}}; {{AE}} {{MMJ}}
==Overview==
Common causes of adult-onset hypertrophic pyloric stenosis (HPS) include persisting duodenal hyperacidity, [[Inheritance (genetic algorithm)|inheritance]] of a [[parietal cell]] mass (PCM) at the upper end of the normal range which causes persisting duodenal hyperacidity.

==Causes==
Various causes of pyloric stenosis include:<ref name="pmid16449017">{{cite journal| author=Rogers IM| title=The true cause of pyloric stenosis is hyperacidity. | journal=Acta Paediatr | year= 2006 | volume= 95 | issue= 2 | pages= 132-6 | pmid=16449017 | doi=10.1080/08035250500431385 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16449017 }} </ref>

===Causes of adult-onset hypertrophic pyloric stenosis===
*Persisting duodenal hyperacidity
*Inheritance of a [[parietal cell]] mass (PCM) at the upper end of the normal range which causes persisting duodenal hyperacidity.

=== '''Causes of infantile pyloric stenosis''' ===
Diseases such as gastroentritis ,[[Urinary tract infection|urinary tract infections]] ,[[pyelonephritis]] ,[[adrenal insufficiency]] ,[[Acute renal failure (patient information)|acute renal failure]] and inborn errors of [[metabolism]] are the most common causes of infantile pyloric stenosis.

==References==
{{Reflist|2}}

{{WH}}
{{WS}}
[[Category: (name of the system)]]

Pyloric stenosis differential diagnosis

2017-11-16T15:49:29Z

Anthony Gallo:

__NOTOC__
{{Pyloric stenosis}}
{{CMG}}; {{AE}} {{MMJ}}

==Overview==
*Infantile hypertrophic pyloric stenosis must be differentiated from other diseases that cause [[vomiting]], poor feeding, [[dehydration]], such as [[adrenal insufficiency]], [[gastroenteritis]], [[UTI]], and [[acute renal failure]].

==Differentiating Pyloric Stenosis from Other Diseases==
Pyloric stenosis must be differentiated from other diseases, such as:
* [[Adrenal Insufficiency|Adrenal insufficiency]]
* [[Gastroenteritis]]
* Inborn errors of [[metabolism]]
* [[Urinary Tract Infections|Urinary tract infections]] and [[Pyelonephritis]]
* [[Renal Failure|Acute renal failure]]

==Preferred Table==

{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! rowspan="2" |Diseases
! colspan="4" |Laboratory Findings
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
!Dehydration
!Hypokalemia
!Acidosis or Alkalosis
!Hypochloremia or hyperchloremia
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Pyloric stenosis
| style="background: #F5F5F5; padding: 5px;" | ++
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |Alkalosis
| style="background: #F5F5F5; padding: 5px;" | Hypochloremia
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Adrenal insufficency
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |Hyperkalemia
| style="background: #F5F5F5; padding: 5px;" |Acidosis
| style="background: #F5F5F5; padding: 5px;" | Hypochloremia
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Gastroenteritis
| style="background: #F5F5F5; padding: 5px;" | ++
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |Acidosis
| style="background: #F5F5F5; padding: 5px;" | Hypochloremia
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |UTI
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" | Acidosis or alkalosis
| style="background: #F5F5F5; padding: 5px;" | Hypochloremia or hyperchloremia
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Acute renal failure
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |Hyperkalemia
| style="background: #F5F5F5; padding: 5px;" |Acidosis
| style="background: #F5F5F5; padding: 5px;" |hyperchloremia
|}

==References==
{{Reflist|2}}

[[Category:Needs content]]
[[Category:Gastroenterology]]
[[Category:Surgery]]

{{WH}}
{{WS}}

Pyloric stenosis pathophysiology

2017-11-16T15:41:32Z

Anthony Gallo:

__NOTOC__
{{Pyloric stenosis}}
{{CMG}} {{AE}} {{MMJ}}

==Overview==
The [[pathogenesis]] of infantile hypertrophic pyloric stenosis is not fully understood but it may be due abnormal innervation of the pyloric [[smooth muscle]].

== Pathophysiology ==
The [[pathogenesis]] of infantile hypertrophic pyloric stenosis is not fully understood but it may be due abnormal innervation of the pyloric [[smooth muscle]]. There are also some [[locus]] evidences of increased [[collagen]] production and abnormal amounts of [[extracellular matrix protein]]s in hypertrophic pyloric [[muscle]].<ref name="pmid9553181">{{cite journal| author=Ohshiro K, Puri P| title=Pathogenesis of infantile hypertrophic pyloric stenosis: recent progress. | journal=Pediatr Surg Int | year= 1998 | volume= 13 | issue= 4 | pages= 243-52 | pmid=9553181 | doi=10.1007/s003830050308 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9553181 }} </ref> There is no known [[pathophysiology]] for adult-onset hypertrophic pyloric stenosis (HPS).

== Genetics ==
In one study, the familial recurrence pattern among monozygotic cotwins and more remote relatives of IHPS probands was found to be inconsistent with generalized single major inheritance.<ref name="pmid8237916">{{cite journal| author=Mitchell LE, Risch N| title=The genetics of infantile hypertrophic pyloric stenosis. A reanalysis. | journal=Am J Dis Child | year= 1993 | volume= 147 | issue= 11 | pages= 1203-11 | pmid=8237916 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8237916 }} </ref>

== Associated Conditions of Infantile Hypertrophic Pyloric Stenosis ==
*Neuromuscular disorders
*[[Connective tissue]] disorders
*[[Metabolic disorder|Metabolic disorders]]
*[[Intracellular]] signalling pathway disturbances
*Intercellular communication disturbances
*Ciliopathies
*[[DNA]]-repair disturbances
*[[Transcription (genetics)|Transcription]] regulation disorders
*MAPK-pathway disturbances
*[[Lymphatic system|Lymphatic]] abnormalities
*Environmental factors<ref name="pmid22777173">{{cite journal| author=Peeters B, Benninga MA, Hennekam RC| title=Infantile hypertrophic pyloric stenosis--genetics and syndromes. | journal=Nat Rev Gastroenterol Hepatol | year= 2012 | volume= 9 | issue= 11 | pages= 646-60 | pmid=22777173 | doi=10.1038/nrgastro.2012.133 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22777173 }} </ref>

==References==
{{Reflist|2}}

Pyloric stenosis classification

2017-11-16T15:35:30Z

Anthony Gallo:

__NOTOC__
{{Pyloric stenosis}}
{{CMG}}; {{AE}} {{MMJ}}

==Overview==
There is no established system for the classification of pyloric stenosis. It may be subclassified as infantile pyloric stenosis and adult-onset hypertrophic pyloric stenosis (HPS).

==Classification==
There is no established system for the classification of pyloric stenosis. It may be subclassified as infantile pyloric stenosis and adult-onset hypertrophic pyloric stenosis (HPS).

==References==
{{Reflist|2}}

{{WH}}
{{WS}}

Pyloric stenosis historical perspective

2017-11-16T15:29:56Z

Anthony Gallo:

__NOTOC__
{{Pyloric stenosis}}
{{CMG}}; {{AE}} {{MMJ}}

==Overview==
In 1717, Dr. Patrick Blair reported [[autopsy]] findings of pyloric stenosis for first time. In 1912, Conrad Ramstedt observed an uneventful recovery in a patient following [[pyloroplasty]], which remains standard procedure for pyloric stenosis today.

==Historical Perspective==
* In 1717, Dr. Patrick Blair first reported [[autopsy]] findings of pyloric stenosis.
* In 1887, the clinical picture and [[pathology]] of pyloric stenosis was described by the Danish pediatrician Harald [[Hirschsprung's disease|Hirschsprung]].
* In 1912, Conrad Ramstedt observed an uneventful recovery in a patient following [[pyloroplasty]].
* The Ramstedt [[pyloromyotomy]] remains the standard procedure for pyloric stenosis today.

==References==
{{Reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Surgery]]

Constipation historical perspective

2017-11-16T15:22:59Z

Anthony Gallo:

__NOTOC__
{{Constipation}}
{{CMG}}; {{AE}} {{EG}}

==Overview==
The Egyptian [[Ebers Papyrus|Ebers papyrus]], from 16th century BC is the first book that presented a basic description for constipation. [[Ebers papyrus]] defined constipation as [[intoxication]] of body with hazardous agents from [[feces]] in [[bowels]]. In early 1900s, all-bran products were first introduced to prevent and treat auto-[[Intoxication|intoxicated]] [[patients]] due to constipation. In 1970s and 1980s, Denis Burkitt, an English [[surgeon]], claimed the [[hypothesis]] about [[dietary]] fibers followed by the definition of '''''"The Commonest Western disease"'''''.

==Historical Perspective==
<div style="align:center;">
{{family tree/start}}
{{family tree| | | | |,| A01 | | |A01='''''16th century BC''''' '''The Egyptian [[Ebers Papyrus|Ebers papyrus]]''' First definition of constipation as [[intoxication]] of body toxins from [[feces]] in [[bowels]]}}
{{family tree| | | | |!| |:| | | |}}
{{family tree| | | | |!| |:| | | |}}
{{family tree| | | | |)| B01 | | |B01='''''18th century''''' '''Personal [[physician]] of Louis XV in France''' Definie constipation as [[blood]] pollution with released [[toxins]] from remained wastes in the [[intestines]]}}
{{family tree| | | | |!| |:| | | |}}
{{family tree| | | | |)| C01 | | |C01='''''Beginning of 19th century''''' '''[[Physicians]]''' Believed constipation as [[disease]] of civilization and urban population}}
{{family tree| | | | |!| |!| | | |}}
{{family tree| | | | |)| D01 | | |D01='''''1850s''''' '''An American health manual''' Revealed that "daily emptying the [[bowels]] is of the utmost importance in being [[healthy]]"}}
{{family tree| | | | |!| |!| | | |}}
{{family tree| | E01 |+| E02 | | |E01='''Disease perspective'''|E02='''''1906''''' '''Charles Bouchard, a French [[physician]]''' Proposed the "'''''auto-[[intoxication]] theory'''''"}}
{{family tree| | | | |!| |!| | | |}}
{{family tree| | | | |)| F01 | | |F01='''''1923''''' '''William Walsh, an American [[physician]]''' Mentioned that not all the symptoms related to [[poisons]] released from remained [[feces]]}}
{{family tree| | | | |!| |!| | | |}}
{{family tree| | | | |)| G01 | | |G01='''''1924''''' '''Arbuthnot Lane, a British [[physician]]''' Pointed out “'''''the whiter your bread, the sooner you're dead'''''”}}
{{family tree| | | | |!| |!| | | |}}
{{family tree| | | | |)| H01 | | |H01='''''1928''''' '''Charles Campbell, an American [[physician]]''' Postulated that remained wastes in [[colon]] are decomposing and may make the body full of [[poisons]]}}
{{family tree| | | | |!| |!| | | |}}
{{family tree| | | | |`| I01 | | |I01='''''1928''''' '''Victor Paucher, a French [[internist]]''' Suggested that stasis of [[feces]] in [[bowels]] creates "Sewer-like blood"}}
{{family tree/end}}
</div>

* The Egyptian [[Ebers Papyrus|Ebers papyrus]], from 16th century BC, is the first book that presented a basic description for constipation. [[Ebers papyrus]] defined constipation as [[intoxication]] of body with hazardous agents from [[feces]] in [[bowels]].<ref>{{Citation | author1=Ebbell, B. (Bendix), 1865- | title=The Papyrus Ebers : the greatest Egyptian medical document | publication-date=1937 | publisher=Levin & Munksgaard | url=http://trove.nla.gov.au/work/26409288 | accessdate=14 November 2017}}</ref>
* In 18th century, the personal [[physician]] of Louis XV in France, presented a similar definition as [[Ebers papyrus]]. He mentioned the constipation as [[blood]] pollution with released [[toxins]] from remained wastes in the [[intestines]].<ref>{{Citation
| title=Synopsis of the universal practice of medicine [electronic resource] : exhibiting a concise view of all diseases, both internal and external : illustrated with complete commentaries / by Joseph Lieutaud ; translated from the Latin by Edwin A. Atlee
| author1=Lieutaud, Joseph, 1703-1780
| author2=Atlee, Edwin Augustus, 1776-1852
| year=1816
| publisher=Edward and Richard Parker
| language=English
}}</ref>
* In the beginning of 19th century, [[physicians]] believed that constipation was a [[disease]] of civilization and urban population was mostly involved with constipation.<ref name="pmid11124189">{{cite journal| author=Whorton J| title=Civilisation and the colon: constipation as the "disease of diseases". | journal=BMJ | year= 2000 | volume= 321 | issue= 7276 | pages= 1586-9 | pmid=11124189 | doi= | pmc=1119264 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11124189 }}</ref>
* In 1850s, an American health manual revealed that "daily emptying the [[bowels]] is of the utmost importance in being [[healthy]]". Daily [[bowel]] movement was also suggested to prevent derangement and disturbance in body.<ref>{{Citation
| title=People's medical lighthouse; a series of popular and scientific essays.
| author1=Root, Harmon Knox
| year=1854
| publisher=New York, Ranney
| language=English
}}</ref>
* In 1906, Charles Bouchard, a French [[physician]] proposed the "'''''auto-[[intoxication]] theory'''''", constipated person is continuously try to [[suicide]] by [[intoxication]] with [[toxins]] which are produced by remained [[feces]] in his [[intestine]].<ref>{{Citation
| title=Lectures on Auto-Intoxication in Disease: Or, Self-Poisoning of the Individual
| author1=Bouchard, Charles
| year=1906
| publisher=Philadelphia, F. A. Davis Company
| language=English
}}</ref>
* In 1923, William Walsh, an American [[physician]], mentioned that not all the symptoms and severity of constipation related to [[poisons]] released from remained [[feces]] in [[bowels]].<ref>{{cite journal|title=The Conquest of Constipation.|journal=JAMA: The Journal of the American Medical Association|volume=81|issue=2|year=1923|pages=158|issn=0098-7484|doi=10.1001/jama.1923.02650020076035}}</ref>
* In 1924, Arbuthnot Lane, a British [[physician]], pointed out the relation between [[colon cancer]] and constipation. Lane presented that “'''''the whiter your bread, the sooner you're dead'''''”.<ref name="pmid20766844" />
* In 1928, Charles Campbell, an American [[physician]], postulated that remained wastes in [[colon]] are decomposing and may make the body full of [[poisons]].<ref>{{cite journal|title=The Lazy Colon. Newer Methods and Latest Advances of Science in the Treatment of Constipation.|journal=JAMA: The Journal of the American Medical Association|volume=90|issue=26|year=1928|pages=2134|issn=0098-7484|doi=10.1001/jama.1928.02690530062033}}</ref>
* In 1928, Victor Paucher, a French [[internist]], suggested that stasis of [[feces]] in [[bowels]] make their [[poisons]] secreted into [[blood]] and creates "Sewer-like blood".<ref>{{Citation
| title=The trainers bible
| author1=Bilik, Samuel Ernest
| year=1928
| publisher=New York city, Athletic trainers supply Co.
| language=English
}}</ref>

== Landmark Events in the Development of Treatment Strategies ==
{{family tree/start}}
{{family tree| | | | | A01 | | |A01='''1900'''}}
{{family tree| | | | | |!| | | | |,| A01 | |A01='''All-bran products''' first introduced to the [[Prevention|prevention]] and treatment of auto-[[Intoxication]] due to constipation}}
{{family tree| | | | | |)|-| B01 |(| | | | |B01='''''Early 1900s''''' }}
{{family tree| | | | | |!| | | | |`| C01 | |C01='''Yeasts''' introduced in the yogurt were also postulated to prevent the constipation and following auto-[[intoxication]]}}
{{family tree| | | | | |!| | | | | | | | | |}}
{{family tree| | | | | |!| | | | |,| A01 | |A01=Arbuthnot Lane, a British [[physician]], introduced '''[[Phenolphthalein]]''' as a strong [[laxative]] for children}}
{{family tree| | | | | |)|-| D01 |(| | | | |D01='''''1913'''''}}
{{family tree| | | | | |!| | | | |`| A01 | |A01=Arbuthnot Lane, a British [[physician]], revealed that maintaining the normal human '''"drainage scheme"''' is the main treatment for constipation}}
{{family tree| | | | | |!| | | | | | | | | |}}
{{family tree| | | | | |!| | | | | | | | | |}}
{{family tree| | | | | |!| | | | | | | | | |}}
{{family tree| | | | | |!| | | | | | | | | |}}
{{family tree| | | | | |!| | | | | | | | | |}}
{{family tree| | | | | |)|-| E01 |-| E02 | |E01='''''1981'''''|E02=Denis Burkitt an English [[surgeon]], claimed the [[hypothesis]] about [[dietary]] fibers followed by the definition of '''''"The Commonest Western disease"'''''}}
{{family tree| | | | | |!| | | | | | | | | |}}
{{family tree| | | | | B01 | | |B01='''2000'''}}
{{family tree/end}}
* In early 1900s, all-bran products first introduced to the [[Prevention|prevention]] and treatment of auto-[[Intoxication]] due to constipation.<ref name="pmid11124189" />
* In early 1900s, yeasts were also postulated to prevent the constipation and following auto-[[intoxication]], when introduced in the yogurt.<ref name="pmid11124189" />
* In 1913, [[Phenolphthalein]] was introduced as a strong [[laxative]] for children. [[Phenolphthalein]] quickly claimed as the best [[laxative]] through maneuvering about auto-[[intoxication]].<ref name="pmid20766844">{{cite journal| author=Lane WA| title=An Address ON CHRONIC INTESTINAL STASIS: Delivered at the North-East London Post-Graduate College. | journal=Br Med J | year= 1913 | volume= 2 | issue= 2757 | pages= 1125-8 | pmid=20766844 | doi= | pmc=2346322 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20766844 }}</ref>
* From 1900 to 1920, Arbuthnot Lane, a British [[physician]], revealed that maintaining the normal human "drainage scheme" is the main treatment for constipation.<ref name="pmid20766844" />
* In 1970s and 1980s, Denis Burkitt an English [[surgeon]], claimed the [[hypothesis]] about [[dietary]] fibers followed by the definition of '''''"The Commonest Western disease"'''''.<ref>{{cite book | last = Burkitt | first = D. P. | title = Western diseases, their emergence and prevention | publisher = Harvard University Press | location = Cambridge, Mass | year = 1981 | isbn = 978-0674950207 }}</ref>

==References==
{{Reflist|2}}

[[Category:Medicine]]
[[Category:Gastroenterology]]
[[Category:Primary care]]

{{WH}}
{{WS}}

User talk:Kristenkristen

2017-06-16T03:34:23Z

Anthony Gallo: Welcome!

'''Welcome to ''wikidoc''!'''
We hope you will contribute much and well.
You will probably want to read the [https://www.mediawiki.org/wiki/Special:MyLanguage/Help:Contents help pages].
Again, welcome and have fun! [[User:Anthony Gallo|Anthony Gallo]] ([[User talk:Anthony Gallo|talk]]) 03:34, 16 June 2017 (UTC)

User:Kristenkristen

2017-06-16T03:34:23Z

Anthony Gallo: Creating user page for new user.

Medical school student at the Lewis Katz School of Medicine at Temple University, class of 2020.
Bachelor of Arts from Colgate University with a concentration in behavioral neuroscience, class of 2015.

I am always interested in finding and utilizing relevant and helpful resources for medical students. I believe that sharing these resources with my classmates will better prepare us to enter the team-based field of healthcare.

Appendicular abscess surgery

2017-05-02T15:23:56Z

Anthony Gallo: /* Interval Appendectomy */

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}

==Overview==
Following drain and antibiotics, an [[Appendectomy|interval appendectomy]] is recommended for patients after six to eight weeks. The surgical approach can be either laparoscopic or open (laparotomic).

==Surgery==
===Percutaneous drainage===
*Percutaneous drainage can be performed under ultrasound or CT guidance, using either the [[Seldinger technique|Seldinger]] or [[trocar]] technique.<ref name="pmid14767853">{{cite journal |vauthors=Hogan MJ |title=Appendiceal abscess drainage |journal=Tech Vasc Interv Radiol |volume=6 |issue=4 |pages=205–14 |year=2003 |pmid=14767853 |doi= |url=}}</ref>
*Ultrasound is limited if the [[abscess]] is small, obscured by other structures, or if precise placement is required because of nearby [[vessels]] or [[organs]]. In these cases, CT is the optimal imaging modality.<ref name="pmid11232683">{{cite journal |vauthors=Gress F, Schmitt C, Sherman S, Ciaccia D, Ikenberry S, Lehman G |title=Endoscopic ultrasound-guided celiac plexus block for managing abdominal pain associated with chronic pancreatitis: a prospective single center experience |journal=Am. J. Gastroenterol. |volume=96 |issue=2 |pages=409–16 |year=2001 |pmid=11232683 |doi=10.1111/j.1572-0241.2001.03551.x |url=}}</ref>
*When an [[abscess]] is deep in the [[pelvis]], depending on the specific location of the [[fluid]] collection, access may be obtained via transgluteal, transvaginal, or transrectal approaches.<ref name="urlRetroperitoneal Perforation of the Appendix Presenting as a Right Thigh Abscess">{{cite web |url=http://dx.doi.org/10.1155/2015/707191 |title=Retroperitoneal Perforation of the Appendix Presenting as a Right Thigh Abscess |format= |work= |accessdate=}}</ref>
*If the [[fluid]] collection is [[sterile]], a transgluteal approach is preferred because it allows for sterile technique.<ref name="pmid11232683">{{cite journal |vauthors=Gress F, Schmitt C, Sherman S, Ciaccia D, Ikenberry S, Lehman G |title=Endoscopic ultrasound-guided celiac plexus block for managing abdominal pain associated with chronic pancreatitis: a prospective single center experience |journal=Am. J. Gastroenterol. |volume=96 |issue=2 |pages=409–16 |year=2001 |pmid=11232683 |doi=10.1111/j.1572-0241.2001.03551.x |url=}}</ref>
*Depending on the location of abscess, patient is placed in prone or supine position on the CT table.
*Localization scan using CT allows in selecting a safe window of access into the collection.
*A coaxial micropuncture introducer set is advanced into the abscess under CT guidance.
*An Amplatz guidewire is advanced through the sheath and coiled within the abscess.
*After serial dilatation of the tract with a dilator, an pigtail drain is advanced over the guidewire and deployed.

===Emergency appendectomy===
Indications:
*When patients present with life-threatening signs of [[peritonitis]]
*Large appendiceal abscess
*Patients with an extraluminal [[appendicolith]]

===Interval Appendectomy===
Following drain and antibiotics, an [[Appendectomy|interval appendectomy]] is recommended for patients after six to eight weeks. It may be performed to:
*Prevent recurrence of [[appendicitis]]<ref name="pmid21540609">{{cite journal |vauthors=Ansaloni L, Catena F, Coccolini F, Ercolani G, Gazzotti F, Pasqualini E, Pinna AD |title=Surgery versus conservative antibiotic treatment in acute appendicitis: a systematic review and meta-analysis of randomized controlled trials |journal=Dig Surg |volume=28 |issue=3 |pages=210–21 |year=2011 |pmid=21540609 |doi=10.1159/000324595 |url=}}</ref>
*Exclude [[neoplasms]] as a cause (such as [[Carcinoid|carcinoid,]] [[adenocarcinoma]], [[mucinous cystadenoma]], and [[Cystadenocarcinoma|cystadenocarcinomas]])
Complications of interval appendectomy may include:
*[[Infection|Wound infection]] ([[sepsis]])
*[[Pelvic abscess]]
*[[Aspiration pneumonia]]
Late complications can include:
*[[Adhesions|Abdominal adhesions]]
*Fecal fistula<ref name="pmid22451186">{{cite journal |vauthors=Singal R, Gupta S, Mittal A, Gupta S, Singh M, Dalal AK, Goyal S, Singh B |title=Appendico-cutaneous fistula presenting as a large wound: a rare phenomenon-brief review |journal=Acta Med Indones |volume=44 |issue=1 |pages=53–6 |year=2012 |pmid=22451186 |doi= |url=}}</ref>

The following video demonstrates visualization of appendicular abscess:
{{#ev:youtube|SRMOktFZim0}}

==References==
{{reflist|2}}

Appendicular abscess

2017-05-02T15:14:58Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}
==[[Appendicular abscess overview|Overview]]==

==[[Appendicular abscess historical perspective|Historical Perspective]]==

==[[Appendicular abscess classification|Classification]]==

==[[Appendicular abscess pathophysiology|Pathophysiology]]==

==[[Appendicular abscess causes|Causes]]==

==[[Appendicular abscess differential diagnosis|Differentiating Appendicular abscess from other Diseases]]==

==[[Appendicular abscess epidemiology and demographics|Epidemiology and Demographics]]==

==[[Appendicular abscess risk factors|Risk Factors]]==

==[[Appendicular abscess screening|Screening]]==

==[[Appendicular abscess natural history, complications and prognosis|Natural History, Complications, and Prognosis]]==

==Diagnosis==
[[Appendicular abscess history and symptoms|History and Symptoms]] | [[Appendicular abscess physical examination|Physical Examination]] | [[Appendicular abscess laboratory findings|Laboratory Findings]] | [[Appendicular abscess electrocardiogram|Electrocardiogram]] | [[Appendicular abscess chest x ray|Chest X Ray]] | [[Appendicular abscess CT|CT]] | [[Appendicular abscess MRI|MRI]] | [[Appendicular abscess echocardiography or ultrasound|Echocardiography or Ultrasound]]

==Treatment==
[[Appendicular abscess medical therapy|Medical Therapy]] | [[Appendicular abscess surgery|Surgery]] | [[Appendicular abscess primary prevention|Primary Prevention]] | [[Appendicular abscess secondary prevention|Secondary Prevention]] | [[Appendicular abscess cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Appendicular abscess future or investigational therapies|Future or Investigational Therapies]]

==Case Studies==
[[Appendicular abscess case study one|Case #1]]

[[Category:Infectious disease]]
[[Category:Surgery]]

Appendicular abscess

2017-05-02T15:14:39Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}
==[[Appendicular abscess overview|Overview]]==

==[[Appendicular abscess historical perspective|Historical Perspective]]==

==[[Appendicular abscess classification|Classification]]==

==[[Appendicular abscess pathophysiology|Pathophysiology]]==

==[[Appendicular abscess causes|Causes]]==

==[[Appendicular abscess differential diagnosis|Differentiating Appendicular abscess from other Diseases]]==

==[[Appendicular abscess epidemiology and demographics|Epidemiology and Demographics]]==

==[[Appendicular abscess risk factors|Risk Factors]]==

==[[Appendicular abscess screening|Screening]]==

==[[Appendicular abscess natural history, complications and prognosis|Natural History, Complications and Prognosis]]==

==Diagnosis==
[[Appendicular abscess history and symptoms|History and Symptoms]] | [[Appendicular abscess physical examination|Physical Examination]] | [[Appendicular abscess laboratory findings|Laboratory Findings]] | [[Appendicular abscess electrocardiogram|Electrocardiogram]] | [[Appendicular abscess chest x ray|Chest X Ray]] | [[Appendicular abscess CT|CT]] | [[Appendicular abscess MRI|MRI]] | [[Appendicular abscess echocardiography or ultrasound|Echocardiography or Ultrasound]]

==Treatment==
[[Appendicular abscess medical therapy|Medical Therapy]] | [[Appendicular abscess surgery|Surgery]] | [[Appendicular abscess primary prevention|Primary Prevention]] | [[Appendicular abscess secondary prevention|Secondary Prevention]] | [[Appendicular abscess cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Appendicular abscess future or investigational therapies|Future or Investigational Therapies]]

==Case Studies==
[[Appendicular abscess case study one|Case #1]]

[[Category:Infectious disease]]
[[Category:Surgery]]

Appendicular abscess overview

2017-05-02T15:13:28Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}

==Overview==
Appendicular abscess is defined as a collection of [[pus]] resulting from [[necrosis]] of the [[tissue]] superimposed with [[infection]] in an [[Appendicitis|inflamed appendix]]. It is unusual and rare entity; appendicular abscess is a life-threatening complication of [[acute appendicitis]] (preoperatively) or [[appendectomy]] (postoperatively). It is observed in 2-7% of population presenting with [[appendicitis]]. Complications arise if [[appendicitis]] is not treated promptly. The [[abscess]] develops and is limited by the [[Intestines|inflamed coils of intestine]]. [[Abscess|The abscess]] can spread to [[pelvis]] leading to [[peritonitis]] if the [[abdominal wall]] is ruptured. In most of the patients, the [[Intestine|intestinal coils]] and [[omentum]] in the [[abdominal cavity]] tend to cover the [[Appendicitis|inflamed appendix]] forming an [[appendicular]] [[mass]]. <ref>{{cite book | last = Williams | first = Norman | title = Bailey & Love's short practice of surgery | publisher = CRC Press | location = Boca Raton, FLa | year = 2013 | isbn = 978-1444121285 }}</ref>

==Historical Perspective==
[[Appendicitis]] was first described by Reginald J. Fitz of Harvard University in 1886. He also coined the term [[appendix]]. Since then, the [[appendectomy]] has become one of the most common surgical procedures. The [[laparoscopic]] [[appendectomy]] was invented in the 1980s, and has led to reduced length of hospital stay a decreased risk of [[infection]], and a reduction in post-operative pain.<ref name="pmid6342553">{{cite journal |author=Williams GR |title=Presidential Address: a history of appendicitis. With anecdotes illustrating its importance |journal=[[Annals of Surgery]] |volume=197 |issue=5 |pages=495–506|pmid=6342553 |pmc=1353017 |doi= |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0003-4932&volume=197&issue=5&spage=495 |accessdate=2012-08-09}}</ref>
<ref name="book1">McCarty, Arthur C. "History of Appendicitis Vermiformis Its diseases and treatment." The Innominate Society http://www.innominatesociety.com/Articles/History%20of%20Appendicitis.htm (1927). APA</ref><ref name="book1">McCarty, Arthur C. "History of Appendicitis Vermiformis Its diseases and treatment." The Innominate Society http://www.innominatesociety.com/Articles/History%20of%20Appendicitis.htm (1927). APA</ref>

==Classification==
There is no classification system established for appendicular abscess.

==Pathophysiology==
An [[appendicular]] [[abscess]] is a complication of [[Appendicitis|acute appendicitis]]. It is resulted due to the invasion of the appendix by [[bacteria]] following an [[obstruction]]. The [[appendix]] exists at the junction of the [[Intestine|small and large intestine]] and is a natural habitat of wide variety of bacteria. It is, therefore, prone to develop complications when blocked. Coupled with an [[infection]], [[acute appendicitis]] can be life threatening. Other serious complications which may develop as a result of neglected [[appendicitis]] or appendicular abscess include [[gangrene]], [[appendicular]] [[Mass|masses]], [[rupture]], and [[Peritonitis|general peritoneal infections]]. Obstruction of the tubular space inside the [[appendix]] is the main inciting event, this initial problem leads to the [[inflammation]] of the appendix, [[obstruction]] of the [[blood vessels]] supplying it, and finally infection. [[Inflammation|Inflammatory mediators]] along with various [[bacterial]] [[toxins]] and [[proteolytic]] [[enzymes]] from the [[neutrophils]] are released, resulting in the formation of an [[abscess]] in the appendix.<ref name="pmid626573">{{cite journal |vauthors=Bradley EL, Isaacs J |title=Appendiceal abscess revisited |journal=Arch Surg |volume=113 |issue=2 |pages=130–2 |year=1978 |pmid=626573 |doi= |url=}}</ref>
<ref> Wangensteen OH, Bowers WF. Significance of the obstructive factor in the genesis of acute appendicitis. Arch Surg 1937;34:496-526 </ref>

==Causes==
Microbiology responsible for [[appendicular]] [[abscess]] includes a mixture of [[aerobic]] and [[anaerobic]] [[organisms]] that are natural habitat of [[gut]]. The most commonly isolated [[aerobic]] organism is ''[[Escherichia coli]]'', and the most commonly observed [[anaerobic]] organism is ''[[Bacteroides fragilis]]''. The type and density of [[aerobic]] and [[anaerobic]] bacteria isolated from appendicular abscesses depends upon the organism that dominates the habitat and degree of obstruction.<ref name="pmid20034345">{{cite journal| author=Solomkin JS, Mazuski JE, Bradley JS, Rodvold KA, Goldstein EJ, Baron EJ et al.| title=Diagnosis and management of complicated intra-abdominal infection in adults and children: guidelines by the Surgical Infection Society and the Infectious Diseases Society of America. | journal=Clin Infect Dis | year= 2010 | volume= 50 | issue= 2 | pages= 133-64 | pmid=20034345 | doi=10.1086/649554 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20034345 }} </ref>

==Differential Diagnosis==
Appendicular abscess should be diagnosed early and treat promptly not only to reduce [[morbidity]] and [[mortality]], but it is also important to differentiate from other abdominal diseases presenting with [[Right lower quadrant abdominal pain resident survival guide|RLQ pain]], [[fever]], [[nausea]], and [[vomiting]] such as [[psoas abscess]], [[cellulitis]], torsion of [[Testicular torsion|testis]] and [[Ovarian torsion|ovaries]], and [[ectopic pregnancy]] as the undrained abscess carries high risk of mortality.

==Risk Factors==
Identifying risk factors that predict the likelihood of complications of [[appendicitis]] is a crucial step in managing [[appendicular]] [[abscess]]. Appendicitis is most common risk factor of developing abscess; it is more common among people in the age group of 10 to 30 years old. Appendicitis is a medical emergency that requires proper attention, especially more than any other [[abdominal]] causes if symptoms are not conclusive.

==Screening==
According to the Guidelines by the Surgical Infection Society and the Infectious Diseases Society of America, there is insufficient evidence to recommend routine screening for appendicular abscess.

==Natural History, Complications, and Prognosis==
Without treatment, the patient will likely develop symptoms of diffuse [[abdominal]] [[pain]], which is different from typical [[appendicitis]] [[pain]], starting centrally (in the periumbilical region) before localizing to the [[right iliac fossa]] in the right lower quadrant of the [[abdomen]]. During the final stage of the untreated [[disease]] process, the [[appendix]] will rupture, and this may eventually lead to death if [[peritonitis]] develops. Complications that can develop as a result of the untreated [[appendicular]] [[abscess]] include:[[septicemia]], [[rupture]], [[peritonitis]], [[hemorrhage]] and death. Prognosis of the abscess is good with [[antibiotics]] and [[percutaneous]] drain and resolves without the need for [[Appendectomy|appendectomy,]] but it is recommended to follow and appendicular abscess by [[Appendectomy|interval appendectomy]] after 8-12 weeks to prevent recurrence.

==Diagnosis==
===History and Symptoms===
The key to an efficient and accurate diagnosis is a detailed and thorough history. The onset, location, radiation, and duration of [[pain]], aggravating or relieving factors, severity of pain (constant or intermittent), characteristics of the pain should be obtained in helping out the cause of [[abdominal pain]]. Symptoms of appendicular abscess are mostly atypical compared to [[appendicitis]] and include include [[Fever|high grade fever]], constant pain in the [[right iliac fossa]], [[Diarrhea|prolonged diarrhea]] associated with [[nausea and vomiting]] and increased [[micturition]] and [[tenesmus]].

===Physical Examination===
Physical examinations mostly focus on [[abdominal]] findings. The patient may appear toxic with [[diffuse]] [[abdominal]] [[pain]] and [[Fever|high grade fever]] and [[tachycardia]]. Even minimal pressure on the [[abdomen]] can elicit a marked response from the patient due to pain. Typical signs of [[appendicitis]] may not be elicited.

===Laboratory Findings===
Hematologic parameters suggestive of [[infection]]-like [[leukocytosis]], [[anemia]], [[Thrombosis|abnormal platelet counts]], and [[Abnormal liver function test|abnormal liver function]] frequently are present in patients with appendicular abscess. Patients who are debilitated or elderly often fail to mount reactive [[leukocytosis]] or [[fever]]. [[Blood cultures]] indicating persistent polymicrobial [[bacteremia]] strongly implicate the presence of an abscess. Common [[electrolyte]] and [[Biomarker|bio-marker]] indicators of appendicitis include [[leukocytosis]] and a shift to the left in the segmented [[neutrophils]].

===Abdominal X-Ray===
[[Abdominal X-ray|Plain abdominal radiography]] is not the most useful tool in making a diagnosis of [[appendicular]] [[abscess]].

===Ultrasound===
In general, whenever available, CT scans are preferred over ultrasounds for diagnosing appendicular abscess. Ultrasound imaging presents the least amount of radiation and is therefore the investigation of choice for young patients. Findings include [[fluid]] collection (hypoechoic) in the appendicular region which may be well circumscribed with dilated [[Appendix Normal|appendicular wall]].

===Abdominal CT===
CT scans are the diagnostic test of choice for detecting appendicular abscess. They can provide critical information regarding the size of the [[abscess]]. CT scans are preferred over ultrasounds for the detection of abscess but is contraindicated in children due to risk of exposure. Findings include Appendiceal wall thickening (wall ≥ 3mm), appendiceal wall hyperenhancement, mural stratification of the appendiceal wall.

===MRI===
Magnetic resonance imaging (MRI) has become the common technique for diagnosing abscess in children and pregnant patients. On an MRI, a periappendiceal stranding appears as an increased fluid signal on the T2 weighted sequence.

===Ultrasound===
Findings of appendicular abscess on ultrasound include fluid collection in the appendicular region.

==Treatment==
===Medical Therapy===
No universal standard treatment exists for appendicitis complicated by abscess. The mainstay of treatment includes abscess drainage along with empiric [[antibiotics]]. [[Antibiotics]] should be started immediately once the diagnosis of [[abscess]] is made. The duration of treatment with intravenous [[antibiotics]] ranges from 5 to 10 days, until [[fever]] resolves. [[Monotherapy]] with a beta-lactam/beta-lactamase inhibitor is the preferred choice of drugs. Combination third generation [[cephalosporins]] plus [[metronidazole]] is also employed. [[Percutaneous|Percutaneous drainage]] can be performed under ultrasound or CT guidance, using either the [[Seldinger technique|Seldinger]] or trocar technique. When an abscess is deep in the [[pelvis]], depending on the specific location of the [[fluid]] collection, access may be obtained via transgluteal, transvaginal, or transrectal approaches.

===Surgery===
Following drain and [[antibiotics]] an [[Appendectomy|interval appendectomy]] is recommended for patients after six to eight weeks, it is done to prevent recurrence of [[appendicitis]] and to exclude [[neoplasms]] as a cause (such as [[carcinoid]], [[adenocarcinoma]], [[mucinous cystadenoma]], and [[Cystadenocarcinoma|cystadenocarcinom<nowiki/>as]]). The surgical approach can be eithe[[Laparoscopic surgery|r laparoscopic]] or open ([[Laparotomy|laparotom]]<nowiki/>y)

===Prevention===
There are no primary preventive measures available for appendicular abscess. Secondary prevention strategies following appendicular abscess include treatment of appendicitis in order to prevent significant morbidity.

==References==
{{reflist|2}}

Appendicular abscess laboratory findings

2017-05-02T15:10:20Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}

==Overview==
Hematologic parameters suggestive of [[infection]]-like [[leukocytosis]], [[anemia]], [[Thrombosis|abnormal platelet counts]], and [[Abnormal liver function test|abnormal liver function]] frequently are present in patients with appendicular abscess. Patients who are debilitated or elderly often fail to mount reactive [[leukocytosis]] or [[fever]]. [[Blood cultures]] indicating persistent polymicrobial [[bacteremia]] strongly implicate the presence of an abscess. Common [[electrolyte]] and [[Biomarker|bio-marker]] indicators of appendicitis include [[leukocytosis]] and a shift to the left in the segmented [[neutrophils]].

==Laboratory findings==
====Blood Tests====
'''CBC with differential:'''
*[[Leukocytosis]] (range between 10,500 to 35,00O/mm3)
*[[Anemia]]
*[[Thrombocytopaenia|Abnormal platelet counts]]
*[[Abnormal liver function test|Abnormal liver function]]
'''Blood culture:'''
*Low sensitivity in diagnosing the causative organism in [[appendicular]] [[abscess]] as it shows positivity in few cases, but it also helps to distinguish abscesses from sterile abscess from infected and provide guidance for selection of [[antibiotics]].

==References==
{{reflist|2}}

Appendicular abscess risk factors

2017-05-02T15:05:28Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}

==Overview==
Identifying risk factors that predict the likelihood of complications of [[appendicitis]] is a crucial step in managing [[appendicular]] [[abscess]]. Appendicitis is most common risk factor of developing abscess; it is more common among people in the age group of 10 to 30 years old. Appendicitis is a medical emergency that requires proper attention, especially more than any other [[abdominal]] causes if symptoms are not conclusive.

==Risk Factors==
Appendicitis is the major preinciting event that results in abscess if left untreated.
Common risk factors for adults include:<ref name="pmid12832966">{{cite journal |vauthors=Margenthaler JA, Longo WE, Virgo KS, Johnson FE, Oprian CA, Henderson WG, Daley J, Khuri SF |title=Risk factors for adverse outcomes after the surgical treatment of appendicitis in adults |journal=Ann. Surg. |volume=238 |issue=1 |pages=59–66 |year=2003 |pmid=12832966 |pmc=1422654 |doi=10.1097/01.SLA.0000074961.50020.f8 |url=}}</ref><ref name="urlAppendicitis - The University of Chicago Medicine">{{cite web |url=http://www.uchospitals.edu/online-library/content=P00815 |title=Appendicitis - The University of Chicago Medicine |format= |work= |accessdate=November 30, 2015}}</ref>
*[[Diabetes]]
*A family history of appendicitis increases the child's risk, especially in males
*Male children with [[cystic fibrosis]]

==References==
{{reflist|2}}

Appendicular abscess secondary prevention

2017-05-02T14:55:19Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}

==Overview==
Secondary prevention strategies following appendicular abscess include treatment of appendicitis in order to prevent significant morbidity.

==Secondary Prevention==
[[Peritonitis]] develops from the rupturing of the [[appendix]] and can lead to death is left untreated. Acute appendicitis that is evaluated and treated early with an [[appendectomy]] generally leads to no further complications and a patient's full recovery.

==References==
{{reflist|2}}

Appendicular abscess primary prevention

2017-05-02T14:52:29Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}} {{ADG}}

==Overview==
There are no primary preventive measures available for appendicular abscess.

==Primary Prevention==
There are no primary preventive measures available for appendicular abscess. Reducing the risk of appendicitis, can help in the first place. Following a diet that includes fresh vegetables and fruit may lower the risk of appendicitis.<ref>{{cite book | last = Williams | first = Norman | title = Bailey & Love's short practice of surgery | publisher = CRC Press | location = Boca Raton, FLa | year = 2013 | isbn = 978-1444121285 }}</ref>

==References==
{{reflist|2}}

Appendicular abscess surgery

2017-05-02T14:51:19Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}

==Overview==
Following drain and antibiotics, an [[Appendectomy|interval appendectomy]] is recommended for patients after six to eight weeks. The surgical approach can be either laparoscopic or open (laparotomic).

==Surgery==
===Percutaneous drainage===
*Percutaneous drainage can be performed under ultrasound or CT guidance, using either the [[Seldinger technique|Seldinger]] or [[trocar]] technique.<ref name="pmid14767853">{{cite journal |vauthors=Hogan MJ |title=Appendiceal abscess drainage |journal=Tech Vasc Interv Radiol |volume=6 |issue=4 |pages=205–14 |year=2003 |pmid=14767853 |doi= |url=}}</ref>
*Ultrasound is limited if the [[abscess]] is small, obscured by other structures, or if precise placement is required because of nearby [[vessels]] or [[organs]]. In these cases, CT is the optimal imaging modality.<ref name="pmid11232683">{{cite journal |vauthors=Gress F, Schmitt C, Sherman S, Ciaccia D, Ikenberry S, Lehman G |title=Endoscopic ultrasound-guided celiac plexus block for managing abdominal pain associated with chronic pancreatitis: a prospective single center experience |journal=Am. J. Gastroenterol. |volume=96 |issue=2 |pages=409–16 |year=2001 |pmid=11232683 |doi=10.1111/j.1572-0241.2001.03551.x |url=}}</ref>
*When an [[abscess]] is deep in the [[pelvis]], depending on the specific location of the [[fluid]] collection, access may be obtained via transgluteal, transvaginal, or transrectal approaches.<ref name="urlRetroperitoneal Perforation of the Appendix Presenting as a Right Thigh Abscess">{{cite web |url=http://dx.doi.org/10.1155/2015/707191 |title=Retroperitoneal Perforation of the Appendix Presenting as a Right Thigh Abscess |format= |work= |accessdate=}}</ref>
*If the [[fluid]] collection is [[sterile]], a transgluteal approach is preferred because it allows for sterile technique.<ref name="pmid11232683">{{cite journal |vauthors=Gress F, Schmitt C, Sherman S, Ciaccia D, Ikenberry S, Lehman G |title=Endoscopic ultrasound-guided celiac plexus block for managing abdominal pain associated with chronic pancreatitis: a prospective single center experience |journal=Am. J. Gastroenterol. |volume=96 |issue=2 |pages=409–16 |year=2001 |pmid=11232683 |doi=10.1111/j.1572-0241.2001.03551.x |url=}}</ref>
*Depending on the location of abscess, patient is placed in prone or supine position on the CT table.
*Localization scan using CT allows in selecting a safe window of access into the collection.
*A coaxial micropuncture introducer set is advanced into the abscess under CT guidance.
*An Amplatz guidewire is advanced through the sheath and coiled within the abscess.
*After serial dilatation of the tract with a dilator, an pigtail drain is advanced over the guidewire and deployed.

===Emergency appendectomy===
Indications:
*When patients present with life-threatening signs of [[peritonitis]]
*Large appendiceal abscess
*Patients with an extraluminal [[appendicolith]]

===Interval Appendectomy===
Following drain and antibiotics, an [[Appendectomy|interval appendectomy]] is recommended for patients after six to eight weeks. It may be performed to:
*Prevent recurrence of [[appendicitis]]<ref name="pmid21540609">{{cite journal |vauthors=Ansaloni L, Catena F, Coccolini F, Ercolani G, Gazzotti F, Pasqualini E, Pinna AD |title=Surgery versus conservative antibiotic treatment in acute appendicitis: a systematic review and meta-analysis of randomized controlled trials |journal=Dig Surg |volume=28 |issue=3 |pages=210–21 |year=2011 |pmid=21540609 |doi=10.1159/000324595 |url=}}</ref>
*Exclude [[neoplasms]] as a cause (such as [[Carcinoid|carcinoid,]] [[adenocarcinoma]], [[mucinous cystadenoma]], and [[Cystadenocarcinoma|cystadenocarcinomas]])
Complications of interval appendectomy may include:
*[[Infection|Wound Infection]] ([[sepsis]])
*[[Pelvic abscess]]
*[[Aspiration pneumonia]]
Late complications can include:
*[[Adhesions|Abdominal adhesions]]
*Fecal fistula<ref name="pmid22451186">{{cite journal |vauthors=Singal R, Gupta S, Mittal A, Gupta S, Singh M, Dalal AK, Goyal S, Singh B |title=Appendico-cutaneous fistula presenting as a large wound: a rare phenomenon-brief review |journal=Acta Med Indones |volume=44 |issue=1 |pages=53–6 |year=2012 |pmid=22451186 |doi= |url=}}</ref>

The following video demonstrates visualization of appendicular abscess:
{{#ev:youtube|SRMOktFZim0}}

==References==
{{reflist|2}}

Appendicular abscess surgery

2017-05-02T14:45:48Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}

==Overview==
Following drain and antibiotics, an [[Appendectomy|interval appendectomy]] is recommended for patients after six to eight weeks. The surgical approach can be either laparoscopic or open (laparotomic).

==Surgery==
===Percutaneous drainage===
*Percutaneous drainage can be performed under ultrasound or CT guidance, using either the [[Seldinger technique|Seldinger]] or [[trocar]] technique.<ref name="pmid14767853">{{cite journal |vauthors=Hogan MJ |title=Appendiceal abscess drainage |journal=Tech Vasc Interv Radiol |volume=6 |issue=4 |pages=205–14 |year=2003 |pmid=14767853 |doi= |url=}}</ref>
*Ultrasound is limited if the [[abscess]] is small, obscured by other structures, or if precise placement is required because of nearby [[vessels]] or [[organs]]. In these cases, CT is the optimal imaging modality.<ref name="pmid11232683">{{cite journal |vauthors=Gress F, Schmitt C, Sherman S, Ciaccia D, Ikenberry S, Lehman G |title=Endoscopic ultrasound-guided celiac plexus block for managing abdominal pain associated with chronic pancreatitis: a prospective single center experience |journal=Am. J. Gastroenterol. |volume=96 |issue=2 |pages=409–16 |year=2001 |pmid=11232683 |doi=10.1111/j.1572-0241.2001.03551.x |url=}}</ref>
*When an [[abscess]] is deep in the [[pelvis]], depending on the specific location of the [[fluid]] collection, access may be obtained via transgluteal, transvaginal, or transrectal approaches.<ref name="urlRetroperitoneal Perforation of the Appendix Presenting as a Right Thigh Abscess">{{cite web |url=http://dx.doi.org/10.1155/2015/707191 |title=Retroperitoneal Perforation of the Appendix Presenting as a Right Thigh Abscess |format= |work= |accessdate=}}</ref>
*If the [[fluid]] collection is [[sterile]], a transgluteal approach is preferred because it allows for sterile technique.<ref name="pmid11232683">{{cite journal |vauthors=Gress F, Schmitt C, Sherman S, Ciaccia D, Ikenberry S, Lehman G |title=Endoscopic ultrasound-guided celiac plexus block for managing abdominal pain associated with chronic pancreatitis: a prospective single center experience |journal=Am. J. Gastroenterol. |volume=96 |issue=2 |pages=409–16 |year=2001 |pmid=11232683 |doi=10.1111/j.1572-0241.2001.03551.x |url=}}</ref>
*Depending on the location of abscess, patient is placed in prone or supine position on the CT table.
*Localization scan using CT allows in selecting a safe window of access into the collection.
*A coaxial micropuncture introducer set is advanced into the abscess under CT guidance.
*An Amplatz guidewire is advanced through the sheath and coiled within the abscess.
*After serial dilatation of the tract with a dilator, an pigtail drain is advanced over the guidewire and deployed.
===Emergency appendectomy===
Indications:
*When patients present with life-threatening signs of [[peritonitis]]
*Large appendiceal abscess
*In patients with an extraluminal [[appendicolith]]

===Interval Appendectomy===
Following drain and antibiotics, an [[Appendectomy|interval appendectomy]] is recommended for patients after six to eight weeks. It may be performed to:
*Prevent recurrence of [[Appendicitis|appendicitis.]]<ref name="pmid21540609">{{cite journal |vauthors=Ansaloni L, Catena F, Coccolini F, Ercolani G, Gazzotti F, Pasqualini E, Pinna AD |title=Surgery versus conservative antibiotic treatment in acute appendicitis: a systematic review and meta-analysis of randomized controlled trials |journal=Dig Surg |volume=28 |issue=3 |pages=210–21 |year=2011 |pmid=21540609 |doi=10.1159/000324595 |url=}}</ref>
*Exclude [[neoplasms]] as a cause (such as [[Carcinoid|carcinoid,]] [[adenocarcinoma]], [[mucinous cystadenoma]], and [[Cystadenocarcinoma|cystadenocarcinomas]])
Complications of interval appendectomy may include:
*[[Infection|Wound Infection]] ([[sepsis]])
*[[Pelvic abscess]]
*[[Aspiration pneumonia]]
Late complications can include:
*[[Adhesions|Abdominal adhesions]]
*Fecal fistula<ref name="pmid22451186">{{cite journal |vauthors=Singal R, Gupta S, Mittal A, Gupta S, Singh M, Dalal AK, Goyal S, Singh B |title=Appendico-cutaneous fistula presenting as a large wound: a rare phenomenon-brief review |journal=Acta Med Indones |volume=44 |issue=1 |pages=53–6 |year=2012 |pmid=22451186 |doi= |url=}}</ref>

The following video demonstrates visualization of appendicular abscess:
{{#ev:youtube|SRMOktFZim0}}

==References==
{{reflist|2}}

Appendicular abscess medical therapy

2017-05-02T14:35:47Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}

==Overview==
No universal standard treatment exists for [[appendicitis]] complicated by [[abscess]]. The mainstay of treatment includes abscess drainage along with empiric [[antibiotics]]. It resolves most of the abscess. Once the abscess is resolved, it is followed by [[appendectomy]] 8-12 weeks after the [[pharmacological]] treatment.

==Treatment==
The preferred treatment includes non-operative management such as drainage and broad spectrum IV antibiotics along with [[IV fluids]] followed by surgery which includes interval [[laparoscopic]] [[appendectomy]]. It has proved to have a high success rates up to 97% and low incidences of complications.<ref name="pmid12037755">{{cite journal |vauthors=Samuel M, Hosie G, Holmes K |title=Prospective evaluation of nonsurgical versus surgical management of appendiceal mass |journal=J. Pediatr. Surg. |volume=37 |issue=6 |pages=882–6 |year=2002 |pmid=12037755 |doi= |url=}}</ref><ref name="pmid16175691">{{cite journal |vauthors=Kaminski A, Liu IL, Applebaum H, Lee SL, Haigh PI |title=Routine interval appendectomy is not justified after initial nonoperative treatment of acute appendicitis |journal=Arch Surg |volume=140 |issue=9 |pages=897–901 |year=2005 |pmid=16175691 |doi= |url=}}</ref><ref name="pmid21540609">{{cite journal |vauthors=Ansaloni L, Catena F, Coccolini F, Ercolani G, Gazzotti F, Pasqualini E, Pinna AD |title=Surgery versus conservative antibiotic treatment in acute appendicitis: a systematic review and meta-analysis of randomized controlled trials |journal=Dig Surg |volume=28 |issue=3 |pages=210–21 |year=2011 |pmid=21540609 |doi=10.1159/000324595 |url=}}</ref><ref name="pmid17999120">{{cite journal |vauthors=Meshikhes AW |title=Management of appendiceal mass: controversial issues revisited |journal=J. Gastrointest. Surg. |volume=12 |issue=4 |pages=767–75 |year=2008 |pmid=17999120 |doi=10.1007/s11605-007-0399-1 |url=}}</ref>
===Medical Therapy===
[[Antibiotics]] should be started immediately once the diagnosis of abscess is made. Preoperative antibiotics have been associated with lower rates of [[wound]] and [[Intra-abdominal infection|intra-abdominal infections]].<ref name="pmid20034345">{{cite journal| author=Solomkin JS, Mazuski JE, Bradley JS, Rodvold KA, Goldstein EJ, Baron EJ et al.| title=Diagnosis and management of complicated intra-abdominal infection in adults and children: guidelines by the Surgical Infection Society and the Infectious Diseases Society of America. | journal=Clin Infect Dis | year= 2010 | volume= 50 | issue= 2 | pages= 133-64 | pmid=20034345 | doi=10.1086/649554 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20034345 }} </ref><ref name="SartelliViale2013">{{cite journal|last1=Sartelli|first1=Massimo|last2=Viale|first2=Pierluigi|last3=Catena|first3=Fausto|last4=Ansaloni|first4=Luca|last5=Moore|first5=Ernest|last6=Malangoni|first6=Mark|last7=Moore|first7=Frederick A|last8=Velmahos|first8=George|last9=Coimbra|first9=Raul|last10=Ivatury|first10=Rao|last11=Peitzman|first11=Andrew|last12=Koike|first12=Kaoru|last13=Leppaniemi|first13=Ari|last14=Biffl|first14=Walter|last15=Burlew|first15=Clay Cothren|last16=Balogh|first16=Zsolt J|last17=Boffard|first17=Ken|last18=Bendinelli|first18=Cino|last19=Gupta|first19=Sanjay|last20=Kluger|first20=Yoram|last21=Agresta|first21=Ferdinando|last22=Di Saverio|first22=Salomone|last23=Wani|first23=Imtiaz|last24=Escalona|first24=Alex|last25=Ordonez|first25=Carlos|last26=Fraga|first26=Gustavo P|last27=Junior|first27=Gerson Alves Pereira|last28=Bala|first28=Miklosh|last29=Cui|first29=Yunfeng|last30=Marwah|first30=Sanjay|last31=Sakakushev|first31=Boris|last32=Kong|first32=Victor|last33=Naidoo|first33=Noel|last34=Ahmed|first34=Adamu|last35=Abbas|first35=Ashraf|last36=Guercioni|first36=Gianluca|last37=Vettoretto|first37=Nereo|last38=Díaz-Nieto|first38=Rafael|last39=Gerych|first39=Ihor|last40=Tranà|first40=Cristian|last41=Faro|first41=Mario Paulo|last42=Yuan|first42=Kuo-Ching|last43=Kok|first43=Kenneth Yuh Yen|last44=Mefire|first44=Alain Chichom|last45=Lee|first45=Jae Gil|last46=Hong|first46=Suk-Kyung|last47=Ghnnam|first47=Wagih|last48=Siribumrungwong|first48=Boonying|last49=Sato|first49=Norio|last50=Murata|first50=Kiyoshi|last51=Irahara|first51=Takayuki|last52=Coccolini|first52=Federico|last53=Lohse|first53=Helmut A Segovia|last54=Verni|first54=Alfredo|last55=Shoko|first55=Tomohisa|title=2013 WSES guidelines for management of intra-abdominal infections|journal=World Journal of Emergency Surgery|volume=8|issue=1|year=2013|pages=3|issn=1749-7922|doi=10.1186/1749-7922-8-3}}</ref>
====Duration====
The duration of treatment with intravenous antibiotics ranges from 5 to 10 days, until fever resolves, white blood cell count normalizes, and bowel function returns.<ref name="SartelliViale2013">{{cite journal|last1=Sartelli|first1=Massimo|last2=Viale|first2=Pierluigi|last3=Catena|first3=Fausto|last4=Ansaloni|first4=Luca|last5=Moore|first5=Ernest|last6=Malangoni|first6=Mark|last7=Moore|first7=Frederick A|last8=Velmahos|first8=George|last9=Coimbra|first9=Raul|last10=Ivatury|first10=Rao|last11=Peitzman|first11=Andrew|last12=Koike|first12=Kaoru|last13=Leppaniemi|first13=Ari|last14=Biffl|first14=Walter|last15=Burlew|first15=Clay Cothren|last16=Balogh|first16=Zsolt J|last17=Boffard|first17=Ken|last18=Bendinelli|first18=Cino|last19=Gupta|first19=Sanjay|last20=Kluger|first20=Yoram|last21=Agresta|first21=Ferdinando|last22=Di Saverio|first22=Salomone|last23=Wani|first23=Imtiaz|last24=Escalona|first24=Alex|last25=Ordonez|first25=Carlos|last26=Fraga|first26=Gustavo P|last27=Junior|first27=Gerson Alves Pereira|last28=Bala|first28=Miklosh|last29=Cui|first29=Yunfeng|last30=Marwah|first30=Sanjay|last31=Sakakushev|first31=Boris|last32=Kong|first32=Victor|last33=Naidoo|first33=Noel|last34=Ahmed|first34=Adamu|last35=Abbas|first35=Ashraf|last36=Guercioni|first36=Gianluca|last37=Vettoretto|first37=Nereo|last38=Díaz-Nieto|first38=Rafael|last39=Gerych|first39=Ihor|last40=Tranà|first40=Cristian|last41=Faro|first41=Mario Paulo|last42=Yuan|first42=Kuo-Ching|last43=Kok|first43=Kenneth Yuh Yen|last44=Mefire|first44=Alain Chichom|last45=Lee|first45=Jae Gil|last46=Hong|first46=Suk-Kyung|last47=Ghnnam|first47=Wagih|last48=Siribumrungwong|first48=Boonying|last49=Sato|first49=Norio|last50=Murata|first50=Kiyoshi|last51=Irahara|first51=Takayuki|last52=Coccolini|first52=Federico|last53=Lohse|first53=Helmut A Segovia|last54=Verni|first54=Alfredo|last55=Shoko|first55=Tomohisa|title=2013 WSES guidelines for management of intra-abdominal infections|journal=World Journal of Emergency Surgery|volume=8|issue=1|year=2013|pages=3|issn=1749-7922|doi=10.1186/1749-7922-8-3}}</ref>
====Empiric therapy====
Monotherapy with a beta-lactam/beta-lactamase inhibitor:
*Preferred regimen (1):[[Ampicillin-Sulbactam|Ampicillin-sulbactam]] 3 g IV q6h
*Preferred regimen (2):[[Ticarcillin-Clavulanate|Ticarcillin-clavulanate]] 3 g IV q4h
*Preferred regimen (3):[[Piperacillin-tazobactam]] 3 g or 4.5 g IV q6h
Combination third generation cephalosporins PLUS metronidazole
*Preferred regimen (1): [[Ceftriaxone]] 1 g IV q24h {{and}} [[Metronidazole]] 500 mg IV q8h {{or}} 1500 mg q24h.
*Preferred regimen (2): [[Cefazolin]] 1–2 g IV q8h {{and}} [[Metronidazole]] 500 mg IV q8–12 h {{or}} 1500 mg q24h
*Preferred regimen (3): [[Cefuroxime]] 1.5 g IV q8h {{and}} [[Metronidazole]] 500 mg IV q8–12 h {{or}} 1500 mg q24h
*Preferred regimen (4): [[Cefotaxime]] 1–2 g IV q6–8 h {{and}} [[Metronidazole]] 500 mg IV q8–12 h {{or}} 1500 mg q24h
====Alternative empiric regimens====
Combination fluoroquinolone PLUS metronidazole:
*Preferred regimen (1): [[Ciprofloxacin]] 400 mg IV q12h {{and}} [[Metronidazole]] 500 mg IV q8–12 h {{or}} 1500 mg q24h
*Preferred regimen (2): [[Levofloxacin]] 750 mg IV q24h {{and}} [[Metronidazole]] 500 mg IV q8–12 h {{or}} 1500 mg q24h
Monotherapy with a carbapenem
*Preferred regimen (1): [[Imipenem-Cilastatin|Imipenem-cilastatin]] 500 mg IV q6h {{or}} 1 g q8h
*Preferred regimen (2): [[Meropenem]] 1 g IV q8h
*Preferred regimen (3): [[Doripenem]] 500 mg IV q8h
*Preferred regimen (4): [[Ertapenem]] 1 g IV q24h
==References==
{{reflist|2}}

Appendicular abscess echocardiography or ultrasound

2017-05-02T14:31:54Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}} {{ADG}}

==Overview==
Findings of appendicular abscess on ultrasound include fluid collection in the appendicular region.

==Ultrasound==
Findings of an appendicular abscess include:
*Fluid collection (hypoechoic) in the appendicular region
*Well circumscribed and rounded or ill-defined and irregular in appearance appendix may be visualized within the mass.
[[File:Appendicular_abscess_USG.gif.gif|594x594px|center|frame|Ultrasound showing an area of high echogenicity measuring 5.2 X 6.7 cm in relation to the right hepatic lobe with echogenic rim.]] 

The following video demonstrates appendicular abscess on ultrasound:
{{#ev:youtube|4qOYL0y3hPY}}

==References==
{{reflist|2}}

Appendicular abscess echocardiography or ultrasound

2017-05-02T14:24:07Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}};{{AE}}{{ADG}}

==Overview==
Findings of appendicular abscess on ultrasound include fluid collection in the appendicular region.

==Ultrasound==
Findings of an appendicular abscess include:
*Fluid collection (hypoechoic) in the appendicular region
*Well circumscribed and rounded or ill-defined and irregular in appearance appendix may be visualized within the mass.
[[File:Appendicular_abscess_USG.gif.gif|frameless|594x594px]] 

US showing an area of high echogenicity measuring 5.2 X 6.7 cm in relation to the right hepatic lobe with echogenic rim. 
{{#ev:youtube|4qOYL0y3hPY}}

==References==
{{reflist|2}}

Appendicular abscess MRI

2017-05-02T14:09:36Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}} {{ADG}}

==Overview==
Magnetic resonance imaging (MRI) has become the common technique for diagnosing appendicitis in children and pregnant patients.

==MRI==
MRI usage has become investigation of choice in the diagnosis of appendicular abscess for children and pregnant patients because of <ref name="rad1">Image courtesy of Radiologypics. [http://radiologypics.com/2014/03/21/perforated-appendicitis-mri/ Radiologypics]</ref>
*Low risk of radiation in children and developing fetus.
*The enlarged [[uterus]] displaces the [[appendix]] during the second and third trimesters of [[pregnancy]], making it difficult to find by ultrasound. An MRI is therefore preferred.
On an MRI, a periappendiceal stranding appears as an increased fluid signal on the T2 weighted sequence (while it is reflected by fat stranding on a CT scan).<ref name="rad1">Image courtesy of Radiologypics. [http://radiologypics.com/2014/03/21/perforated-appendicitis-mri/ Radiologypics]</ref>
[[Image:Perforated-appendicitis-axial-t2-mr.png|center|frame|Axial T2-Weighted MRI demonstrates a dilated fluid filled appendix with periappendiceal fat stranding and two appendicoliths (dark signal within the appendix lumen).<ref name="rad1">Image courtesy of Radiologypics. [http://radiologypics.com/2014/03/21/perforated-appendicitis-mri/ Radiologypics]</ref>]]

==References==
{{reflist|2}}

Appendicular abscess CT

2017-05-02T14:07:36Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}};{{AE}} {{ADG}}

==Overview==
CT scans are the diagnostic test of choice for detecting appendicular abscess. They can provide critical information regarding the size of the [[abscess]]. CT scans are preferred over ultrasounds for the detection of abscess, but is contraindicated in children due to risk of exposure.

==CT==
*CT is significantly more sensitive than ultrasound for the diagnosis of appendicitis.<ref name="pmid14616200">{{cite journal |vauthors=Choi D, Park H, Lee YR, Kook SH, Kim SK, Kwag HJ, Chung EC |title=The most useful findings for diagnosing acute appendicitis on contrast-enhanced helical CT |journal=Acta Radiol |volume=44 |issue=6 |pages=574–82 |year=2003 |pmid=14616200 |doi= |url=}}</ref>
*Appendiceal wall thickening (wall ≥ 3mm) with hyperenhancement and mural stratification can be seen
*[[Fluid]] collection is seen in the [[appendicular]] region sometimes mixed with air.
[[File:Appendicular_abscess_CT_gif.gif|center|frame|CT of abdomen showing an abscess in the retrocaecal location with an adjacent appendicolith with ascending colon being displaced anteriorly.]]

==References==
{{reflist|2}}

Appendicular abscess CT

2017-05-02T14:02:49Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}};{{AE}} {{ADG}}

==Overview==
CT scans are the diagnostic test of choice for detecting appendicular abscess. They can provide critical information regarding the size of the [[abscess]]. CT scans are preferred over ultrasounds for the detection of abscess, but is contraindicated in children due to risk of exposure.

==CT==
*CT is significantly more sensitive than ultrasound for the diagnosis of appendicitis.<ref name="pmid14616200">{{cite journal |vauthors=Choi D, Park H, Lee YR, Kook SH, Kim SK, Kwag HJ, Chung EC |title=The most useful findings for diagnosing acute appendicitis on contrast-enhanced helical CT |journal=Acta Radiol |volume=44 |issue=6 |pages=574–82 |year=2003 |pmid=14616200 |doi= |url=}}</ref>
*Appendiceal wall thickening (wall ≥ 3mm) with hyperenhancement and mural stratification can be seen
*[[Fluid]] collection is seen in the [[appendicular]] region sometimes mixed with air.
[[File:Appendicular_abscess_CT_gif.gif|frameless|center|500x500px|CT of abdomen showing an abscess in the retrocaecal location with an adjacent appendicolith with ascending colon being displaced anteriorly.]] 
CT of abdomen showing an abscess in the retrocaecal location with an adjacent appendicolith with ascending colon being displaced anteriorly.

==References==
{{reflist|2}}

Rheumatic fever chest x ray

2017-05-02T13:44:52Z

Anthony Gallo:

__NOTOC__
{{Rheumatic fever}}
{{CMG}}; {{AE}} {{VK}}; {{AG}}

==Overview==
On chest x-ray, rheumatic fever is characterized by [[cardiomegaly]] and [[pulmonary edema]] secondary to [[heart failure]].<ref name=RADIOP> Acute Rhuematic Fever. Radiopaedia.org (2015). http://radiopaedia.org/cases/acute-rheumatic-fever-chest-radiographic-findings Accessed on October 19, 2015.</ref>

==Chest X Ray==
On chest x-ray, rheumatic fever is characterized by [[cardiomegaly]] secondary to [[carditis]] and valve abnormalities. [[Pulmonary edema]] may be noted if rheumatic fever has progressed to [[cardiac failure]].<ref name=RADIOP> Acute Rhuematic Fever. Radiopaedia.org (2015). http://radiopaedia.org/cases/acute-rheumatic-fever-chest-radiographic-findings Accessed on October 19, 2015.</ref>

The following are a collection of radiological findings demonstrating the presence of rheumatic fever:
<gallery>

Image:Acute_Rheumatic_Fever_X-Ray_1.jpg|Plain chest film demonstrates bilateral increased interstitial markings.<ref name=XRAY> Image courtesy of Dr. David Preston [http://www.radiopaedia.org Radiopaedia] (original file [http://radiopaedia.org/cases/acute-rheumatic-fever-chest-radiographic-findings]). [http://radiopaedia.org/licence Creative Commons BY-SA-NC] </ref>

Image:Acute_Rheumatic_Fever_X-Ray_2.jpg|Patient was intubated and ventilated. A diagnosis of rheumatic heart disease was made. [[Cardiomegaly]] is present.<ref name=XRAY> Image courtesy of Dr. David Preston [http://www.radiopaedia.org Radiopaedia] (original file [http://radiopaedia.org/cases/acute-rheumatic-fever-chest-radiographic-findings]). [http://radiopaedia.org/licence Creative Commons BY-SA-NC] </ref>

Image:Acute_Rheumatic_Fever_X-Ray_3.jpg|Patient went to surgery and had a [[mitral valve|mitral]] and [[aortic valve replacement]].<ref name=XRAY> Image courtesy of Dr. David Preston [http://www.radiopaedia.org Radiopaedia] (original file [http://radiopaedia.org/cases/acute-rheumatic-fever-chest-radiographic-findings]). [http://radiopaedia.org/licence Creative Commons BY-SA-NC] </ref>

</gallery>

==References==
{{Reflist|2}}

Appendicular abscess CT

2017-05-02T13:43:21Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}};{{AE}} {{ADG}}

==Overview==
CT scans are the diagnostic test of choice for detecting appendicular abscess. They can provide critical information regarding the size of the [[abscess]]. CT scans are preferred over ultrasounds for the detection of abscess, but is contraindicated in children due to risk of exposure.

==CT==
*CT is significantly more sensitive than ultrasound for the diagnosis of appendicitis.<ref name="pmid14616200">{{cite journal |vauthors=Choi D, Park H, Lee YR, Kook SH, Kim SK, Kwag HJ, Chung EC |title=The most useful findings for diagnosing acute appendicitis on contrast-enhanced helical CT |journal=Acta Radiol |volume=44 |issue=6 |pages=574–82 |year=2003 |pmid=14616200 |doi= |url=}}</ref>
*Appendiceal wall thickening (wall ≥ 3mm) with hyperenhancement and mural stratification can be seen
*[[Fluid]] collection is seen in the [[appendicular]] region sometimes mixed with air.
[[File:Appendicular_abscess_CT_gif.gif|frameless|center|500x500px]] 
CT of abdomen showing an abscess in the retrocaecal location with an adjacent appendicolith with ascending colon being displaced anteriorly.

==References==
{{reflist|2}}

Appendicular abscess abdominal x ray

2017-05-02T13:40:33Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}

==Overview==
[[Abdominal X-ray|Plain abdominal radiography]] is not the most useful tool in making a diagnosis of appendicular abscess.

==X Ray==
[[Abdominal X-ray|Plain abdominal radiography]] is not the most useful tool in making a diagnosis of appendicular abscess.

==References==
{{Reflist|2}}

Appendicular abscess laboratory findings

2017-05-02T13:37:46Z

Anthony Gallo:

__NOTOC__
{{Appendicular abscess}}
{{CMG}}; {{AE}}{{ADG}}
==Overview==
Hematologic parameters suggestive of [[infection]] like, [[leukocytosis]], [[anemia]], [[Thrombosis|abnormal platelet counts]], and [[Abnormal liver function test|abnormal liver function]] frequently are present in patients with appendicular abscess, although patients who are debilitated or elderly often fail to mount reactive [[leukocytosis]] or [[Fever|feve]]<nowiki/>r. [[Blood cultures]] indicating persistent polymicrobial [[bacteremia]] strongly implicate the presence of an abscess.

==Laboratory findings==
====Blood Tests====
'''CBC with differential:'''
*[[Leukocytosis]] (range between 10,500 to 35,00O/mm3)
*[[Anemia]]
*[[Thrombocytopaenia|Abnormal platelet counts]]
*[[Abnormal liver function test|Abnormal liver function]]
'''Blood culture:'''
*Low sensitivity in diagnosing the causative organism in [[appendicular]] [[abscess]] as it shows positivity in few cases, but it also helps to distinguish abscesses from sterile abscess from infected and provide guidance for selection of [[antibiotics]].

==References==
{{reflist|2}}