Asplenia: Difference between revisions

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==[[Asplenia natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
==[[Asplenia natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
===Natural History===
*If left [[untreated]], [[Patients]] with [[asplenia]] or [[hyposplenia]] are at risk of [[life-threatening]] [[infection]].<ref name="pmid25125944">{{cite journal| author=Kirkineska L, Perifanis V, Vasiliadis T| title=Functional hyposplenism. | journal=Hippokratia | year= 2014 | volume= 18 | issue= 1 | pages= 7-11 | pmid=25125944 | doi= | pmc=4103047 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25125944  }} </ref>
*Patients with [[functional asplenia]] and [[hyposplenia]] who have not [[undergone]] a [[splenectomy]] can present with a [[life-threatening]] [[infection]] [[comparable]] to an [[OPSI]].
*[[Overwhelming post-splenectomy infection]] (OPSI) occurs in [[5%]] of [[patients]] and has a [[mortality rate]] of [[38%–70%]].
*Functional asplenia is most common in [[sickle cell disease]] and [[occurs]] within the [[first 3-5 years]] of [[life]].<ref name="pmid32247651">{{cite journal| author=Long B, Koyfman A, Gottlieb M| title=Complications in the adult asplenic patient: A review for the emergency clinician. | journal=Am J Emerg Med | year= 2021 | volume= 44 | issue=  | pages= 452-457 | pmid=32247651 | doi=10.1016/j.ajem.2020.03.049 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32247651  }} </ref>
===Complications===
'''Common complications of asplenia include'''
*[[overwhelming post-splenectomy infection (OPSI)]]<ref name="pmid25125944">{{cite journal| author=Kirkineska L, Perifanis V, Vasiliadis T| title=Functional hyposplenism. | journal=Hippokratia | year= 2014 | volume= 18 | issue= 1 | pages= 7-11 | pmid=25125944 | doi= | pmc=4103047 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25125944  }} </ref>
*[[Infection]] with [[encapsulated microorganisms]] such as [[Streptococcus pneumonia]], [[Neisseria meningitides]] and [[Haemophilous influenzae]]
*[[Waterhouse-Friedrichsen syndrome]] and [[Purpura fulminans]] <ref name="pmid27583208">{{cite journal| author=Hale AJ, LaSalvia M, Kirby JE, Kimball A, Baden R| title=Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult. | journal=IDCases | year= 2016 | volume= 6 | issue=  | pages= 1-4 | pmid=27583208 | doi=10.1016/j.idcr.2016.08.004 | pmc=4995527 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27583208  }} </ref>
*[[Arterial thrombosis]]: includes [[coronary artery disease]] <ref name="pmid32247651">{{cite journal| author=Long B, Koyfman A, Gottlieb M| title=Complications in the adult asplenic patient: A review for the emergency clinician. | journal=Am J Emerg Med | year= 2021 | volume= 44 | issue=  | pages= 452-457 | pmid=32247651 | doi=10.1016/j.ajem.2020.03.049 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32247651  }} </ref>
*[[Venous thrombosis]]: includes [[deep vein thrombosis]], [[pulmonary embolism]], [[splenic]] and [[portal vein thrombosis]]
*[[Pulmonary hypertension]], [[associated]] with [[right ventricular dysfunction]].
'''Less Common complications of asplenia include'''
*Patients with [[asplenia]] are also at risk for less common [[infections]] due to [[Capnocytophaga]], [[Babesia]], and [[malaria]].<ref name="pmid33275684">{{cite journal| author=Lee GM| title=Preventing infections in children and adults with asplenia. | journal=Hematology Am Soc Hematol Educ Program | year= 2020 | volume= 2020 | issue= 1 | pages= 328-335 | pmid=33275684 | doi=10.1182/hematology.2020000117 | pmc=7727556 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=33275684  }} </ref>
===Prognosis===
*Prognosis of asplenia is [[poor]], if asplenic patients are not diagnosed on time, and do not receive proper [[vaccination]]. These patients are at [[high risk]] of [[infection]]  leads to [[sepsis]], [[septic shock]], and [[death]].<ref name="pmid25125944">{{cite journal| author=Kirkineska L, Perifanis V, Vasiliadis T| title=Functional hyposplenism. | journal=Hippokratia | year= 2014 | volume= 18 | issue= 1 | pages= 7-11 | pmid=25125944 | doi= | pmc=4103047 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25125944  }} </ref>
*Huebner and colleagues, in One case report provides evidence of the [[poor]] [[prognosis]] in [[asplenic]] patients who present with infection despite receiving standard medical care.<ref name="pmid26130882">{{cite journal| author=Huebner ML, Milota KA| title=Asplenia and fever. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 3 | pages= 340-1 | pmid=26130882 | doi=10.1080/08998280.2015.11929267 | pmc=4462215 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26130882  }} </ref>
*In [[Right isomerism]] (Ivemark syndrome) [[Prognosis]] is Poor, 80 % die within first year.<ref name="pmid22470785">{{cite journal| author=Agarwal H, Mittal SK, Kulkarni CD, Verma AK, Srivastava SK| title=Right isomerism with complex cardiac anomalies presenting with dysphagia--a case report. | journal=J Radiol Case Rep | year= 2011 | volume= 5 | issue= 4 | pages= 1-9 | pmid=22470785 | doi=10.3941/jrcr.v5i4.702 | pmc=3303439 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22470785  }} </ref>


==Diagnosis==
==Diagnosis==

Revision as of 13:15, 19 July 2021

Asplenia
ICD-10 D73.0, Q89.0
ICD-9 289.59, 759.01
OMIM 208530 %271400 208540

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief:

Synonyms and keywords:

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Asplenia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Interventions | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Medical Therapy

Emergency Medical Management of suspected sepsis in Asplenic patient

Asplenia can cause sepsis and require immediate management:[1]

Surgery

The mainstay of treatment for asplenia is medical therapy and prevention.[3]

Primary prevention

Vaccination

Antibiotic Prophylaxis

Malaria Prophylaxis

Secondary prevention

Effective measures for the secondary prevention of asplenia include:

Case Studies

Case #1

Template:Hematology Template:Phakomatoses and other congenital malformations not elsewhere classified


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Template:WikiDoc Sources

  1. 1.0 1.1 Salvadori MI, Price VE, Canadian Paediatric Society, Infectious Diseases and Immunization Committee (2014). "Preventing and treating infections in children with asplenia or hyposplenia". Paediatr Child Health. 19 (5): 271–8. PMC 4029242. PMID 24855431.
  2. Rhodes A, Evans LE, Alhazzani W, Levy MM, Antonelli M, Ferrer R; et al. (2017). "Surviving Sepsis Campaign: International Guidelines for Management of Sepsis and Septic Shock: 2016". Intensive Care Med. 43 (3): 304–377. doi:10.1007/s00134-017-4683-6. PMID 28101605.
  3. Waghorn DJ (2001). "Overwhelming infection in asplenic patients: current best practice preventive measures are not being followed". J Clin Pathol. 54 (3): 214–8. doi:10.1136/jcp.54.3.214. PMC 1731383. PMID 11253134.
  4. Huebner ML, Milota KA (2015). "Asplenia and fever". Proc (Bayl Univ Med Cent). 28 (3): 340–1. doi:10.1080/08998280.2015.11929267. PMC 4462215. PMID 26130882.
  5. Quéffélec C, Billet L, Duffau P, Lazaro E, Machelart I, Greib C; et al. (2020). "Prevention of infection in asplenic adult patients by general practitioners in France between 2013 and 2016 : Care for the asplenic patient in general practice". BMC Fam Pract. 21 (1): 163. doi:10.1186/s12875-020-01237-3. PMC 7425533 Check |pmc= value (help). PMID 32787857 Check |pmid= value (help).
  6. Committee to Advise on Tropical Medicine and Travel (CATMAT) (2009). "Canadian recommendations for the prevention and treatment of malaria among international travellers--2009". Can Commun Dis Rep. 35 Suppl 1: 1–82. PMID 19750611.
  7. O'Neill NE, Baker J, Ward R, Johnson C, Taggart L, Sholzberg M (2020). "The development of a quality improvement project to improve infection prevention and management in patients with asplenia or hyposplenia". BMJ Open Qual. 9 (3). doi:10.1136/bmjoq-2019-000770. PMC 7410002 Check |pmc= value (help). PMID 32759171 Check |pmid= value (help).
  8. Lee GM (2020). "Preventing infections in children and adults with asplenia". Hematology Am Soc Hematol Educ Program. 2020 (1): 328–335. doi:10.1182/hematology.2020000117. PMC 7727556 Check |pmc= value (help). PMID 33275684 Check |pmid= value (help).