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| {{familytree/start |summary=Sample 1}}
| | [[Syncope]] is classified into three types: |
| {{familytree | | | | | E04 | | | | |E04=[[Infection]]/[[Inflammation]]}}
| | * [[Cardiac]] |
| {{familytree | | | | | |!| | | | }}
| | * [[Neurogenic]] |
| {{familytree | | | | | E05 | | | | |E05=Increased production of [[IL-1]]/[[IL-6]]/[[TNF-α]]}}
| | * [[Vasovagal syncope|vasovagal]] |
| {{familytree | | | | | |!| | | | }}
| |
| {{familytree | | | | | C05 | | | | |C05=Upregulation of [[hepatic]] serum amyloid A production}}
| |
| {{familytree | | | | | |!| | | | | | }}
| |
| {{familytree | | | | | C04 | | | | |C04=SAA production uptake by [[macrophages]]}}
| |
| {{familytree | | | | | |!| | | | }}
| |
| {{familytree | | | | | D05 | | | | |D05=C-terminal cleavage of SAA}}
| |
| {{familytree | | | | | |!| | | | }}
| |
| {{familytree | | | | | F01 | | | | |F01=β-sheet configuration of SAA}}
| |
| {{familytree | | | | | |!| | | | }}
| |
| {{familytree | | | | | G05 | | | | |G05=Fibril deposition in [[extracellular space]]}}
| |
| {{familytree | | | | | |!| | | | }}
| |
| {{familytree | | | | | H05 | | | | |H05=Binding of [[glycosaminoglycan]], serum amyloid P, and [[lipid]] components}}
| |
| {{familytree | | | | | |!| | | | }}
| |
| {{familytree | | | | | K05 | | | | |K05=Resistant to proteolysis}}
| |
| {|
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| ! colspan="2" style="background:#DCDCDC;" align="center" + |The above algorithm is adopted from International Journal of Nephrology and Renovascular Disease<ref>{{cite journal |vauthors=Rumjon A, Coats T, Javaid MM |title=Review of eprodisate for the treatment of renal disease in AA amyloidosis |journal=Int J Nephrol Renovasc Dis |volume=5 |issue= |pages=37–43 |date=2012 |pmid=22427728 |pmc=3304340 |doi=10.2147/IJNRD.S19165 |url=}}</ref>
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| |}
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| {| class="wikitable"
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| | colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |'''Associated Conditions'''
| |
| |-
| |
| | style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Conditions'''
| |
| |'''Examples'''
| |
| |-
| |
| | style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Chronic infections'''
| |
| |
| |
| *[[Tuberculosis]]
| |
| *[[Leprosy]]
| |
| *Whipple Disease
| |
| *[[Osteomyelitis]]
| |
| *[[Chronic pyelonephritis]]
| |
| *[[Subacute bacterial endocarditis]]
| |
| *Chronic cutaneous ulcers
| |
| |-
| |
| | style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Monogenic [[Periodic fever syndrome|periodic fever syndromes]]'''
| |
| |<br />
| |
| *[[TNF receptor associated periodic syndrome|TNF Receptor associated periodic syndrome]] ([[TRAPS]]) | |
| *[[CAPS|Cryopyrin-associated periodic fever syndrome]]
| |
| *[[Mevalonate kinase deficiency]]
| |
| *[[Familial mediterranean fever]]
| |
| |-
| |
| | style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Conditions predisposing to recurrent infections'''
| |
| |
| |
| *[[Cystic fibrosis]]
| |
| *[[Bronchiectasis]]
| |
| *[[Kartagener syndrome]]
| |
| *[[Epidermolysis bullosa]]
| |
| *[[IV drug abusers|Injected drug abuse]]
| |
| *[[Jejuno-ileal bypass]]
| |
| *[[Paraplegia]]
| |
| *[[Sickle cell anemia]]
| |
| *[[Immunodeficiency]]
| |
| *[[Common variable immunodeficiency]]
| |
| *[[Cyclic neutropenia]]
| |
| *[[Hyperimmunoglobulin M syndrome]]
| |
| *[[Hypogammaglobulinemia]]
| |
| *[[X-linked agammaglobulinemia|Sex-linked agammaglobulinemia]]
| |
| *[[Human Immunodeficiency Virus|Human immunodeficiency virus]]/[[AIDS]]
| |
| |-
| |
| | style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Neoplasia'''
| |
| |
| |
| *[[Adenocarcinoma]]
| |
| *[[Basal cell carcinoma]]
| |
| *[[Carcinoid tumors|Carcinoid tumor]]
| |
| *[[Castleman disease]]
| |
| *[[Gastrointestinal stromal tumor]]
| |
| *[[Hairy cell leukemia]]
| |
| *[[Hepatic adenoma]]
| |
| *[[Hodgkin disease]]
| |
| *[[Mesothelioma]]
| |
| *[[Renal cell carcinoma]]
| |
| *[[Sarcoma]]
| |
| |-
| |
| | style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Inflammatory Arthritis'''
| |
| |
| |
| *Adult-onset Still disease
| |
| *[[Ankylosing spondylitis]]
| |
| *[[Juvenile idiopathic arthritis]]
| |
| *[[Psoriatic arthropathy]]
| |
| *[[Reiter's syndrome|Reiter syndrome]]
| |
| *[[Rheumatoid arthritis]]
| |
| *[[Gout]]
| |
| |-
| |
| | style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Systemic [[Vasculitis]]'''
| |
| |
| |
| *Antineutrophil cytoplasmic antibody-associated vasculitis
| |
| *[[Behcet disease]]
| |
| *[[Giant cell arteritis]]
| |
| *[[Polyarteritis nodosa]]
| |
| *[[Polymyalgia rheumatica]]
| |
| *[[Systemic lupus erythematosus]]
| |
| *[[Takayasu arteritis]]
| |
| *[[Inflammatory Bowel Disease]]
| |
| *[[Ulcerative colitis]]
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| *[[Crohn disease]]
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| |-
| |
| | style="background: #DCDCDC; padding: 5px; text-align: left;" |'''Others'''
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| *[[Atrial myxoma]]
| |
| *Inflammatory abdominal aortic aneurism
| |
| *[[Retroperitoneal fibrosis]]
| |
| *[[SAPHO syndrome|SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome]]
| |
| *[[Sarcoidosis]]
| |
| *[[Sinus histiocytosis with massive lymphadenopathy]]
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| |-
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| |+
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| |-
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| |+
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| |-
| |
| |-
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| |}
| |
| {| class="wikitable"
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Organ System Involvement}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Differential Diagnosis}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Causes}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Clinical Features}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Laboratory Findings}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Gold Standard Test}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Therapy}}
| |
| |-
| |
| | rowspan="9" |'''Nephrotic Syndrome and Renal Failure'''
| |
| |'''[[Secondary (AA) Amyloidosis]]'''
| |
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| * Chronic [[inflammatory disorders]]
| |
| * Chronic [[infections]]
| |
| |
| |
| * [[Periorbital edema]]
| |
| * [[Lower extremity edema]]
| |
| * [[Signs]]/[[symptoms]] of underlying inflammatory disorder
| |
| |
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| * Proteinuria
| |
| * Elevated level of [[acute phase reactant]] due to underlying inflammatory condition
| |
| |
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| * Biopsy and congo-red staining of the sample
| |
| |
| |
| * Treatment of the undelying [[iflammatory disorder]]
| |
| |-
| |
| |'''[[Primary (AL) Amyloidosis]]'''
| |
| |
| |
| * [[Monoclonal]] [[plasma cell]] proliferation
| |
| * Extracellular [[amyloid]] [[fibril]] deposition
| |
| |
| |
| * [[Anasarca]]
| |
| * [[Bleeding tendency]]
| |
| * Swelling of [[lower limbs]]
| |
| * Frothy [[urine]]
| |
| * [[Chest pain]]
| |
| * [[Numbness]] or [[tingling]]
| |
| * [[Early satiety]]
| |
| * [[Joint pains]]
| |
| * [[Macroglossia|Enlarged tongue]]
| |
| * [[Taste loss]]
| |
| * [[Hoarseness|Hoarseness of voice]]
| |
| * [[Alopecia|Hair loss]]
| |
| |
| |
| * Increased [[erythrocyte sedimentation rate]] ([[ESR]])
| |
| * Increased [[Alanine aminotransferase|alanine aminotrasnferase]] ([[Alanine transaminase|ALT]]) and [[Aspartate aminotransferase|aspartate aminotrasnferase]] ([[Aspartate transaminase|AST]])
| |
| * Increased [[Cardiac troponin I (cTnI) and T (cTnT)|cardiac troponins]]
| |
| * Increased [[brain natriuretic peptide]] ([[BNP]])
| |
| * Increased [[blood urea nitrogen]] ([[BUN]]) and [[creatinine]]
| |
| * [[Proteinuria]]
| |
| * Urinary [[Hyaline cast|hyaline]] and fatty casts
| |
| * [[Hypercholesterolemia]]
| |
| | |
| | |
| |
| |
| * Biopsy:
| |
| ** Diffuse glomerular deposition of amorphous [[hyaline]] material (nodular pattern - 8 to15 nm in diameter), in [[mesangium]] (weakly staining with [[Periodic acid-Schiff stain|periodic acid-Schiff]] ([[Periodic acid-Schiff stain|PAS]])
| |
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| * [[Melphalan]]-[[prednisone]]/[[dexamethasone]]
| |
| * [[Dexamethasone]] plus [[Cyclophosphamide]]-[[thalidomide]]
| |
| * [[Stem cell transplantation]] | |
| * [[Kidney transplantation]] | |
| |-
| |
| |'''[[Diabetic nephropathy|Diabetic Nephropathy]]'''
| |
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| * Hyperfiltration
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| * Constriction of [[efferent arteriole]]
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| * [[Microalbuminuria]]
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| * [[Mesangial cell|Mesangial]] proliferation
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| |
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| * [[Nocturia]]
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| * [[Fatigue]]
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| * [[Pruritis]]
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| * [[Peripheral edema]]
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| * [[Hyperglycemia]] (random plasma glucose ≥200 mg/dL)
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| * [[Proteinuria]]
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| * [[Glucosuria]]
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| * [[Glycosylated hemoglobin|HbA1C]] ≥6.5% (48 mmol/mol).<br />
| |
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| * Biopsy:
| |
| ** [[Periodic acid-Schiff stain|PAS]] positive [[Kimmelstiel-Wilson syndrome|Kimmelstiel-Wilson nodules]]
| |
| ** [[Glomerulosclerosis]]
| |
| |
| |
| * [[ACE inhibitor|ACE inhibitors]]
| |
| * [[Angiotensin receptor blockers]]
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| * [[Glycemic]] control
| |
| |-
| |
| |'''[[Minimal change disease|Minimal Change Disease]]'''
| |
| |
| |
| * [[Upper respiratory tract infection]]
| |
| * [[Allergy]] to bee sting
| |
| * [[Non-steroidal anti-inflammatory drug|NSAID]]
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| * [[Gold]]
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| * [[Penicillamine]]
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| * [[Ampicillin]]
| |
| * [[Mercury (element)|Mercury]]
| |
| * [[Hodgkin's lymphoma|Hodgkin's]] and [[Non-Hodgkin lymphoma|non-Hodgkin's lymphoma]]
| |
| |
| |
| * [[Peripheral edema]]
| |
| * [[Hypertension]]
| |
| * [[Peripheral edema]]
| |
| |
| |
| * [[Proteinuria]]
| |
| * [[Hypertension]]
| |
| * [[Hyperlipidemia]]
| |
| * [[Hypoalbuminemia]]
| |
| * [[Microscopic hematuria]]
| |
| |
| |
| * Biopsy:
| |
| ** Fused [[podocytes]]/effacement
| |
| |
| |
| * [[Prednisone]] with taper
| |
| * [[ACE inhibitor|ACE inhibitors]]
| |
| * [[Angiotensin receptor blockers]]
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| * Salt restriction
| |
| |-
| |
| |'''[[Focal segmental glomerulosclerosis|Focal Segmental Glomerulosclerosis]]'''
| |
| |
| |
| * [[HIV]]
| |
| * [[Parvovirus B19]]
| |
| * [[Cytomegalovirus]]
| |
| * [[Heroin]]
| |
| * [[Interferon alpha]]
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| * [[Lithium]]
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| * [[Pamidronate]]/aledronate
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| * [[Anabolic steroids]]
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| * [[Diabetes mellitus]]
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| * [[Hypertension]]
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| * [[Obesity]]
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| * [[Congenital heart disease cyanotic|Cyanotic congenital heart disease]]
| |
| * [[Sickle cell anemia]]
| |
| |
| |
| * [[Peripheral edema]]
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| * [[Hypertension]]
| |
| * [[Peripheral edema]]
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| |
| |
| * [[Proteinuria]]
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| * [[Hypertension]]
| |
| * [[Hyperlipidemia]]
| |
| * [[Hypoalbuminemia]]
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| * [[Microscopic hematuria]]
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| |
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| * Biopsy:
| |
| ** [[Podocytes|Podocyte]] foot process effacement
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| ** [[Capillary]] lumen abolished by the segmental increase in matrix<br />
| |
| |
| |
| * [[Prednisone]]
| |
| * [[Calcineurin inhibitor|Calcineurin inhibitors]] ([[Cyclosporine|Cyclosporin]], [[tacrolimus]])
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| * [[Rituximab]]
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| * [[Cyclophosphamide]]/[[chlorambucil]]
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| * [[Mycophenolate|Mycophenolate motefil]]
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| |-
| |
| |'''[[Fabry's disease|Fabry's Disease]]'''
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| * Deficient alpha galactosidase A
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| * [[Abdominal pain]]
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| * [[Arthralgia]]
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| * [[Febrile]] episodes
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| * [[Angiokeratomas]]
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| * Burning pain and tingling ([[Peripheral neuropathy|peripheral neuropathy)]]
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| * [[Hypohidrosis]]
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| * [[X-linked recessive]] inheritance
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| * Deficient alpha galactosidase A
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| * Increased ceramide trihexoside ([[Globotriaosylceramide 3-beta-N-acetylgalactosaminyltransferase|globotriaosylceramide]])
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| * Alpha-galactosidase A activity
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| * [[GLA|GLA gene]] analysis for [[Heterozygote|heterozygotes]]
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| * Enzyme replacement therapy
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| * [[ACE inhibitor|ACE inhibitors]]
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| * [[Gabapentin]], [[carbamazepine]]
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| * [[Migalastat]]
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| |-
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| |'''Light Chain Deposition Disease'''
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| * [[Multiple myeloma]]
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| * [[Waldenström's macroglobulinemia]]
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| * [[Monoclonal gammopathy of undetermined significance]]
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| * [[Asymptomatic]]
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| * [[Fatigue]]
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| * [[Weight loss]]
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| * [[Dyspnea]]
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| * [[Peripheral edema]]
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| * [[Proteinuria]]
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| * [[Portal hypertension]]
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| * Increased [[Alanine transaminase|ALT]], [[Aspartate transaminase|AST]]
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| * Biopsy:
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| ** Non-amyloid granules
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| * [[Bortezomib]]
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| * Autologous [[stem cell transplantation]]
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| * Immunomodulatory drugs
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| * [[Kidney transplantation|Kidney transplant]]
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| |-
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| |'''[[Membranous glomerulonephritis|Membranous Glomerulonephritis]]'''
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| * [[Hepatitis B]] and [[Hepatitis C|C]]
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| * [[HIV]]
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| * [[Non-Hodgkin lymphoma|Non-Hodgkin`s lymphoma]]
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| * [[Chronic lymphocytic leukemia]]
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| * [[Hodgkin lymphoma|Hodgkin`s lymphoma]]
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| * Solid tissue tumors
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| * [[Schistosomiasis]]
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| * [[Leprosy]]
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| * [[Hydatid disease]]
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| * [[Loaiasis]] (filaria)
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| * [[Malaria|Quartan malaria]]
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| * [[Systemic lupus erythematosis]] ([[SLE]])<br />
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| * [[Headache]]
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| * [[Edema]] affecting any area of the body
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| * Foamy appearance of urine
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| * [[Weight gain]]
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| * [[Poor appetite]]
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| * [[Nocturia]]
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| * [[Fatigue]]
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| * [[Hematuria]]
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| * [[Proteinuria]]
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| * [[Hypertension]]
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| * [[Hyperlipidemia]]
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| * [[Hypoalbuminemia]]
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| * Microscopic or gross hematuria
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| * [[Hypoalbuminemia]]
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| * [[Antinuclear antibodies|ANA]] and [[Anti-dsDNA antibody|anti-dsDNA]] positivity
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| |
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| * Biopsy:
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| ** [[Immunoglobulin G|IgG]] and [[C3 (complement)|C3]] deposits with thickened [[basement membrane]] with spikes and vacuolization
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| ** [[Glomerulosclerosis]]
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| |
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| * [[Prednisone]]
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| * [[Methylprednisolone]] with [[cyclophosphamide]]
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| * [[Tacrolimus]] with a six-month taper
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| * [[Rituximab]]
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| |-
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| |'''Fibrillary-Immunotactoid Glomerulopathy'''
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| * [[Idiopathic]]
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| * [[Hepatitis C]]
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| * Microscopic or [[Hematuria|gross hematuria]]
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| |
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| * [[Proteinuria]]
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| * [[Hypertension]]
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| * Increased [[blood urea nitrogen]] ([[Blood urea nitrogen|BUN]]) and [[creatinine]]
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| |
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| * Biopsy:
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| ** Polycloncal [[Immunoglobulin G|IgG]] deposits
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| ** Infiltration of glomerular structures by amorphous acellular material (nonbranching fibrils 12-24nm in diameter)
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| ** Ig heavy-chain and one light-chain subclass
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| |-
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| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Organ System Involvement}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Differential Diagnosis}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Causes}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Clinical Features}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Laboratory Findings}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Gold Standard Test}}
| |
| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Therapy}}
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| |-
| |
| | rowspan="6" |'''Polyneuropathy'''
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| |'''[[POEMS syndrome]] (Demyelinating)'''
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| * [[Monoclonal]] [[plasma cell]] proliferation
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| * [[Cytokine]] storm ([[IL-1]], [[Interleukin 6|IL-6]], [[Interleukin 12|IL-12]], [[TNF alpha]], [[VEGF]])
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| * Symmetrical, ascending chronic progressive [[polyneuropathy]] with both [[Sensory system|sensory]] (pin-prick and vibration) and [[Motor skill|motor]] disability ([[Motor skill|motor]] > [[sensory]])
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| * Generalized/extermity [[pain]]
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| * [[Areflexia]]
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| * Increased number of [[Platelet|thrombocytes]]
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| * Increased number of [[Red blood cell|erythrocytes]]
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| * Elevated [[Cerebrospinal fluid|cerebrospinal fluid (CSF)]] [[protein]] content
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| * Increased number of [[White blood cells|leukocytes]]
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| * High levels of [[Immunoglobulin G|IgG]] lambda or [[Immunoglobulin A|IgA]] lambda [[M protein|M-protein]] in the [[serum]]
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| * Increased number of [[Plasma cell|plasma cells]] in the [[bone marrow]]
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| * Increased serum [[Vascular endothelial growth factor|VEGF]] level
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| * Elevated levels of antitiroglobulin [[antibody]] and antithyroid peroxydase [[antibody]]
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| |
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| * [[POEMS syndrome diagnostic criteria|International Myeloma Working Group (IMWG) clinical and laboratory diagnostic criteria]]
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| |-
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| |'''[[Metabolic syndrome|Metabolic Syndrome]] (Axonal pathology)'''
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| * [[Diabetes mellitus]]
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| * Symmetric sensorimotor distal polyneuropathy
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| * Asymmetric proximal neuropathy
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| * [[Oculomotor nerve palsy|3rd nerve palsy]]
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| * [[Carpal tunnel syndrome|Carpel tunnel syndrome]]
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| * [[Autonomic neuropathy]]
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| * "Glove and stocking" type pain
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| * [[Muscle wasting]]
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| * [[Hammer toe|Hammer toes]]
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| * [[Polyuria]]
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| * [[Polydipsia]]
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| |
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| * Uncontrolled [[hyperglycemia]]
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| * Slowed [[Nerve conduction study|nerve conduction]]
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| * [[Small fiber peripheral neuropathy|Small fiber dysfunction]]
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| * [[Monofilament|Monofilament testing]]
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| |
| |
| * [[Fasting blood sugar|Fasting blood sugar level]] greater than equal to 126 mg/dl on 2 separate occasions
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| |
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| * [[Diabetes mellitus medical therapy|Anti-diabetic therapy]]
| |
| * [[Gabapentin]]
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| * [[Carbamazepine]]
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| * [[Foot care]]
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| |-
| |
| |'''[[Vitamin deficiencies|Vitamin Deficiencies]] (Axonal Pathology)'''
| |
| |
| |
| * [[Vitamin B12 deficiency]] (Decreased [[S-Adenosyl methionine|S-adenosyl methionine]])
| |
| * [[Thiamine deficiency|Vitamin B1 deficiency]]
| |
| |
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| * Primarily [[sensory]] deficits
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| * Vibration and [[proprioception]] affected
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| * [[Gait abnormality|Gait abnormalities]]
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| * [[Cognitive impairment]]
| |
| * [[Irritability]]
| |
| * [[Glossitis]]
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| | |
| | |
| |
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| * [[Anemia]] ([[Megaloblastic Anemias|megaloblastic]] in case of [[Vitamin B12 deficiency|B12 deficiency]])
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| * Decreased [[serum]] [[Vitamin B12]] levels (< 200 pg/ml)
| |
| * [[Methylmalonic acidemia|Elevated methylmalonic acid]]
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| |
| |
| * [[Serum]] [[Vitamin B12]] levels
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| * [[Methylmalonic acid|Methylmalonic acid levels]]
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| * [[Intrinsic factor|Intrinsic factor antibodies]]
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| | |
| | |
| |
| |
| * [[Vitamin B12]] supplement ([[parenteral]])
| |
| |-
| |
| |'''[[Guillain-Barré syndrome|Guillain-Barre Syndrome]] (Demyelinating)'''
| |
| |
| |
| * [[Anti-ganglioside antibodies|Anti-ganglioside]] and anti-[[myelin]] antibodies
| |
| * [[Viral]] infections:
| |
| ** [[Epstein Barr virus]]
| |
| ** [[Human Immunodeficiency Virus (HIV)|HIV]]
| |
| ** [[Cytomegalovirus]]
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| ** [[Varicella Zoster Virus|Varicella Zoster virus]]
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| * [[Bacterial]] infections:
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| ** [[Campylobacter]] infection
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| ** [[Mycoplasma pneumoniae]]
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| * Rapid onset and quick progression
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| * Progression stops after 2-3 weeks
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| * Bilateral ascending [[Paraesthesia|paraesthesias]] and [[paralysis]] (generalized)
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| * [[Muscle weakness|Weakness]]
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| * [[Ataxia]]
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| * [[Areflexia]]
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| * No fever
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| * 4 sub-types:
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| ** [[Acute inflammatory demyelinating polyneuropathy]]
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| ** [[Acute motor axonal neuropathy]]
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| ** Acute motor and sensory axonal neuropathy
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| ** [[Miller Fisher syndrome]]
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| * Delayed F waves
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| * Clinical diagnostic criteria (progressive weakness of more than two [[limbs]], [[areflexia]], and progression for no more than four weeks)
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| |
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| * [[Intravenous]] [[immunoglobulins]]
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| * [[Plasma]] exchange
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| * [[Mechanical ventilation|Respiratory support]]
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| * [[DVT]]/[[PE]] [[prevention]]
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| |-
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| |'''[[Chronic inflammatory demyelinating polyneuropathy|Chronic Inflammatory Demyelinating Polyneuropathy]] (CIDP) (Mixed axonal and demyelinatiing)'''
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| * Abnormal [[immune response]] (both [[Immunoglobulin G|IgG]] based [[Humoral immunity|humoral]] and [[Cell mediated immunity|T-Cell mediated]]) response to unknown [[antigen]] (possible culprits include [[myelin]] [[proteins]] P0, P2 and PMP22)
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| | |
| | |
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| * Slow onset and gradual progression
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| * [[Relapse|Relapsing]] and remitting course
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| * Symmetrical [[Proximal muscle weakness|proximal]] and [[Distal muscle weakness|distal motor]] and [[Sensory system|sensory weakness]] (legs>arms)
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| * [[Foot drop]]
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| * [[Numbness]], [[tingling]] and [[pain]]
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| * [[Areflexia]]
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| |
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| * Elevated [[CSF]] [[protein]] ([[oligoclonal bands]] with normal [[White blood cells|WBCs]])
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| * Slowed [[Nerve conduction study|motor nerve conduction velocities]]
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| * Prolonged distal [[Motor skill|motor]] latencies (period between F wave and initial stimulation)
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| * Delayed F wave latencies (recorded from the [[feet]], hence called "F" waves)
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| * [[MRI]] contrast enhancement and enlargement of [[Vertebra|T2]] [[spinal segments]]
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| * EFNS/PNS criteria
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| * Koski criteria
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| * [[Corticosteroids]]
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| * [[Intravenous immunoglobulin]] ([[IVIG]])
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| * [[Immunosupressive drug|Immunosupressants]] ([[Alemtuzumab|Alemtuzemab]] [[Azathioprine]] [[Cyclophosphamide]] [[Cyclosporine|Cyclosporin]] [[Etanercept]] [[Interferon-alpha]])
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| |-
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| |'''[[Multifocal motor neuropathy|Multifocal Motor Neuropathy]]'''
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| * [[Immune response|Abnormal immune response]] ([[Anti-ganglioside antibodies|Anti ganglioside]] [[Anti-ganglioside antibodies|GM-1]] [[IgM]] [[antibodies]])
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| * Progressive, asymmetric, distal and upper [[Limb (anatomy)|limb]] predominant weakness
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| * No significant [[sensory]] abnormalities
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| * [[Areflexia]]
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| * Elevated [[CSF]] [[protein]]
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| * Clinical criteria (EFNS/PNS):
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| ** Slowly progressive or step-wise progressive, focal, asymmetric [[limb]] weakness; i.e., [[Motor skill|motor]] involvement in the [[motor nerve]] distribution of at least two nerves for > 1 month.
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| ** No objective [[Sensory system|sensory]] abnormalities except for minor vibration sense abnormalities in the [[lower limbs]]
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| | |
| | |
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| * [[Intravenous immunoglobulin|Intravenous immunoglobulins]]
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| * [[Cyclophosphamide]]
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| * [[Rituximab]]
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| |-
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| | style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Organ System Involvement'''}}
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| | style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Differential Diagnosis'''}}
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| | style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Causes'''}}
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| | style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Features'''}}
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| | style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Laboratory Findings'''}}
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| | style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Gold Standard Test'''}}
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| | style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Therapy'''}}
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| |-
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| | rowspan="7" |'''Organomegaly (Hepatosplenomegaly and Lymphadenopathy)'''
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| |'''[[Malaria]]'''
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| * [[Plasmodium falciparum]]
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| * [[Plasmodium ovale|P. ovale]]
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| * [[P. malariae]]
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| * [[Plasmodium knowlesi|P. knowlesi]]
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| * Tertian ([[Plasmodium vivax|vivax]], [[Plasmodium ovale|ovale]], [[Plasmodium falciparum|falciparum]]), quartan (malariae), quotidian fever ([[Plasmodium knowlesi|knowlesi]])
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| * [[Vector]] is female [[Anopheles]] mosquito
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| * [[Hepatosplenomegaly]]
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| * [[Lymphadenopathy]]
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| * [[Jaundice]]
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| * [[Icterus (medicine)|Icterus]]
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| * [[Tachycardia]]
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| * [[Tachypnea]]
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| * [[Productive cough]]
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| * [[Hematuria]]
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| * [[Altered mental status]]
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| * [[Microcytic anemia]]
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| * [[Malaria differential diagnosis|Thick and thin blood films]] ([[Giemsa stain|Giemsa staining]])
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| | |
| * Rapid diagnostic test ([[antigen]] detection
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| * [[Polymerase chain reaction]] ([[Polymerase chain reaction|PCR]])
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| * [[Enzyme linked immunosorbent assay (ELISA)|Enzyme linked immunosorbent assay]] ([[Enzyme linked immunosorbent assay (ELISA)|ELISA]])
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| * [[Malaria differential diagnosis|Thick and thin films]]
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| * Non-[[Plasmodium falciparum|falciparum]] species:
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| ** [[Chloroquine]] (in susceptible)
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| ** [[Artemisinin]] plus [[Mefloquine|mefloquin]] or [[lumefantrine]] (in [[chloroquine]] resistant)
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| * [[Plasmodium falciparum|Falciparum]] species:
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| ** [[Chloroquine]] (in susceptible)
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| ** [[Artemether]] plus [[lumefantrine]] (in [[Chloroquine|chloroquin]] resistant) OR
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| ** [[Artesunate]] plus [[Mefloquine|mefloquin]] OR
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| ** [[Artesunate]] plus [[Sulfadoxine|sulfadoxine-pyrimethamine]]
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| ** [[Atovaquone-Proguanil|Atovaquone plus proguanil]]
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| |-
| |
| |'''[[Kala-azar]]'''
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| * [[Leishmaniasis|Leshmania donovani]]
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| * L. infantum
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| * L. chagasi
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| | |
| | |
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| * [[Fever]]
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| * Vector is [[sandfly]]
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| * [[Hepatosplenomegaly]]
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| * [[Lymphadenopathy]]
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| * [[Hyperpigmentation]]
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| * [[Anemia]]
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| * [[Direct agglutination test]] (DAT)
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| * rk39 dipstick
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| * [[Enzyme linked immunosorbent assay (ELISA)|ELISA]]
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| |
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| * [[Splenic]] aspiration
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| |
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| * [[Liposomal amphotericin B]]
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| * [[Sodium stibogluconate]]
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| * [[Pentamidine]]
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| |-
| |
| |'''[[Hepatitis|Infective Hepatitis]]'''
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| |
| |
| * [[Hepatitis A virus|Hepatitis A virus (HAV)]]
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| * [[HBV]]
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| * [[Hepatitis C|HCV]]
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| * [[Hepatitis D|HDV]] (co-infection with [[HBV]])
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| * [[Hepatitis E|HEV]]
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| |
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| * [[Fever]]
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| * Transmitted via [[fecal-oral route]] ([[Hepatitis A|HAV]], [[Hepatitis B virus|HBV]], [[HDV]], [[HEV]]), infected sera ([[HCV]]), [[sexual contact]] with infected individuals
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| * [[Hepatosplenomegaly]] (may become shrunken in cases of [[cirrhosis]] due to chronic infection)
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| * [[Lymphadenopathy]]
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| * [[Jaundice]]
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| * [[Palmar erythema]]
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| * [[Spider angiomata]]
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| * [[Gynecomastia]]
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| * [[Arthritis-dermatitis syndrome]]
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| |
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| * [[Antigen]] and [[antibody]] detection
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| * Total and direct [[bilirubin]] (increased)
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| * Severe disease is often associated with persistent [[bilirubin]] levels >340 mmol/L
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| * [[ALT]] and [[AST]] (increased)
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| * [[Alkaline phosphatase]] (normal or mildly elevated)
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| * [[Prothrombin time]] (prolonged from synthetic defect, caused by hepatocellular [[necrosis]])
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| * [[Total protein]] (decreased)
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| * [[Globulin]] (mildly elevated)
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| * Initial [[lymphopenia]] and [[neutropenia]], followed by relative [[lymphocytosis]]
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| * [[Anemia|Low hemoglobin]]
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| |
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| * [[Antigen]] and [[antibody]] detection
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| |
| |
| * [[Interferon]] ([[IFN]])
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| | |
| * [[Antivirals|Nucleoside analogs]]
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| | |
| * [[Antivirals|Nucleotide analogs]]
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| |-
| |
| |'''[[Chronic myelogenous leukemia|Chronic Myelogenous Leukemia]] (CML)'''
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| |
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| * [[Philadelphia chromosome|BCR/ABL gene]] fusion product due to [[Chromosomal translocation|translocation]] [[mutation]] [[Philadelphia chromosome|t(9;22)]](q34;q11)
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| |
| |
| * [[Fever]]
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| * [[Weight loss]]
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| * [[Hepatosplenomegaly]]
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| * [[Lymphadenopathy]]
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| | |
| * [[Bruises]]
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| * [[Petechiae]]
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| * [[Ulcers]]
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| * [[Vesicles]]
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| * [[Malaise]]
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| * [[Early satiety]]
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| |
| |
| * [[Anemia]]
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| | |
| * [[Leukocytosis]] (median of 100,000/µL) with a [[left shift]]
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| * [[Thrombocytosis]]
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| * [[Blast|Blasts]] usually <2%
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| * Absolute [[basophilia]]
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| * Absolute [[eosinophilia]]
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| * [[Monocytosis]]
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| * [[Thrombocytosis]]
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| * [[Thrombocytopenia]] suggests an alternative diagnosis or the presence of advanced stage
| |
| * Elevated [[uric acid]]
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| * Elevated [[histamine]] levels
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| |
| |
| * [[Fluorescence in situ hybridization|Fluoroscent insitu hybridization (FISH)]]
| |
| |
| |
| * [[Imatinib]]
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| * [[Dasatinib]]
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| * [[Nilotinib]]
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| * [[Bosutinib]]
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| * [[Ponatinib]]
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| | |
| * [[Cytarabine]]
| |
| * [[Cytarabine|HDAC]] (high-dose [[cytarabine]])
| |
| * [[Hydroxyurea]]
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| * [[Busulfan]]
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| * [[Busulfex]]
| |
| * [[Stem cell transplantation]]
| |
| |-
| |
| |'''[[Lymphoma]]'''
| |
| |
| |
| * Various causes based on type:
| |
| ** [[Hodgkin's lymphoma|Hodgkin's]]
| |
| ** [[Non-Hodgkin lymphoma|Non-Hodgkin's]]
| |
| |
| |
| * [[Fever]]
| |
| * [[Weight loss]]
| |
| * [[Lymphadenopathy]]
| |
| * [[Hepatosplenomegaly]]
| |
| * [[Night sweats]], constant [[fatigue]]
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| * Purplish scaly rash in cases of [[cutaneous lymphoma]]
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| |
| |
| * [[Erythrocyte sedimentation rate|Elevated ESR]]
| |
| * [[C-reactive protein|Increased CRP]]
| |
| * [[Lactate dehydrogenase|Increased LDH]]
| |
| * [[Anemia of chronic disease]]
| |
| |
| |
| * [[Lymph node biopsy]]
| |
| |
| |
| |-
| |
| |'''[[Primary amyloidosis|Primary (AL) Amyloidosis]]'''
| |
| |
| |
| * Aggregation and deposition of [[immunoglobulin light chains]] that are usually produced by [[plasma cell]] clones
| |
| |
| |
| * [[Nephrotic syndrome]] ([[peripheral edema]])
| |
| * [[Restrictive cardiomyopathy]] ([[fatigue]], [[dyspnea]], [[syncope]])
| |
| * [[Peripheral neuropathy]] ([[numbness]], [[tingling]])
| |
| * [[Hepatomegaly]] with elevated [[liver enzymes]]
| |
| * [[Macroglossia]]
| |
| * [[Purpura]]
| |
| * [[Bleeding diathesis]]
| |
| |
| |
| * Typical green birefringence under polarized light after Congo red staining (appears in red under normal light)
| |
| |
| |
| * Congo red staining
| |
| |
| |
| * Melphalan-prednisone/dexamethasone
| |
| * Dexamethasone plus Cyclophosphamide-thalidomide
| |
| * Stem cell transplantation
| |
| |-
| |
| |'''[[Gaucher's disease|Gaucher's Disease]]'''
| |
| |
| |
| * [[GBA (gene)|GBA gene]] [[mutation]]
| |
| * Aberrant metabolism of [[glucocerebroside]] ([[lipid]])
| |
| |
| |
| * [[Hydrops fetalis]]
| |
| * Dry, scaly skin ([[ichthyosis]]) or other [[skin]] abnormalities
| |
| * [[Hepatosplenomegaly]]
| |
| * Distinctive facial features
| |
| * [[Neurological disorder|Neurological problems]]
| |
| * [[Gall stones]]
| |
| * [[Growth retardation]]
| |
| |
| |
| * [[Hypocholesterolemia]]
| |
| * [[Splenic]] nodules
| |
| * [[Cytopenias]] (especially [[thrombocytopenia]])
| |
| * Increased [[ferritin]] levels
| |
| * Increased tartarate resistant acid phosphatase (TRAP) levels
| |
| |
| |
| * Enzyme assay for [[glucocerebrosidase]]
| |
| * [[DNA|DNA analysis]] for [[GBA (gene)|GBA mutation]]
| |
| | | |
| * [[Enzyme replacement therapy|Enzyme replacement]]
| |
| * [[Splenectomy]]
| |
| * [[Blood transfusion]]
| |
| |}
| |
| | |
| | |
| | |
| | |
| ===Example #1===
| |
| The patient presented with S.O.B. one year after hysterectomy for a leiomyomatous uterus.
| |
| <div align="left">
| |
| <gallery heights="175" widths="175">
| |
| Image:Intravascular-extension-leiomyoma-001.jpg|CT in Intravenous leiomyomatosis
| |
| Image:Intravascular-extension-leiomyoma-002.jpg
| |
| </gallery>
| |
| </div>
| |
| <div align="left">
| |
| <gallery heights="175" widths="175">
| |
| Image:Intravascular-extension-leiomyoma-003.jpg
| |
| Image:Intravascular-extension-leiomyoma-004.jpg
| |
| </gallery>
| |
| </div>
| |
| <div align="left">
| |
| <gallery heights="175" widths="175">
| |
| Image:Intravascular-extension-leiomyoma-005.jpg
| |
| Image:Intravascular-extension-leiomyoma-006.jpg
| |
| </gallery>
| |
| </div>
| |
| | |
| <div align="left">
| |
| <gallery heights="175" widths="175">
| |
| Image:Intravascular-extension-leiomyoma-007.jpg
| |
| Image:Intravascular-extension-leiomyoma-008.jpg
| |
| </gallery>
| |
| </div>
| |