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| __NOTOC__ | | __NOTOC__ |
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| {{Infobox_Disease | | | {{Infobox_Disease | |
| Name = {{PAGENAME}} | | | Name = {{PAGENAME}} | |
| Image = Gray591.png | | | Image = Gray591.png | |
| Caption = The portal vein and its tributaries. | | | Caption = The portal vein and its tributaries- By Henry Vandyke Carter [Public domain], via Wikimedia Commons<ref><"https://commons.wikimedia.org/wiki/File%3AGray591.png">via Wikimedia Commons</ref>| |
| }} | | }} |
| | '''For the patient information page on this topic, click [[Portal hypertension (patient information)|here]].''' |
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| {{Portal hypertension}} | | {{Portal hypertension}} |
| {{CMG}},'''Assistant Editor-in-Chief:''' [[User:Soumya Sachdeva|Soumya Sachdeva]]
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| __NOTOC__
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| ==Pathophysiology==
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|
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| ==Natural History and Complications==
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| There is an increased risk of
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| * [[Spontaneous bacterial peritonitis]]
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| * [[Hepatorenal syndrome]]
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| * [[Hepatic encephalopathy]]
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| * [[Congestive gastropathy]]
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| * [[Hypersplenism]]
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| * [[Ascites]]
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| * [[Variceal bleeding]]
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| * [[Renal failure]]
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|
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| ==Causes==
| | {{CMG}} {{AE}} {{EG}} |
| '''Prehepatic'''
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| *Congenital abnormalities
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| *Idiopathic tropical [[splenomegaly]]
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| *[[Portal vein occlusion]]
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| *[[Splenic Vein Thrombosis]]
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| *Umbilical sepsis
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| '''Intrahepatic'''
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| *[[Alcoholic hepatitis]]
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| *[[Boeck's Diesease]]
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| *[[Chronic hepatitis]]
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| *[[Cirrhosis]]
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| *[[Fatty liver]]
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| *[[Fulminant hepatic failure]]
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| *[[Hodgkin's Lymphoma]]
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| *[[Osteomyelosclerosis]]
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| *Partial nodular transformation
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| *[[Schistosomiasis]]
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| *[[Wilson's Disease]]
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| '''Posthepatic'''
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| *[[Cardiomyopathy]]
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| *[[Congestive Heart Failure]]
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| *[[Constructive Pericarditis]]
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| *[[Inferior vena cava obstruction]]
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| *[[Tricuspid insufficiency]]
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|
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| ===Common Causes===
| | {{SK}}Hypertension of portal system, Portal vein hypertension, Portal high blood pressure, Portal vein high pressure. |
| ===Causes by Organ System===
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| {|style="width:70%; height:100px" border="1"
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| |style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
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| |style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | [[Cardiomyopathy]], [[Congestive heart failure]], [[Constructive pericarditis]], [[Inferior vena cava obstruction]], [[Splanchnic arteriovenous fistula]], [[Tricuspid insufficiency ]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Chemical / poisoning'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Dermatologic'''
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| |bgcolor="Beige"| [[NISCH syndrome]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Drug Side Effect'''
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| |bgcolor="Beige"| [[Didanosine]], [[Thioguanine]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Ear Nose Throat'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Endocrine'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Environmental'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Gastroenterologic'''
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| |bgcolor="Beige"| [[Alcoholic hepatitis]], [[Biliary atresia]], [[Budd-Chiari syndrome]], [[Cholestasis]], [[Chronic hepatitis]], [[Chronic liver disease]], [[Cirrhosis]], [[Congenital atresia or stenosis of portal vein]], [[Congenital hepatic fibrosis]], [[Congenital narrowing of the portal vein]], [[Cruveilhier-Baumgarten syndrome]], [[Fatty liver]], [[Focal nodular hyperplasia]], [[Fulminant hepatic failure]], [[Hepatic amyloidosis with intrahepatic cholestasis]], [[Hepatic arterioportal fistula]], [[Hepatic portal vein obstruction]], [[Hepatic vein occlusion]], [[Hepatic vein thrombosis]], [[Hepatic venoocclusive disease with immunodeficiency]], [[Idiopathic liver cirrhosis]], [[Idiopathic portal hypertension ]], [[Idiopathic tropical splenomegaly]], [[Liver fibrosis]], [[Mosse syndrome]], [[Neonatal hepatitis]], [[Nodular regenerative hyperplasia of the liver]], [[Obliterative portal venopathy]], [[Partial nodular transformation]], [[Portal hypertension due to intrahepatic block]], [[Portal vein abnormality]], [[Portal vein compression]], [[Portal vein occlusion]], [[Portal vein thrombosis ]], [[Primary biliary cirrhosis]], [[Primary sclerosing cholangitis]], [[Splenic vein thrombosis]], [[Alpha-1-antitrypsin deficiency]], [[Progressive familial intrahepatic cholestasis]], [[Berry aneurysm, cirrhosis, pulmonary emphysema, and cerebral calcification]], [[Hepatic metastasis]]
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| [[Pancreatic cancer]], [[NISCH syndrome]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Genetic'''
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| |bgcolor="Beige"| [[Alpha-1-antitrypsin deficiency]], [[COACH syndrome]], [[Cystic fibrosis]], [[Gaucher disease]], [[Glycosylphosphatidylinositol deficiency]], [[Hemochromatosis]], [[Interferon gamma receptor 1 deficiency]], [[NISCH syndrome]], [[Progressive familial intrahepatic cholestasis]], [[Wilson's Disease ]], [[Hepatic venoocclusive disease with immunodeficiency]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Hematologic'''
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| |bgcolor="Beige"| [[Blackfan Diamond anemia]], [[Congenital pure red cell aplasia ]], [[Myeloproliferative diseases]], [[Mosse syndrome]], [[Hemochromatosis]], [[Osteomyelosclerosis]], [[Hodgkin's lymphoma]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Iatrogenic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Infectious Disease'''
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| |bgcolor="Beige"| [[Schistosomiasis ]], [[Umbilical sepsis ]], [[Viral hepatitis]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Musculoskeletal / Ortho'''
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| |bgcolor="Beige"| [[Osteomyelosclerosis]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Neurologic'''
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| |bgcolor="Beige"| [[Berry aneurysm, cirrhosis, pulmonary emphysema, and cerebral calcification]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Nutritional / Metabolic'''
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| |bgcolor="Beige"| [[Gaucher disease]], [[Glycosylphosphatidylinositol deficiency]], [[Wilson's Disease ]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Obstetric/Gynecologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Oncologic'''
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| |bgcolor="Beige"| [[Extrinsic compression (tumors)]], [[Hepatic metastasis]], [[Hodgkin's lymphoma]], [[Pancreatic cancer]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Opthalmologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Overdose / Toxicity'''
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| |bgcolor="Beige"| [[Didanosine]], [[Thioguanine]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Psychiatric'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Pulmonary'''
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| |bgcolor="Beige"| [[Cystic fibrosis]], [[Alpha-1-antitrypsin deficiency]], [[Berry aneurysm]], [[Cirrhosis]], [[Pulmonary emphysema]], and [[cerebral calcification]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Renal / Electrolyte'''
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| |bgcolor="Beige"| [[Polycystic kidney disease]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Rheum / Immune / Allergy'''
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| |bgcolor="Beige"| [[Hepatic venoocclusive disease with immunodeficiency]], [[Granulomatous diseases]] ([[Sarcoidosis]], [[Tuberculosis)]]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Sexual'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Trauma'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Urologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Dental'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Miscellaneous'''
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| |bgcolor="Beige"| Granulomatous diseases (Sarcoidosis, Tuberculosis)]], [[Extrinsic compression]] ([[tumors]])
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| |-
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| |}
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|
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| ===Causes in Alphabetical Order=== | | ==[[Portal hypertension overview|Overview]]== |
| {{MultiCol}}
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| *[[Alcoholic hepatitis]]
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| *[[Alpha-1-antitrypsin deficiency]]
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| *[[Berry aneurysm, cirrhosis, pulmonary emphysema, and cerebral calcification]]
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| *[[Biliary atresia]]
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| *[[Blackfan Diamond anemia]]
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| *[[Budd-Chiari syndrome]]
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| *[[Cardiomyopathy]]
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| *[[Cholestasis]]
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| *[[Chronic hepatitis]]
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| *[[Chronic liver disease]]
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| *[[Cirrhosis]]
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| *[[COACH syndrome]]
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| *[[Congenital atresia or stenosis of portal vein]]
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| *[[Congenital hepatic fibrosis]]
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| *[[Congenital narrowing of the portal vein]]
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| *[[Congenital pure red cell aplasia ]]
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| *[[Congestive heart failure]]
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| *[[Constructive pericarditis]]
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| *[[Cruveilhier-Baumgarten syndrome]]
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| *[[Cystic fibrosis]]
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| *[[Didanosine]]
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| *[[Extrinsic compression (tumors)]]
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| *[[Fatty liver]]
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| *[[Focal nodular hyperplasia]]
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| *[[Fulminant hepatic failure]]
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| *[[Gaucher disease]]
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| *[[Glycosylphosphatidylinositol deficiency]]
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| *[[Granulomatous diseases (sarcoidosis, tuberculosis)]]
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| *[[Hemochromatosis]]
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| *[[Hepatic amyloidosis with intrahepatic cholestasis]]
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| *[[Hepatic arterioportal fistula]]
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| *[[Hepatic metastasis]]
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| *[[Hepatic portal vein obstruction]]
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| *[[Hepatic vein occlusion]]
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| *[[Hepatic vein thrombosis]]
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| {{ColBreak}}
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| *[[Hepatic venoocclusive disease with immunodeficiency]]
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| *[[Hodgkin's lymphoma]]
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| *[[Idiopathic liver cirrhosis]]
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| *[[Idiopathic portal hypertension ]]
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| *[[Idiopathic tropical splenomegaly]]
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| *[[Inferior vena cava obstruction]]
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| *[[Interferon gamma receptor 1 deficiency]]
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| *[[Liver fibrosis]]
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| *[[Mosse syndrome]]
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| *[[Myeloproliferative diseases]]
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| *[[Neonatal hepatitis]]
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| *[[NISCH syndrome]]
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| *[[Nodular regenerative hyperplasia of the liver]]
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| *[[Obliterative portal venopathy]]
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| *[[Osteomyelosclerosis]]
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| *[[Pancreatic cancer]]
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| *[[Partial nodular transformation]]
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| *[[Polycystic kidney disease]]
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| *[[Portal hypertension due to intrahepatic block]]
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| *[[Portal vein abnormality]]
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| *[[Portal vein compression]]
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| *[[Portal vein occlusion]]
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| *[[Portal vein thrombosis ]]
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| *[[Primary biliary cirrhosis]]
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| *[[Primary sclerosing cholangitis]]
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| *[[Progressive familial intrahepatic cholestasis]]
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| *[[Sarcoidosis]]
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| *[[Schistosomiasis ]]
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| *[[Splanchnic arteriovenous fistula]]
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| *[[Splenic vein thrombosis]]
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| *[[Thioguanine]]
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| *[[Tricuspid insufficiency ]]
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| *[[Umbilical sepsis ]]
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| *[[Viral hepatitis]]
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| *[[Wilson's Disease ]]
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| {{EndMultiCol}}
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|
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| ==Diagnosis== | | ==[[Portal hypertension historical perspective|Historical Perspective]]== |
| ===Physical Examination===
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| ====Abdomen====
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| * [[Ascites]] (free fluid in the [[peritoneal cavity]])
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| * [[Hepatic encephalopathy]]
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| * [[Splenomegaly]] (enlargement of the spleen) with consequent sequestration therein of [[red blood cells]], [[white blood cells]], and [[platelets]], together leading to mild [[pancytopenia]]
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| * [[Portacaval anastomosis|portacaval anastomoses]] ([[Esophageal varices]], [[hemorrhoid]]s, [[caput medusae]]), with [[esophageal varices]] posing an ongoing risk of life-threatening hemorrhage.<ref>Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:291</ref>
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| ===Investigations=== | | ==[[Portal hypertension classification|Classification]]== |
| # Barim swallow - presence of varices is seen as filling defects(bag of worms appearnace)
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| # Upper gastrointestinal endoscopy - presence of cherry red spots.Very reliable
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| # Ultrasonography - to note the size of liver,spleen,poratl vein,splenic vein and to look for the presence of collaterals
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| # Hepatic venous pressure drainage measurement - Gold standard for measuring portal hypertension.If ore than 5 mmHg it is considered as significant
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| # Portal venography - patency and the calibre of portal vein and splenic vein , presence of collaterals
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| # Liver function tests for liver diseases
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| # Proctoscopy - rectal varices
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| # Barium enema - colonic varices
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|
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|
| '''Patient #1'''
| | ==[[Portal hypertension pathophysiology|Pathophysiology]]== |
|
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|
| <gallery>
| | ==[[Portal hypertension causes|Causes]]== |
| Image:
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| Portal-venous-hypertension-001.jpg | | ==[[Portal hypertension differential diagnosis|Differentiating Portal Hypertension from other Diseases]]== |
|
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| Image:
| | ==[[Portal hypertension epidemiology and demographics|Epidemiology and Demographics]]== |
|
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| Portal-venous-hypertension-002.jpg | | ==[[Portal hypertension risk factors|Risk Factors]]== |
|
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| Image:
| | ==[[Portal hypertension screening|Screening]]== |
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| Portal-venous-hypertension-003.jpg | | ==[[Portal hypertension natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
|
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| Image:
| | ==Diagnosis== |
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| Portal-venous-hypertension-004.jpg
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| </gallery>
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| '''Patient #2: Recanalized umbilican vein'''
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| <gallery>
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| Image:
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| Uv1.jpg
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| Image:
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| Uv2.jpg
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| Image:
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| Uv3.jpg
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| Image:
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| Uv4.jpg
| | [[Portal hypertension history and symptoms|History and Symptoms]] | [[Portal hypertension physical examination|Physical Examination]] | [[Portal hypertension laboratory findings|Laboratory Findings]] | [[Portal hypertension electrocardiogram|Electrocardiogram]] | [[Portal hypertension x ray|X Ray]] | [[Portal hypertension CT|CT]] | [[Portal hypertension MRI|MRI]] | [[Portal hypertension ultrasound|Ultrasound]] | [[Portal hypertension other imaging findings|Other Imaging Findings]] | [[Portal hypertension other diagnostic studies|Other Diagnostic Studies]] |
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| Image:
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| Uv5.jpg
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| Image:
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| Uv6.jpg
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| </gallery>
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|
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| ==Treatment== | | ==Treatment== |
| ===Medical management===
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| Treatment with a non-selective [[beta blocker]] is often commenced once [[portal hypertension]] has been diagnosed, and almost always if there has already been bleeding from esophageal varices. Typically, this is done with either [[propranolol]] or [[nadolol]]. The addition of a [[nitrate]], such as [[isosorbide mononitrate]], to the [[beta blocker]] is more effective than using beta blockers alone and may be the preferred regimen in those people with portal [[hypertension]] who have already experienced variceal bleeding. In acute or severe complications of the [[hypertension]], such as bleeding varices, intravenous [[octreotide]] (a [[somatostatin]] analogue) or intravenous [[terlipressin]] (an [[antidiuretic hormone]] analogue) is commenced to decrease the portal pressure.
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| ===Percutaneous interventions===
| | [[Portal hypertension medical therapy|Medical Therapy]] | [[Portal hypertension surgery|Surgery]] | [[Portal hypertension primary prevention|Primary Prevention]] | [[Portal hypertension secondary prevention|Secondary Prevention]] | [[Portal hypertension cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | |
| [[Transjugular intrahepatic portosystemic shunt]]ing is the creation of a connection between the portal and the venous system. As the pressure over the venous system is lower than over a hypertensive portal system, this would decrease the pressure over the portal system and a decreased risk of complications. | | [[Portal hypertension future or investigational therapies|Future or Investigational Therapies]] |
|
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| ===Surgical interventions=== | | ==Case Studies== |
| *[[Distal splenorenal shunt procedure|Distal splenorenal shunt]]
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| *[[Liver transplant]]
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|
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| The most definitive treatment of portal hypertension is a [[liver transplant]].
| | [[Portal hypertension case study one|Case #1]] |
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| == References ==
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| {{Reflist}}
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| ==External links==
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| * {{Chorus|00863}}
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| {{Gastroenterology}} | | {{Gastroenterology}} |
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| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WikiDoc Sources}} | | {{WikiDoc Sources}} |
| | ==References== |
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| [[Category:Gastroenterology]] | | [[Category:Gastroenterology]] |
| [[Category:Hepatology]] | | [[Category:Hepatology]] |
| [[Category:Disease]] | | [[Category:Disease]] |
| | [[Category:Emergency medicine]] |
| | [[Category:Up-To-Date]] |
