Portal hypertension causes

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Eiman Ghaffarpasand, M.D. [2]


Life-threatening causes of portal hypertension include cirrhosis, severe portal venous obstruction or thrombosis (Budd-Chiari syndrome), and fulminant hepatic failure (e.g., due to hepatitis). Common causes for portal hypertension include alcoholic hepatitis, autoimmune disease, bacterial intestinal infections (e.g., recurrent E.coli infection), chronic hepatitis, cirrhosis, fatty liver, schistosomiasis, and sickle cell disease.


Life-threatening Causes

Common Causes

Portal hypertension may be caused by:[1][2][3]

Less Common Causes

Less common causes of portal hypertension include:[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]

Genetic Causes

Portal hypertension is caused by a mutation in the following genes:[21][22][23][24][25][26][21][27][28][29][30]

Causes classified by etiology


Prehepatic causes of portal hypertension include:[2][15][16]


Intrahepatic causes of portal hypertension include:[1][5][6][7][8][9][10][11][12][13][14][17][18][19][20]


Posthepatic causes of portal hypertension include:[3][4]

Causes by Organ System

Cardiovascular Cardiomyopathy, Congestive heart failure, Constrictive pericarditis, Inferior vena cava obstruction, Splanchnic arteriovenous fistula, Tricuspid insufficiency.
Chemical/Poisoning Arsenicals.
Dermatological NISCH syndrome.
Drug Side Effect Didanosine, Thioguanine, Vitamin A, Azathioprine, Cis-thioguanine, Antiretroviral therapy, Adams-Olivier syndrome.
Ear Nose Throat No underlying causes
Endocrine Hashimoto's thyroiditis.
Environmental Vitamin A, Alcoholic hepatitis.
Gastroenterology 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, Hepatic metastasis, Pancreatic cancer, NISCH syndrome, Crohn’s disease.
Genetic 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, Turner syndrome, Leukotriene B4 (LTB4) mutation, Ectonucleoside triphosphate diphosphohydrolase 4 (ENTPD4) mutation, Endothelin receptor type A (EDNRA) mutation, Deoxyguanosine kinase (DGUOK) mutation, Adenosine deaminase (ADA) mutation, Phospholipase A2 (PL2G10) mutation, Cytochrome P450, family 4, subfamily F, polypeptide 3 (CYP4F3) mutation, Glutathione peroxidase 3 (GPX3) mutation, Prostaglandin E receptor 2 (PTGER2) mutation, Endothelin (EDN1) mutation, Natriuretic peptide receptor 3 (NPR3) mutation, Cluster of differentiation 44 (CD44) mutation, Transforming growth factor (TGF)-β mutation, ATP-binding cassette, subfamily C, member 1 (ABCC1) mutation, Phosphomannose isomerase deficiency
Hematologic Blackfan Diamond anemia, Congenital pure red cell aplasia , Myeloproliferative diseases, Mosse syndrome, Hemochromatosis, Osteomyelosclerosis, Hodgkin's lymphoma, Sickle cell disease, Inherited thrombophilias, Antiphospholipid syndrome, Human immunodeficiency virus (HIV) infection, Myeloproliferative neoplasm, Common variable immunodeficiency syndrome.
Iatrogenic Solid organ transplant, Renal transplantation, Liver transplantation.
Infectious Disease Schistosomiasis , Umbilical sepsis, Viral hepatitis, Recurrent E.coli infection.
Musculoskeletal / Ortho Osteomyelosclerosis, Connective tissue diseases.
Neurologic Berry aneurysm, Cerebral calcification, Beck's disease.
Nutritional / Metabolic Gaucher disease, Glycosylphosphatidylinositol deficiency, Wilson's disease, Vitamin A, Alcoholic hepatitis.
Obstetric/Gynecologic Antiphospholipid syndrome.
Oncologic Extrinsic compression (tumors), Hepatic metastasis, Hodgkin's lymphoma, Pancreatic cancer.
Opthalmologic No underlying causes
Overdose / Toxicity Didanosine, Thioguanine.
Psychiatric No underlying causes
Pulmonary Cystic fibrosis, Alpha-1-antitrypsin deficiency, Berry aneurysm, Cirrhosis, Pulmonary emphysema, Cerebral calcification.
Renal / Electrolyte Polycystic kidney disease.
Rheum / Immune / Allergy Hepatic venoocclusive disease with immunodeficiency, Granulomatous diseases (Sarcoidosis, Tuberculosis), Hashimoto's thyroiditis.
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Dental No underlying causes
Miscellaneous Granulomatous diseases (Sarcoidosis, Tuberculosis), Extrinsic compression (tumors), Inferior vena cava obstruction.

Causes in Alphabetical Order


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  2. 2.0 2.1 Kono K, Ohnishi K, Omata M, Saito M, Nakayama T, Hatano H, Nakajima Y, Sugita S, Okuda K (1988). "Experimental portal fibrosis produced by intraportal injection of killed nonpathogenic Escherichia coli in rabbits". Gastroenterology. 94 (3): 787–96. PMID 3276575.
  3. 3.0 3.1 Kumar S, Joshi R, Jain AP (2007). "Portal hypertension associated with sickle cell disease". Indian J Gastroenterol. 26 (2): 94. PMID 17558079.
  4. 4.0 4.1 Bayan K, Tüzün Y, Yilmaz S, Canoruc N, Dursun M (2009). "Analysis of inherited thrombophilic mutations and natural anticoagulant deficiency in patients with idiopathic portal hypertension". J. Thromb. Thrombolysis. 28 (1): 57–62. doi:10.1007/s11239-008-0244-8. PMID 18685811.
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  10. 10.0 10.1 De Boer NK, Tuynman H, Bloemena E, Westerga J, Van Der Peet DL, Mulder CJ, Cuesta MA, Meuwissen SG, Van Nieuwkerk CM, Van Bodegraven AA (2008). "Histopathology of liver biopsies from a thiopurine-naïve inflammatory bowel disease cohort: prevalence of nodular regenerative hyperplasia". Scand. J. Gastroenterol. 43 (5): 604–8. doi:10.1080/00365520701800266. PMID 18415755.
  11. 11.0 11.1 Sarin SK, Mehra NK, Agarwal A, Malhotra V, Anand BS, Taneja V (1987). "Familial aggregation in noncirrhotic portal fibrosis: a report of four families". Am. J. Gastroenterol. 82 (11): 1130–3. PMID 3499813.
  12. 12.0 12.1 Imai Y, Minami Y, Miyoshi S, Kawata S, Saito R, Noda S, Tamura S, Nishikawa M, Tajima K, Tarui S (1986). "Idiopathic portal hypertension associated with Hashimoto's disease: report of three cases". Am. J. Gastroenterol. 81 (9): 791–5. PMID 2944377.
  13. 13.0 13.1 Siramolpiwat S, Seijo S, Miquel R, Berzigotti A, Garcia-Criado A, Darnell A, Turon F, Hernandez-Gea V, Bosch J, Garcia-Pagán JC (2014). "Idiopathic portal hypertension: natural history and long-term outcome". Hepatology. 59 (6): 2276–85. doi:10.1002/hep.26904. PMID 24155091.
  14. 14.0 14.1 de Lonlay P, Seta N (2009). "The clinical spectrum of phosphomannose isomerase deficiency, with an evaluation of mannose treatment for CDG-Ib". Biochim. Biophys. Acta. 1792 (9): 841–3. doi:10.1016/j.bbadis.2008.11.012. PMID 19101627.
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