Portal hypertension causes: Difference between revisions
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{{Portal hypertension}} | {{Portal hypertension}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}} | ||
==Overview== | ==Overview== | ||
Many conditions can result in portal hypertension. In North America and Europe, it is usually the result of an intrahepatic block due to [[cirrhosis]] of the [[liver]]. However, in less industrialized parts of the world, climate permitting, the major cause is [[schistosomiasis]]. | Many conditions can result in portal hypertension. In North America and Europe, it is usually the result of an intrahepatic block due to [[cirrhosis]] of the [[liver]]. However, in less industrialized parts of the world, climate permitting, the major cause is [[schistosomiasis]]. | ||
==Causes== | ==Causes== | ||
===Life-threatening Causes=== | |||
*Life-threatening causes of portal hypertension include [[cirrhosis]], severe [[Portal venous system|portal venous]] obstruction or [[thrombosis]] ([[Budd-Chiari syndrome]]), and [[fulminant hepatic failure]] (e.g., due to [[hepatitis]]). | |||
===Common Causes=== | |||
Portal hypertension may be caused by: | |||
*[[Alcoholic hepatitis]] | |||
*[[Autoimmune disease]]<ref name="pmid11831999">{{cite journal |vauthors=Li X, Gao W, Chen J, Tang W |title=[Non-cirrhotic portal hypertension associated with autoimmune disease] |language=Chinese |journal=Zhonghua Wai Ke Za Zhi |volume=38 |issue=2 |pages=101–3 |year=2000 |pmid=11831999 |doi= |url=}}</ref> | |||
*[[Bacterial]] intestinal [[Infection|infections]] | |||
** Recurrent [[Escherichia coli|E.coli]] infection<ref name="pmid3276575">{{cite journal |vauthors=Kono K, Ohnishi K, Omata M, Saito M, Nakayama T, Hatano H, Nakajima Y, Sugita S, Okuda K |title=Experimental portal fibrosis produced by intraportal injection of killed nonpathogenic Escherichia coli in rabbits |journal=Gastroenterology |volume=94 |issue=3 |pages=787–96 |year=1988 |pmid=3276575 |doi= |url=}}</ref> | |||
*[[Chronic hepatitis]] | |||
*[[Cirrhosis]] | |||
*[[Fatty liver]] | |||
*[[Schistosomiasis]] | |||
*[[Sickle cell disease]]<ref name="pmid17558079">{{cite journal |vauthors=Kumar S, Joshi R, Jain AP |title=Portal hypertension associated with sickle cell disease |journal=Indian J Gastroenterol |volume=26 |issue=2 |pages=94 |year=2007 |pmid=17558079 |doi= |url=}}</ref> | |||
===Less Common Causes=== | |||
Less common causes of portal hypertension include: | |||
*[[Antiphospholipid syndrome]]<ref name="pmid18685811">{{cite journal |vauthors=Bayan K, Tüzün Y, Yilmaz S, Canoruc N, Dursun M |title=Analysis of inherited thrombophilic mutations and natural anticoagulant deficiency in patients with idiopathic portal hypertension |journal=J. Thromb. Thrombolysis |volume=28 |issue=1 |pages=57–62 |year=2009 |pmid=18685811 |doi=10.1007/s11239-008-0244-8 |url=}}</ref> | |||
*Adams-Olivier syndrome<ref name="pmid15832360">{{cite journal |vauthors=Girard M, Amiel J, Fabre M, Pariente D, Lyonnet S, Jacquemin E |title=Adams-Oliver syndrome and hepatoportal sclerosis: occasional association or common mechanism? |journal=Am. J. Med. Genet. A |volume=135 |issue=2 |pages=186–9 |year=2005 |pmid=15832360 |doi=10.1002/ajmg.a.30724 |url=}}</ref> | |||
*[[AIDS antiretroviral drugs|Antiretroviral therapy]]<ref name="pmid18389904">{{cite journal |vauthors=Maida I, Garcia-Gasco P, Sotgiu G, Rios MJ, Vispo ME, Martin-Carbonero L, Barreiro P, Mura MS, Babudieri S, Albertos S, Garcia-Samaniego J, Soriano V |title=Antiretroviral-associated portal hypertension: a new clinical condition? Prevalence, predictors and outcome |journal=Antivir. Ther. (Lond.) |volume=13 |issue=1 |pages=103–7 |year=2008 |pmid=18389904 |doi= |url=}}</ref> | |||
*[[Arsenicals]]<ref name="pmid2398270">{{cite journal |vauthors=Nevens F, Fevery J, Van Steenbergen W, Sciot R, Desmet V, De Groote J |title=Arsenic and non-cirrhotic portal hypertension. A report of eight cases |journal=J. Hepatol. |volume=11 |issue=1 |pages=80–5 |year=1990 |pmid=2398270 |doi= |url=}}</ref> | |||
*[[Beck syndrome|Beck's disease]] | |||
*[[Common variable immunodeficiency|Common variable immunodeficiency syndrome]]<ref name="pmid23420139">{{cite journal |vauthors=Fuss IJ, Friend J, Yang Z, He JP, Hooda L, Boyer J, Xi L, Raffeld M, Kleiner DE, Heller T, Strober W |title=Nodular regenerative hyperplasia in common variable immunodeficiency |journal=J. Clin. Immunol. |volume=33 |issue=4 |pages=748–58 |year=2013 |pmid=23420139 |pmc=3731765 |doi=10.1007/s10875-013-9873-6 |url=}}</ref> | |||
* [[Connective tissue disease|Connective tissue diseases]]<ref name="pmid21393872">{{cite journal |vauthors=Vaiphei K, Bhatia A, Sinha SK |title=Liver pathology in collagen vascular disorders highlighting the vascular changes within portal tracts |journal=Indian J Pathol Microbiol |volume=54 |issue=1 |pages=25–31 |year=2011 |pmid=21393872 |doi=10.4103/0377-4929.77319 |url=}}</ref> | |||
* [[Crohn’s disease]]<ref name="pmid18415755">{{cite journal |vauthors=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 |title=Histopathology of liver biopsies from a thiopurine-naïve inflammatory bowel disease cohort: prevalence of nodular regenerative hyperplasia |journal=Scand. J. Gastroenterol. |volume=43 |issue=5 |pages=604–8 |year=2008 |pmid=18415755 |doi=10.1080/00365520701800266 |url=}}</ref> | |||
*[[Cardiomyopathy]] | |||
*[[Congestive heart failure]] | |||
*[[Constrictive pericarditis]] | |||
*Congenital abnormalities | |||
*Familial cases<ref name="pmid3499813">{{cite journal |vauthors=Sarin SK, Mehra NK, Agarwal A, Malhotra V, Anand BS, Taneja V |title=Familial aggregation in noncirrhotic portal fibrosis: a report of four families |journal=Am. J. Gastroenterol. |volume=82 |issue=11 |pages=1130–3 |year=1987 |pmid=3499813 |doi= |url=}}</ref> | |||
*[[Fulminant hepatic failure]] | |||
*[[Hashimoto's thyroiditis]]<ref name="pmid2944377">{{cite journal |vauthors=Imai Y, Minami Y, Miyoshi S, Kawata S, Saito R, Noda S, Tamura S, Nishikawa M, Tajima K, Tarui S |title=Idiopathic portal hypertension associated with Hashimoto's disease: report of three cases |journal=Am. J. Gastroenterol. |volume=81 |issue=9 |pages=791–5 |year=1986 |pmid=2944377 |doi= |url=}}</ref> | |||
*[[Hodgkin's lymphoma]] | |||
*[[Human Immunodeficiency Virus (HIV)|Human immunodeficiency virus (HIV) infection]]<ref name="pmid24155091">{{cite journal |vauthors=Siramolpiwat S, Seijo S, Miquel R, Berzigotti A, Garcia-Criado A, Darnell A, Turon F, Hernandez-Gea V, Bosch J, Garcia-Pagán JC |title=Idiopathic portal hypertension: natural history and long-term outcome |journal=Hepatology |volume=59 |issue=6 |pages=2276–85 |year=2014 |pmid=24155091 |doi=10.1002/hep.26904 |url=}}</ref> | |||
*[[Inferior vena cava obstruction]] | |||
*[[Inherited thrombophilia|Inherited thrombophilias]]<ref name="pmid18685811" /> | |||
*Idiopathic tropical [[splenomegaly]] | |||
*[[Myeloproliferative neoplasm]]<ref name="pmid18685811" /> | |||
*[[Osteomyelosclerosis]] | |||
*Partial nodular transformation | |||
*Phosphomannose isomerase deficiency<ref name="pmid19101627">{{cite journal |vauthors=de Lonlay P, Seta N |title=The clinical spectrum of phosphomannose isomerase deficiency, with an evaluation of mannose treatment for CDG-Ib |journal=Biochim. Biophys. Acta |volume=1792 |issue=9 |pages=841–3 |year=2009 |pmid=19101627 |doi=10.1016/j.bbadis.2008.11.012 |url=}}</ref> | |||
*[[Portal vein occlusion]] | |||
*[[Organ transplant|Solid organ transplant]] | |||
** [[Renal transplantation]]<ref name="pmid1438671">{{cite journal |vauthors=Allison MC, Mowat A, McCruden EA, McGregor E, Burt AD, Briggs JD, Junor BJ, Follett EA, MacSween RN, Mills PR |title=The spectrum of chronic liver disease in renal transplant recipients |journal=Q. J. Med. |volume=83 |issue=301 |pages=355–67 |year=1992 |pmid=1438671 |doi= |url=}}</ref> | |||
** [[Liver transplantation]]<ref name="pmid8020909">{{cite journal |vauthors=Gane E, Portmann B, Saxena R, Wong P, Ramage J, Williams R |title=Nodular regenerative hyperplasia of the liver graft after liver transplantation |journal=Hepatology |volume=20 |issue=1 Pt 1 |pages=88–94 |year=1994 |pmid=8020909 |doi= |url=}}</ref> | |||
*[[Splenic vein thrombosis]] | |||
*[[Thiopurine|Thiopurine derivatives]] | |||
** [[Didanosine]] | |||
** [[Azathioprine]]<ref name="pmid17504943">{{cite journal |vauthors=Vernier-Massouille G, Cosnes J, Lemann M, Marteau P, Reinisch W, Laharie D, Cadiot G, Bouhnik Y, De Vos M, Boureille A, Duclos B, Seksik P, Mary JY, Colombel JF |title=Nodular regenerative hyperplasia in patients with inflammatory bowel disease treated with azathioprine |journal=Gut |volume=56 |issue=10 |pages=1404–9 |year=2007 |pmid=17504943 |pmc=2000290 |doi=10.1136/gut.2006.114363 |url=}}</ref> | |||
** [[Thioguanine|Cis-thioguanine]]<ref name="pmid21272804">{{cite journal |vauthors=Calabrese E, Hanauer SB |title=Assessment of non-cirrhotic portal hypertension associated with thiopurine therapy in inflammatory bowel disease |journal=J Crohns Colitis |volume=5 |issue=1 |pages=48–53 |year=2011 |pmid=21272804 |doi=10.1016/j.crohns.2010.08.007 |url=}}</ref> | |||
*[[Turner syndrome]]<ref name="pmid23121401">{{cite journal |vauthors=Roulot D |title=Liver involvement in Turner syndrome |journal=Liver Int. |volume=33 |issue=1 |pages=24–30 |year=2013 |pmid=23121401 |doi=10.1111/liv.12007 |url=}}</ref> | |||
*[[Tricuspid insufficiency]] | |||
*Umbilical sepsis | |||
*[[Vitamin A]]<ref name="pmid2019375">{{cite journal |vauthors=Geubel AP, De Galocsy C, Alves N, Rahier J, Dive C |title=Liver damage caused by therapeutic vitamin A administration: estimate of dose-related toxicity in 41 cases |journal=Gastroenterology |volume=100 |issue=6 |pages=1701–9 |year=1991 |pmid=2019375 |doi= |url=}}</ref> | |||
*[[Wilson's disease]] | |||
===Genetic Causes=== | |||
Portal hypertension is caused by a [[mutation]] in these [[genes]]. | |||
*[[DGUOK|Deoxyguanosine kinase (DGUOK)]] | |||
*[[Adenosine deaminase|Adenosine deaminase (ADA)]] | |||
*[[Phospholipase A2|Phospholipase A2 (PL2G10)]] | |||
*[[CYP4F3|Cytochrome P450, family 4, subfamily F, polypeptide 3 (CYP4F3)]] | |||
*[[Glutathione peroxidase|Glutathione peroxidase 3 (GPX3)]] | |||
*[[Leukotriene B4|Leukotriene B4 (LTB4)]] | |||
*[[Prostaglandin E2 receptor|Prostaglandin E receptor 2 (PTGER2)]] | |||
*[[Endothelin|Endothelin (EDN1)]] | |||
*[[Endothelin receptor type A|Endothelin receptor type A (EDNRA)]] | |||
*[[Natriuretic peptides|Natriuretic peptide receptor 3 (NPR3)]] | |||
*[[Cluster of differentiation|Cluster of differentiation 44 (CD44)]] | |||
*[[Transforming growth factor-β|Transforming growth factor (TGF)-β]] | |||
*Ectonucleoside triphosphate diphosphohydrolase 4 (ENTPD4) | |||
*[[ABCC1|ATP-binding cassette, subfamily C, member 1 (ABCC1)]] | |||
=== Causes classified by etiology === | |||
===Prehepatic=== | ====Prehepatic==== | ||
*[[Bacterial]] intestinal [[Infection|infections]] | |||
** Recurrent [[Escherichia coli|E.coli]] infection<ref name="pmid3276575" /> | |||
*Congenital abnormalities | *Congenital abnormalities | ||
*Idiopathic tropical [[splenomegaly]] | *Idiopathic tropical [[splenomegaly]] | ||
*[[Portal vein occlusion]] | *[[Portal vein occlusion]] | ||
*[[Organ transplant|Solid organ transplant]] | |||
** [[Renal transplantation]]<ref name="pmid1438671" /> | |||
** [[Liver transplantation]]<ref name="pmid8020909" /> | |||
*[[Splenic vein thrombosis]] | *[[Splenic vein thrombosis]] | ||
*Umbilical sepsis | *Umbilical sepsis | ||
===Intrahepatic=== | ====Intrahepatic==== | ||
*Adams-Olivier syndrome<ref name="pmid15832360" /> | |||
*[[Alcoholic hepatitis]] | *[[Alcoholic hepatitis]] | ||
*[[Beck's disease]] | *[[AIDS antiretroviral drugs|Antiretroviral therapy]]<ref name="pmid18389904" /> | ||
*[[Arsenicals]]<ref name="pmid2398270" /> | |||
*[[Autoimmune disease]]<ref name="pmid11831999" /> | |||
*[[Beck syndrome|Beck's disease]] | |||
*[[Chronic hepatitis]] | *[[Chronic hepatitis]] | ||
*[[Cirrhosis]] | *[[Cirrhosis]] | ||
*[[Common variable immunodeficiency|Common variable immunodeficiency syndrome]]<ref name="pmid23420139" /> | |||
* [[Connective tissue disease|Connective tissue diseases]]<ref name="pmid21393872" /> | |||
* [[Crohn’s disease]]<ref name="pmid18415755" /> | |||
* Familial cases<ref name="pmid3499813" /> | |||
*[[Fatty liver]] | *[[Fatty liver]] | ||
*[[Fulminant hepatic failure]] | *[[Fulminant hepatic failure]] | ||
*[[Hashimoto's thyroiditis]]<ref name="pmid2944377" /> | |||
*[[Hodgkin's lymphoma]] | *[[Hodgkin's lymphoma]] | ||
*[[Human Immunodeficiency Virus (HIV)|Human immunodeficiency virus (HIV) infection]]<ref name="pmid24155091" /> | |||
*[[Osteomyelosclerosis]] | *[[Osteomyelosclerosis]] | ||
*Partial nodular transformation | *Partial nodular transformation | ||
*Phosphomannose isomerase deficiency<ref name="pmid19101627" /> | |||
*[[Schistosomiasis]] | *[[Schistosomiasis]] | ||
*[[Thiopurine|Thiopurine derivatives]] | |||
** [[Didanosine]] | |||
** [[Azathioprine]]<ref name="pmid17504943" /> | |||
** [[Thioguanine|Cis-thioguanine]]<ref name="pmid21272804" /> | |||
*[[Turner syndrome]]<ref name="pmid23121401" /> | |||
*[[Vitamin A]]<ref name="pmid2019375" /> | |||
*[[Wilson's disease]] | *[[Wilson's disease]] | ||
===Posthepatic=== | ====Posthepatic==== | ||
*[[Antiphospholipid syndrome]]<ref name="pmid18685811" /> | |||
*[[Cardiomyopathy]] | *[[Cardiomyopathy]] | ||
*[[Congestive heart failure]] | *[[Congestive heart failure]] | ||
*[[ | *[[Constrictive pericarditis]] | ||
*[[Inferior vena cava obstruction]] | *[[Inferior vena cava obstruction]] | ||
*[[Inherited thrombophilia|Inherited thrombophilias]] <ref name="pmid18685811" /> | |||
*[[Myeloproliferative neoplasm]]<ref name="pmid18685811" /> | |||
*[[Sickle cell disease]]<ref name="pmid17558079" /> | |||
*[[Tricuspid insufficiency]] | *[[Tricuspid insufficiency]] | ||
===Causes by Organ System=== | ===Causes by Organ System=== | ||
{|style="width:70%; height:100px" border="1" | {| style="width:70%; height:100px" border="1" | ||
| | | style="width:25%" bgcolor="LightSteelBlue" ; border="1" | '''Cardiovascular''' | ||
| | | style="width:75%" bgcolor="Beige" ; border="1" | [[Cardiomyopathy]], [[Congestive heart failure]], [[Constrictive pericarditis]], [[Inferior vena cava obstruction]], [[Arteriovenous fistula|Splanchnic arteriovenous fistula]], [[Tricuspid insufficiency |Tricuspid insufficiency.]] | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Chemical / | | '''Chemical/Poisoning''' | ||
|bgcolor="Beige"| | | bgcolor="Beige" | [[Arsenicals]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| ''' | | '''Dermatological''' | ||
|bgcolor="Beige"| [[NISCH syndrome]] | | bgcolor="Beige" | [[NISCH syndrome]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Drug Side Effect''' | | '''Drug Side Effect''' | ||
|bgcolor="Beige"| [[Didanosine]], [[Thioguanine]] | | bgcolor="Beige" | [[Didanosine]], [[Thioguanine]], [[Vitamin A]], [[Azathioprine]], [[Thioguanine|Cis-thioguanine]], [[AIDS antiretroviral drugs|Antiretroviral therapy]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Ear Nose Throat''' | | '''Ear Nose Throat''' | ||
|bgcolor="Beige"| No underlying causes | | bgcolor="Beige" | No underlying causes | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Endocrine''' | | '''Endocrine''' | ||
|bgcolor="Beige"| | | bgcolor="Beige" | [[Hashimoto's thyroiditis]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Environmental''' | | '''Environmental''' | ||
|bgcolor="Beige"| | | bgcolor="Beige" | [[Vitamin A]], [[Alcoholic hepatitis]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| ''' | | '''Gastroenterology''' | ||
|bgcolor="Beige"| [[Alcoholic hepatitis]], [[Biliary atresia]], [[Budd-Chiari syndrome]], [[Cholestasis]], [[Chronic hepatitis]], [[Chronic liver disease]], [[Cirrhosis]], | | 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 splenomegaly|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]], [[Metastasis|Hepatic metastasis]], [[Pancreatic cancer]], [[NISCH syndrome]], [[Crohn’s disease]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Genetic''' | | '''Genetic''' | ||
|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]] | | bgcolor="Beige" | [[Alpha-1-antitrypsin deficiency]], [[COACH syndrome]], [[Cystic fibrosis]], [[Gaucher disease]], [[Glycosylphosphatidylinositol|Glycosylphosphatidylinositol deficiency]], [[Hemochromatosis]], [[Interferon gamma receptor 1|Interferon gamma receptor 1 deficiency]], [[NISCH syndrome]], [[Progressive familial intrahepatic cholestasis]], [[Wilson's Disease ]] , [[Hepatic venoocclusive disease with immunodeficiency]], [[Turner syndrome]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Hematologic''' | | '''Hematologic''' | ||
|bgcolor="Beige"| [[Blackfan Diamond anemia]], [[Congenital pure red cell aplasia ]], [[Myeloproliferative diseases]], [[Mosse syndrome]], [[Hemochromatosis]], [[Osteomyelosclerosis]], [[Hodgkin's lymphoma]] | | bgcolor="Beige" | [[Blackfan-Diamond Syndrome|Blackfan Diamond anemia]], [[Congenital pure red cell aplasia ]] , [[Myeloproliferative diseases]], [[Mosse syndrome]], [[Hemochromatosis]], [[Osteomyelosclerosis]], [[Hodgkin's lymphoma]], [[Sickle cell disease]], [[Inherited thrombophilia|Inherited thrombophilias]], [[Antiphospholipid syndrome]] , [[Human Immunodeficiency Virus (HIV)|Human immunodeficiency virus (HIV) infection]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Iatrogenic''' | | '''Iatrogenic''' | ||
|bgcolor="Beige"| | | bgcolor="Beige" | [[Organ transplant|Solid organ transplant]], [[Renal transplantation]], [[Liver transplantation]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Infectious Disease''' | | '''Infectious Disease''' | ||
|bgcolor="Beige"| [[Schistosomiasis ]], [[ | | bgcolor="Beige" | [[Schistosomiasis ]] , Umbilical sepsis, [[Viral hepatitis]], Recurrent [[Escherichia coli|E.coli]] infection. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Musculoskeletal / Ortho''' | | '''Musculoskeletal / Ortho''' | ||
|bgcolor="Beige"| [[Osteomyelosclerosis]] | | bgcolor="Beige" | [[Osteomyelosclerosis]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Neurologic''' | | '''Neurologic''' | ||
|bgcolor="Beige"| [[Berry aneurysm]], [[Cerebral calcification]] | | bgcolor="Beige" | [[Berry aneurysm]], [[Cerebral]] [[calcification]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Nutritional / Metabolic''' | | '''Nutritional / Metabolic''' | ||
|bgcolor="Beige"| [[Gaucher disease]], [[Glycosylphosphatidylinositol deficiency]], [[Wilson's disease ]] | | bgcolor="Beige" | [[Gaucher disease]], [[Glycosylphosphatidylinositol|Glycosylphosphatidylinositol deficiency]], [[Wilson's disease |Wilson's disease,]] [[Vitamin A]], [[Alcoholic hepatitis]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Obstetric/Gynecologic''' | | '''Obstetric/Gynecologic''' | ||
|bgcolor="Beige"| | | bgcolor="Beige" | [[Antiphospholipid syndrome]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Oncologic''' | | '''Oncologic''' | ||
|bgcolor="Beige"| | | bgcolor="Beige" | Extrinsic compression ([[Tumor|tumors]]), Hepatic metastasis, [[Hodgkin's lymphoma]], [[Pancreatic cancer]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Opthalmologic''' | | '''Opthalmologic''' | ||
|bgcolor="Beige"| No underlying causes | | bgcolor="Beige" | No underlying causes | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Overdose / Toxicity''' | | '''Overdose / Toxicity''' | ||
|bgcolor="Beige"| [[Didanosine]], [[Thioguanine]] | | bgcolor="Beige" | [[Didanosine]], [[Thioguanine]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Psychiatric''' | | '''Psychiatric''' | ||
|bgcolor="Beige"| No underlying causes | | bgcolor="Beige" | No underlying causes | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Pulmonary''' | | '''Pulmonary''' | ||
|bgcolor="Beige"| [[Cystic fibrosis]], [[Alpha-1-antitrypsin deficiency]], [[Berry aneurysm]], [[Cirrhosis]], [[Pulmonary emphysema]], | | bgcolor="Beige" | [[Cystic fibrosis]], [[Alpha-1-antitrypsin deficiency]], [[Berry aneurysm]], [[Cirrhosis]], [[Pulmonary emphysema]], [[Cerebral]] [[calcification]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Renal / Electrolyte''' | | '''Renal / Electrolyte''' | ||
|bgcolor="Beige"| [[Polycystic kidney disease]] | | bgcolor="Beige" | [[Polycystic kidney disease]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Rheum / Immune / Allergy''' | | '''Rheum / Immune / Allergy''' | ||
|bgcolor="Beige"| [[Hepatic venoocclusive disease with immunodeficiency]], [[Granulomatous diseases]] ([[Sarcoidosis]], [[Tuberculosis)]]] | | bgcolor="Beige" | [[Hepatic venoocclusive disease with immunodeficiency]], [[Granulomatous Hepatitis|Granulomatous diseases]] ([[Sarcoidosis]], [[Tuberculosis]])], [[Hashimoto's thyroiditis]]. | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Sexual''' | | '''Sexual''' | ||
|bgcolor="Beige"| No underlying causes | | bgcolor="Beige" | No underlying causes | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Trauma''' | | '''Trauma''' | ||
|bgcolor="Beige"| No underlying causes | | bgcolor="Beige" | No underlying causes | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Urologic''' | | '''Urologic''' | ||
|bgcolor="Beige"| No underlying causes | | bgcolor="Beige" | No underlying causes | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Dental''' | | '''Dental''' | ||
|bgcolor="Beige"| No underlying causes | | bgcolor="Beige" | No underlying causes | ||
|- | |- | ||
|-bgcolor="LightSteelBlue" | |- bgcolor="LightSteelBlue" | ||
| '''Miscellaneous''' | | '''Miscellaneous''' | ||
|bgcolor="Beige"| Granulomatous diseases ([[Sarcoidosis]], [[Tuberculosis]]), | | bgcolor="Beige" | [[Granulomatous Hepatitis|Granulomatous diseases]] ([[Sarcoidosis]], [[Tuberculosis]]), Extrinsic compression ([[tumors]]), [[Inferior vena cava obstruction]]. | ||
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Revision as of 16:00, 27 October 2017
Portal Hypertension Microchapters |
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Portal hypertension causes On the Web |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Many conditions can result in portal hypertension. In North America and Europe, it is usually the result of an intrahepatic block due to cirrhosis of the liver. However, in less industrialized parts of the world, climate permitting, the major cause is schistosomiasis.
Causes
Life-threatening Causes
- 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
Portal hypertension may be caused by:
Less Common Causes
Less common causes of portal hypertension include:
- Antiphospholipid syndrome[4]
- Adams-Olivier syndrome[5]
- Antiretroviral therapy[6]
- Arsenicals[7]
- Beck's disease
- Common variable immunodeficiency syndrome[8]
- Connective tissue diseases[9]
- Crohn’s disease[10]
- Cardiomyopathy
- Congestive heart failure
- Constrictive pericarditis
- Congenital abnormalities
- Familial cases[11]
- Fulminant hepatic failure
- Hashimoto's thyroiditis[12]
- Hodgkin's lymphoma
- Human immunodeficiency virus (HIV) infection[13]
- Inferior vena cava obstruction
- Inherited thrombophilias[4]
- Idiopathic tropical splenomegaly
- Myeloproliferative neoplasm[4]
- Osteomyelosclerosis
- Partial nodular transformation
- Phosphomannose isomerase deficiency[14]
- Portal vein occlusion
- Solid organ transplant
- Splenic vein thrombosis
- Thiopurine derivatives
- Turner syndrome[19]
- Tricuspid insufficiency
- Umbilical sepsis
- Vitamin A[20]
- Wilson's disease
Genetic Causes
Portal hypertension is caused by a mutation in these genes.
- Deoxyguanosine kinase (DGUOK)
- Adenosine deaminase (ADA)
- Phospholipase A2 (PL2G10)
- Cytochrome P450, family 4, subfamily F, polypeptide 3 (CYP4F3)
- Glutathione peroxidase 3 (GPX3)
- Leukotriene B4 (LTB4)
- Prostaglandin E receptor 2 (PTGER2)
- Endothelin (EDN1)
- Endothelin receptor type A (EDNRA)
- Natriuretic peptide receptor 3 (NPR3)
- Cluster of differentiation 44 (CD44)
- Transforming growth factor (TGF)-β
- Ectonucleoside triphosphate diphosphohydrolase 4 (ENTPD4)
- ATP-binding cassette, subfamily C, member 1 (ABCC1)
Causes classified by etiology
Prehepatic
- Bacterial intestinal infections
- Congenital abnormalities
- Idiopathic tropical splenomegaly
- Portal vein occlusion
- Solid organ transplant
- Splenic vein thrombosis
- Umbilical sepsis
Intrahepatic
- Adams-Olivier syndrome[5]
- Alcoholic hepatitis
- Antiretroviral therapy[6]
- Arsenicals[7]
- Autoimmune disease[1]
- Beck's disease
- Chronic hepatitis
- Cirrhosis
- Common variable immunodeficiency syndrome[8]
- Connective tissue diseases[9]
- Crohn’s disease[10]
- Familial cases[11]
- Fatty liver
- Fulminant hepatic failure
- Hashimoto's thyroiditis[12]
- Hodgkin's lymphoma
- Human immunodeficiency virus (HIV) infection[13]
- Osteomyelosclerosis
- Partial nodular transformation
- Phosphomannose isomerase deficiency[14]
- Schistosomiasis
- Thiopurine derivatives
- Turner syndrome[19]
- Vitamin A[20]
- Wilson's disease
Posthepatic
- Antiphospholipid syndrome[4]
- Cardiomyopathy
- Congestive heart failure
- Constrictive pericarditis
- Inferior vena cava obstruction
- Inherited thrombophilias [4]
- Myeloproliferative neoplasm[4]
- Sickle cell disease[3]
- Tricuspid insufficiency
Causes by Organ System
Causes in Alphabetical Order
References
- ↑ 1.0 1.1 Li X, Gao W, Chen J, Tang W (2000). "[Non-cirrhotic portal hypertension associated with autoimmune disease]". Zhonghua Wai Ke Za Zhi (in Chinese). 38 (2): 101–3. PMID 11831999.
- ↑ 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.
- ↑ 3.0 3.1 Kumar S, Joshi R, Jain AP (2007). "Portal hypertension associated with sickle cell disease". Indian J Gastroenterol. 26 (2): 94. PMID 17558079.
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 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.
- ↑ 5.0 5.1 Girard M, Amiel J, Fabre M, Pariente D, Lyonnet S, Jacquemin E (2005). "Adams-Oliver syndrome and hepatoportal sclerosis: occasional association or common mechanism?". Am. J. Med. Genet. A. 135 (2): 186–9. doi:10.1002/ajmg.a.30724. PMID 15832360.
- ↑ 6.0 6.1 Maida I, Garcia-Gasco P, Sotgiu G, Rios MJ, Vispo ME, Martin-Carbonero L, Barreiro P, Mura MS, Babudieri S, Albertos S, Garcia-Samaniego J, Soriano V (2008). "Antiretroviral-associated portal hypertension: a new clinical condition? Prevalence, predictors and outcome". Antivir. Ther. (Lond.). 13 (1): 103–7. PMID 18389904.
- ↑ 7.0 7.1 Nevens F, Fevery J, Van Steenbergen W, Sciot R, Desmet V, De Groote J (1990). "Arsenic and non-cirrhotic portal hypertension. A report of eight cases". J. Hepatol. 11 (1): 80–5. PMID 2398270.
- ↑ 8.0 8.1 Fuss IJ, Friend J, Yang Z, He JP, Hooda L, Boyer J, Xi L, Raffeld M, Kleiner DE, Heller T, Strober W (2013). "Nodular regenerative hyperplasia in common variable immunodeficiency". J. Clin. Immunol. 33 (4): 748–58. doi:10.1007/s10875-013-9873-6. PMC 3731765. PMID 23420139.
- ↑ 9.0 9.1 Vaiphei K, Bhatia A, Sinha SK (2011). "Liver pathology in collagen vascular disorders highlighting the vascular changes within portal tracts". Indian J Pathol Microbiol. 54 (1): 25–31. doi:10.4103/0377-4929.77319. PMID 21393872.
- ↑ 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.
- ↑ 11.0 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.
- ↑ 12.0 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.
- ↑ 13.0 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.
- ↑ 14.0 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.
- ↑ 15.0 15.1 Allison MC, Mowat A, McCruden EA, McGregor E, Burt AD, Briggs JD, Junor BJ, Follett EA, MacSween RN, Mills PR (1992). "The spectrum of chronic liver disease in renal transplant recipients". Q. J. Med. 83 (301): 355–67. PMID 1438671.
- ↑ 16.0 16.1 Gane E, Portmann B, Saxena R, Wong P, Ramage J, Williams R (1994). "Nodular regenerative hyperplasia of the liver graft after liver transplantation". Hepatology. 20 (1 Pt 1): 88–94. PMID 8020909.
- ↑ 17.0 17.1 Vernier-Massouille G, Cosnes J, Lemann M, Marteau P, Reinisch W, Laharie D, Cadiot G, Bouhnik Y, De Vos M, Boureille A, Duclos B, Seksik P, Mary JY, Colombel JF (2007). "Nodular regenerative hyperplasia in patients with inflammatory bowel disease treated with azathioprine". Gut. 56 (10): 1404–9. doi:10.1136/gut.2006.114363. PMC 2000290. PMID 17504943.
- ↑ 18.0 18.1 Calabrese E, Hanauer SB (2011). "Assessment of non-cirrhotic portal hypertension associated with thiopurine therapy in inflammatory bowel disease". J Crohns Colitis. 5 (1): 48–53. doi:10.1016/j.crohns.2010.08.007. PMID 21272804.
- ↑ 19.0 19.1 Roulot D (2013). "Liver involvement in Turner syndrome". Liver Int. 33 (1): 24–30. doi:10.1111/liv.12007. PMID 23121401.
- ↑ 20.0 20.1 Geubel AP, De Galocsy C, Alves N, Rahier J, Dive C (1991). "Liver damage caused by therapeutic vitamin A administration: estimate of dose-related toxicity in 41 cases". Gastroenterology. 100 (6): 1701–9. PMID 2019375.