Portal hypertension causes: Difference between revisions

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===Genetic Causes===
===Genetic Causes===
Portal hypertension is caused by a [[mutation]] in these [[genes]].
Portal hypertension is caused by a [[mutation]] in the following [[genes]]:<ref name="pmid15148269" /><ref name="pmid11687800">{{cite journal |vauthors=Mandel H, Szargel R, Labay V, Elpeleg O, Saada A, Shalata A, Anbinder Y, Berkowitz D, Hartman C, Barak M, Eriksson S, Cohen N |title=The deoxyguanosine kinase gene is mutated in individuals with depleted hepatocerebral mitochondrial DNA |journal=Nat. Genet. |volume=29 |issue=3 |pages=337–41 |year=2001 |pmid=11687800 |doi=10.1038/ng746 |url=}}</ref><ref name="pmid12897202">{{cite journal |vauthors=Blackburn MR, Lee CG, Young HW, Zhu Z, Chunn JL, Kang MJ, Banerjee SK, Elias JA |title=Adenosine mediates IL-13-induced inflammation and remodeling in the lung and interacts in an IL-13-adenosine amplification pathway |journal=J. Clin. Invest. |volume=112 |issue=3 |pages=332–44 |year=2003 |pmid=12897202 |pmc=166289 |doi=10.1172/JCI16815 |url=}}</ref><ref name="KotaniKawabe2015">{{cite journal|last1=Kotani|first1=Kohei|last2=Kawabe|first2=Joji|last3=Morikawa|first3=Hiroyasu|last4=Akahoshi|first4=Tomohiko|last5=Hashizume|first5=Makoto|last6=Shiomi|first6=Susumu|title=Comprehensive Screening of Gene Function and Networks by DNA Microarray Analysis in Japanese Patients with Idiopathic Portal Hypertension|journal=Mediators of Inflammation|volume=2015|year=2015|pages=1–10|issn=0962-9351|doi=10.1155/2015/349215}}</ref><ref name="pmid1339300">{{cite journal |vauthors=Chu FF, Esworthy RS, Doroshow JH, Doan K, Liu XF |title=Expression of plasma glutathione peroxidase in human liver in addition to kidney, heart, lung, and breast in humans and rodents |journal=Blood |volume=79 |issue=12 |pages=3233–8 |year=1992 |pmid=1339300 |doi= |url=}}</ref><ref name="pmid9177352">{{cite journal |vauthors=Yokomizo T, Izumi T, Chang K, Takuwa Y, Shimizu T |title=A G-protein-coupled receptor for leukotriene B4 that mediates chemotaxis |journal=Nature |volume=387 |issue=6633 |pages=620–4 |year=1997 |pmid=9177352 |doi=10.1038/42506 |url=}}</ref><ref name="pmid15148269">{{cite journal |vauthors=Campia U, Cardillo C, Panza JA |title=Ethnic differences in the vasoconstrictor activity of endogenous endothelin-1 in hypertensive patients |journal=Circulation |volume=109 |issue=25 |pages=3191–5 |year=2004 |pmid=15148269 |doi=10.1161/01.CIR.0000130590.24107.D3 |url=}}</ref><ref name="pmid7477288">{{cite journal |vauthors=Lopez MJ, Wong SK, Kishimoto I, Dubois S, Mach V, Friesen J, Garbers DL, Beuve A |title=Salt-resistant hypertension in mice lacking the guanylyl cyclase-A receptor for atrial natriuretic peptide |journal=Nature |volume=378 |issue=6552 |pages=65–8 |year=1995 |pmid=7477288 |doi=10.1038/378065a0 |url=}}</ref><ref name="pmid1694723">{{cite journal |vauthors=Aruffo A, Stamenkovic I, Melnick M, Underhill CB, Seed B |title=CD44 is the principal cell surface receptor for hyaluronate |journal=Cell |volume=61 |issue=7 |pages=1303–13 |year=1990 |pmid=1694723 |doi= |url=}}</ref><ref name="pmid11586292">{{cite journal |vauthors=Derynck R, Akhurst RJ, Balmain A |title=TGF-beta signaling in tumor suppression and cancer progression |journal=Nat. Genet. |volume=29 |issue=2 |pages=117–29 |year=2001 |pmid=11586292 |doi=10.1038/ng1001-117 |url=}}</ref><ref name="pmid1360704">{{cite journal |vauthors=Cole SP, Bhardwaj G, Gerlach JH, Mackie JE, Grant CE, Almquist KC, Stewart AJ, Kurz EU, Duncan AM, Deeley RG |title=Overexpression of a transporter gene in a multidrug-resistant human lung cancer cell line |journal=Science |volume=258 |issue=5088 |pages=1650–4 |year=1992 |pmid=1360704 |doi= |url=}}</ref>
*[[Leukotriene B4|Leukotriene B4 (LTB4)]]
*Ectonucleoside triphosphate diphosphohydrolase 4 (ENTPD4)
*[[Endothelin receptor type A|Endothelin receptor type A (EDNRA)]]
*[[DGUOK|Deoxyguanosine kinase (DGUOK)]]
*[[DGUOK|Deoxyguanosine kinase (DGUOK)]]
*[[Adenosine deaminase|Adenosine deaminase (ADA)]]
*[[Adenosine deaminase|Adenosine deaminase (ADA)]]
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*[[CYP4F3|Cytochrome P450, family 4, subfamily F, polypeptide 3 (CYP4F3)]]
*[[CYP4F3|Cytochrome P450, family 4, subfamily F, polypeptide 3 (CYP4F3)]]
*[[Glutathione peroxidase|Glutathione peroxidase 3 (GPX3)]]
*[[Glutathione peroxidase|Glutathione peroxidase 3 (GPX3)]]
*[[Leukotriene B4|Leukotriene B4 (LTB4)]]
*[[Prostaglandin E2 receptor|Prostaglandin E receptor 2 (PTGER2)]]
*[[Prostaglandin E2 receptor|Prostaglandin E receptor 2 (PTGER2)]]
*[[Endothelin|Endothelin (EDN1)]]
*[[Endothelin|Endothelin (EDN1)]]
*[[Endothelin receptor type A|Endothelin receptor type A (EDNRA)]]
*[[Natriuretic peptides|Natriuretic peptide receptor 3 (NPR3)]]
*[[Natriuretic peptides|Natriuretic peptide receptor 3 (NPR3)]]
*[[Cluster of differentiation|Cluster of differentiation 44 (CD44)]]
*[[Cluster of differentiation|Cluster of differentiation 44 (CD44)]]
*[[Transforming growth factor-β|Transforming growth factor (TGF)-β]]
*[[Transforming growth factor-β|Transforming growth factor (TGF)-β]]
*Ectonucleoside triphosphate diphosphohydrolase 4 (ENTPD4)
*[[ABCC1|ATP-binding cassette, subfamily C, member 1 (ABCC1)]]
*[[ABCC1|ATP-binding cassette, subfamily C, member 1 (ABCC1)]]


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====Prehepatic====
====Prehepatic====
Prehepatic causes of portal hypertension include:<ref name="pmid3276575" /><ref name="pmid1438671" /><ref name="pmid8020909" />
*[[Bacterial]] intestinal [[Infection|infections]]
*[[Bacterial]] intestinal [[Infection|infections]]
** Recurrent [[Escherichia coli|E.coli]] infection<ref name="pmid3276575" />
** Recurrent [[Escherichia coli|E.coli]] infection
*Congenital abnormalities
*Congenital abnormalities
*Idiopathic tropical [[splenomegaly]]
*Idiopathic tropical [[splenomegaly]]
*[[Portal vein occlusion]]
*[[Portal vein occlusion]]
*[[Organ transplant|Solid organ transplant]]
*[[Organ transplant|Solid organ transplant]]
** [[Renal transplantation]]<ref name="pmid1438671" />
** [[Renal transplantation]]
** [[Liver transplantation]]<ref name="pmid8020909" />
** [[Liver transplantation]]
*[[Splenic vein thrombosis]]
*[[Splenic vein thrombosis]]
*Umbilical sepsis
*Umbilical sepsis


====Intrahepatic====
====Intrahepatic====
*Adams-Olivier syndrome<ref name="pmid15832360" />
Intrahepatic causes of portal hypertension include:<ref name="pmid11831999" /><ref name="pmid15832360" /><ref name="pmid18389904" /><ref name="pmid2398270" /><ref name="pmid23420139" /><ref name="pmid21393872" /><ref name="pmid18415755" /><ref name="pmid3499813" /><ref name="pmid2944377" /><ref name="pmid24155091" /><ref name="pmid19101627" /><ref name="pmid17504943" /><ref name="pmid21272804" /><ref name="pmid23121401" /><ref name="pmid2019375" />
*Adams-Olivier syndrome
*[[Alcoholic hepatitis]]
*[[Alcoholic hepatitis]]
*[[AIDS antiretroviral drugs|Antiretroviral therapy]]<ref name="pmid18389904" />
*[[AIDS antiretroviral drugs|Antiretroviral therapy]]
*[[Arsenicals]]<ref name="pmid2398270" />
*[[Arsenicals]]
*[[Autoimmune disease]]<ref name="pmid11831999" />
*[[Autoimmune disease]]
*[[Beck syndrome|Beck's disease]]
*[[Beck syndrome|Beck's disease]]
*[[Chronic hepatitis]]
*[[Chronic hepatitis]]
*[[Cirrhosis]]
*[[Cirrhosis]]
*[[Common variable immunodeficiency|Common variable immunodeficiency syndrome]]<ref name="pmid23420139" />
*[[Common variable immunodeficiency|Common variable immunodeficiency syndrome]]
* [[Connective tissue disease|Connective tissue diseases]]<ref name="pmid21393872" />
* [[Connective tissue disease|Connective tissue diseases]]
* [[Crohn’s disease]]<ref name="pmid18415755" />
* [[Crohn’s disease]]
* Familial cases<ref name="pmid3499813" />
* Familial cases
*[[Fatty liver]]
*[[Fatty liver]]
*[[Fulminant hepatic failure]]
*[[Fulminant hepatic failure]]
*[[Hashimoto's thyroiditis]]<ref name="pmid2944377" />
*[[Hashimoto's thyroiditis]]
*[[Hodgkin's lymphoma]]
*[[Hodgkin's lymphoma]]
*[[Human Immunodeficiency Virus (HIV)|Human immunodeficiency virus (HIV) infection]]<ref name="pmid24155091" />
*[[Human Immunodeficiency Virus (HIV)|Human immunodeficiency virus (HIV) infection]]
*[[Osteomyelosclerosis]]
*[[Osteomyelosclerosis]]
*Partial nodular transformation
*Partial nodular transformation
*Phosphomannose isomerase deficiency<ref name="pmid19101627" />
*Phosphomannose isomerase deficiency
*[[Schistosomiasis]]
*[[Schistosomiasis]]
*[[Thiopurine|Thiopurine derivatives]]
*[[Thiopurine|Thiopurine derivatives]]
** [[Didanosine]]
** [[Didanosine]]
** [[Azathioprine]]<ref name="pmid17504943" />
** [[Azathioprine]]
** [[Thioguanine|Cis-thioguanine]]<ref name="pmid21272804" />
** [[Thioguanine|Cis-thioguanine]]
*[[Turner syndrome]]<ref name="pmid23121401" />
*[[Turner syndrome]]
*[[Vitamin A]]<ref name="pmid2019375" />
*[[Vitamin A]]
*[[Wilson's disease]]
*[[Wilson's disease]]


====Posthepatic====
====Posthepatic====
*[[Antiphospholipid syndrome]]<ref name="pmid18685811" />
Posthepatic causes of portal hypertension include:<ref name="pmid17558079" /><ref name="pmid18685811" />
*[[Antiphospholipid syndrome]]
*[[Cardiomyopathy]]
*[[Cardiomyopathy]]
*[[Congestive heart failure]]
*[[Congestive heart failure]]
*[[Constrictive pericarditis]]
*[[Constrictive pericarditis]]
*[[Inferior vena cava obstruction]]
*[[Inferior vena cava obstruction]]
*[[Inherited thrombophilia|Inherited thrombophilias]] <ref name="pmid18685811" />
*[[Inherited thrombophilia|Inherited thrombophilias]]
*[[Myeloproliferative neoplasm]]<ref name="pmid18685811" />
*[[Myeloproliferative neoplasm]]
*[[Sickle cell disease]]<ref name="pmid17558079" />
*[[Sickle cell disease]]
*[[Tricuspid insufficiency]]
*[[Tricuspid insufficiency]]


===Causes by Organ System===
===Causes by Organ System===
{| style="width:70%; height:100px" border="1"
{| border="1" height:100px "
| style="width:25%" bgcolor="LightSteelBlue" ; border="1" | '''Cardiovascular'''
| 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.]]
| 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.]]
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|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
| '''Drug Side Effect'''
| '''Drug Side Effect'''
| bgcolor="Beige" | [[Didanosine]], [[Thioguanine]], [[Vitamin A]], [[Azathioprine]], [[Thioguanine|Cis-thioguanine]], [[AIDS antiretroviral drugs|Antiretroviral therapy]].
| bgcolor="Beige" | [[Didanosine]], [[Thioguanine]], [[Vitamin A]], [[Azathioprine]], [[Thioguanine|Cis-thioguanine]], [[AIDS antiretroviral drugs|Antiretroviral therapy]], Adams-Olivier syndrome.
|-
|-
|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
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|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
| '''Gastroenterology'''
| '''Gastroenterology'''
| 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="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 [[Hepatocellular carcinoma|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|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="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]], [[Leukotriene B4|Leukotriene B4 (LTB4)]] [[mutation]], Ectonucleoside triphosphate diphosphohydrolase 4 (ENTPD4) [[mutation]], [[Endothelin receptor type A|Endothelin receptor type A (EDNRA)]] [[mutation]], [[DGUOK|Deoxyguanosine kinase (DGUOK)]] [[mutation]], [[Adenosine deaminase|Adenosine deaminase (ADA)]] [[mutation]], [[Phospholipase A2|Phospholipase A2 (PL2G10)]] [[mutation]], [[CYP4F3|Cytochrome P450, family 4, subfamily F, polypeptide 3 (CYP4F3)]] [[mutation]], [[Glutathione peroxidase|Glutathione peroxidase 3 (GPX3)]] [[mutation]], [[Prostaglandin E2 receptor|Prostaglandin E receptor 2 (PTGER2)]] [[mutation]], [[Endothelin|Endothelin (EDN1)]] [[mutation]], [[Natriuretic peptides|Natriuretic peptide receptor 3 (NPR3)]] [[mutation]], [[Cluster of differentiation|Cluster of differentiation 44 (CD44)]] [[mutation]], [[Transforming growth factor-β|Transforming growth factor (TGF)-β]] [[mutation]], [[ABCC1|ATP-binding cassette, subfamily C, member 1 (ABCC1)]] [[mutation]], Phosphomannose isomerase deficiency
 
|-
|-
|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
| '''Hematologic'''
| '''Hematologic'''
| 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="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]], [[Myeloproliferative neoplasm]], [[Common variable immunodeficiency|Common variable immunodeficiency syndrome]].
|-
|-
|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
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|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
| '''Musculoskeletal / Ortho'''
| '''Musculoskeletal / Ortho'''
| bgcolor="Beige" | [[Osteomyelosclerosis]].
| bgcolor="Beige" | [[Osteomyelosclerosis]], [[Connective tissue disease|Connective tissue diseases]].
|-
|-
|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
| '''Neurologic'''
| '''Neurologic'''
| bgcolor="Beige" | [[Berry aneurysm]], [[Cerebral]] [[calcification]].
| bgcolor="Beige" | [[Berry aneurysm]], [[Cerebral]] [[calcification]], [[Beck syndrome|Beck's disease]].
|-
|-
|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
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|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
| '''Oncologic'''
| '''Oncologic'''
| bgcolor="Beige" | Extrinsic compression ([[Tumor|tumors]]), Hepatic metastasis, [[Hodgkin's lymphoma]], [[Pancreatic cancer]].
| bgcolor="Beige" | Extrinsic compression ([[Tumor|tumors]]), [[Hepatic]] [[metastasis]], [[Hodgkin's lymphoma]], [[Pancreatic cancer]].
|-
|-
|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
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|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
| '''Rheum / Immune / Allergy'''
| '''Rheum / Immune / Allergy'''
| bgcolor="Beige" | [[Hepatic venoocclusive disease with immunodeficiency]], [[Granulomatous Hepatitis|Granulomatous diseases]] ([[Sarcoidosis]], [[Tuberculosis]])], [[Hashimoto's thyroiditis]].
| bgcolor="Beige" | [[Hepatic venoocclusive disease with immunodeficiency]], [[Granulomatous Hepatitis|Granulomatous diseases]] ([[Sarcoidosis]], [[Tuberculosis]]), [[Hashimoto's thyroiditis]].
|-
|-
|- bgcolor="LightSteelBlue"
|- bgcolor="LightSteelBlue"
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===Causes in Alphabetical Order===
===Causes in Alphabetical Order===
{{MultiCol}}
{{columns-list|
*[[ABCC1|ATP-binding cassette, subfamily C, member 1 (ABCC1)]] [[mutation]]
*Adams-Olivier syndrome
*[[Adenosine deaminase|Adenosine deaminase (ADA)]] [[mutation]]
*[[Alcoholic hepatitis]]
*[[Alcoholic hepatitis]]
*[[Alpha-1-antitrypsin deficiency]]
*[[Alpha-1-antitrypsin deficiency]]
*[[Antiphospholipid syndrome]]
*[[Antiretroviral therapy]]
*[[Arsenicals]]
*[[Autoimmune disease]]
*[[Azathioprine]]
*[[Beck's disease]]
*[[Berry aneurysm]]
*[[Berry aneurysm]]
*[[Biliary atresia]]
*[[Biliary atresia]]
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*[[Chronic hepatitis]]
*[[Chronic hepatitis]]
*[[Chronic liver disease]]
*[[Chronic liver disease]]
*[[Cis-thioguanine]]
*[[Crohn’s disease]]
*[[Cirrhosis]]
*[[Cirrhosis]]
*[[Cluster of differentiation|Cluster of differentiation 44 (CD44)]] [[mutation]]
*[[COACH syndrome]]
*[[COACH syndrome]]
*[[Common variable immunodeficiency syndrome]]
*[[Congenital]] [[atresia]] or [[stenosis]] of [[portal vein]]
*[[Congenital]] [[atresia]] or [[stenosis]] of [[portal vein]]
*[[Congenital hepatic fibrosis]]
*[[Congenital hepatic fibrosis]]
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*[[Congenital pure red cell aplasia ]]
*[[Congenital pure red cell aplasia ]]
*[[Congestive heart failure]]
*[[Congestive heart failure]]
*[[Connective tissue diseases]]
*[[Constrictive pericarditis]]
*[[Constrictive pericarditis]]
*[[Cruveilhier-Baumgarten syndrome]]
*[[Cruveilhier-Baumgarten syndrome]]
*[[Cystic fibrosis]]
*[[Cystic fibrosis]]
*[[CYP4F3|Cytochrome P450, family 4, subfamily F, polypeptide 3 (CYP4F3)]] [[mutation]]
*[[Didanosine]]
*[[Didanosine]]
*[[DGUOK|Deoxyguanosine kinase (DGUOK)]] [[mutation]]
*Extrinsic compression ([[tumors]])
*Extrinsic compression ([[tumors]])
*Ectonucleoside triphosphate diphosphohydrolase 4 (ENTPD4) [[mutation]]
*[[Endothelin|Endothelin (EDN1)]] [[mutation]]
*[[Endothelin receptor type A|Endothelin receptor type A (EDNRA)]] [[mutation]]
*[[Fatty liver]]
*[[Fatty liver]]
*[[Focal nodular hyperplasia]]
*[[Focal nodular hyperplasia]]
*[[Fulminant hepatic failure]]
*[[Fulminant hepatic failure]]
*[[Gaucher disease]]
*[[Gaucher disease]]
*[[Glutathione peroxidase|Glutathione peroxidase 3 (GPX3)]] [[mutation]]
*[[Glycosylphosphatidylinositol|Glycosylphosphatidylinositol deficiency]]
*[[Glycosylphosphatidylinositol|Glycosylphosphatidylinositol deficiency]]
*[[Granulomatous Hepatitis|Granulomatous diseases]] ([[Sarcoidosis]], [[Tuberculosis]])
*[[Granulomatous Hepatitis|Granulomatous diseases]] ([[Sarcoidosis]], [[Tuberculosis]])
*[[Hashimoto's thyroiditis]]
*[[Hemochromatosis]]
*[[Hemochromatosis]]
*[[Hepatic amyloidosis with intrahepatic cholestasis]]
*[[Hepatic amyloidosis with intrahepatic cholestasis]]
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*[[Hepatic vein]] occlusion
*[[Hepatic vein]] occlusion
*[[Hepatic vein thrombosis]]
*[[Hepatic vein thrombosis]]
{{ColBreak}}
*[[Hepatic venoocclusive disease with immunodeficiency]]
*[[Hepatic venoocclusive disease with immunodeficiency]]
*[[Hodgkin's lymphoma]]
*[[Hodgkin's lymphoma]]
*[[Human immunodeficiency virus (HIV) infection]]
*[[Idiopathic liver cirrhosis]]
*[[Idiopathic liver cirrhosis]]
*[[Idiopathic]] [[portal hypertension]]
*[[Idiopathic]] [[portal hypertension]]
*[[Idiopathic]] tropical [[splenomegaly]]
*[[Idiopathic]] tropical [[splenomegaly]]
*[[Inferior vena cava obstruction]]
*[[Inferior vena cava obstruction]]
*[[Inherited thrombophilia]]
*[[Interferon gamma receptor 1|Interferon gamma  receptor 1 deficiency]]
*[[Interferon gamma receptor 1|Interferon gamma  receptor 1 deficiency]]
*[[Liver]] [[fibrosis]]
*[[Liver]] [[fibrosis]]
*[[Liver transplantation]]
*[[Leukotriene B4|Leukotriene B4 (LTB4)]] [[mutation]]
*[[Mosse syndrome]]
*[[Mosse syndrome]]
*[[Myeloproliferative diseases]]
*[[Myeloproliferative diseases]]
*[[Myeloproliferative neoplasm]]
*[[Natriuretic peptides|Natriuretic peptide receptor 3 (NPR3)]] [[mutation]]
*[[Neonatal hepatitis]]
*[[Neonatal hepatitis]]
*[[NISCH syndrome]]
*[[NISCH syndrome]]
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*[[Pancreatic cancer]]
*[[Pancreatic cancer]]
*Partial nodular transformation
*Partial nodular transformation
*[[Phospholipase A2|Phospholipase A2 (PL2G10)]] [[mutation]]
*Phosphomannose isomerase deficiency
*[[Polycystic kidney disease]]
*[[Polycystic kidney disease]]
*[[Portal hypertension]] due to intrahepatic block
*[[Portal hypertension]] due to intrahepatic block
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*[[Primary sclerosing cholangitis]]
*[[Primary sclerosing cholangitis]]
*[[Progressive familial intrahepatic cholestasis]]
*[[Progressive familial intrahepatic cholestasis]]
*[[Prostaglandin E2 receptor|Prostaglandin E receptor 2 (PTGER2)]] [[mutation]]
*[[Renal transplantation]]
*[[Sarcoidosis]]
*[[Sarcoidosis]]
*[[Schistosomiasis]]
*[[Schistosomiasis]]
*[[Sickle cell disease]]
*[[Arteriovenous fistula|Splanchnic arteriovenous fistula]]
*[[Arteriovenous fistula|Splanchnic arteriovenous fistula]]
*[[Splenic vein thrombosis]]
*[[Splenic vein thrombosis]]
*[[Thioguanine]]
*[[Thioguanine]]
*[[Transforming growth factor-β|Transforming growth factor (TGF)-β]] [[mutation]]
*[[Tricuspid insufficiency]]
*[[Tricuspid insufficiency]]
*[[Turner syndrome]]
*[[Umbilical]] [[sepsis]]
*[[Umbilical]] [[sepsis]]
*[[Viral hepatitis]]
*[[Viral hepatitis]]
*[[Vitamin A]]
*[[Wilson's Disease]]
*[[Wilson's Disease]]
{{EndMultiCol}}
}}


==References==
==References==
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Latest revision as of 22:28, 10 January 2020

Portal Hypertension Microchapters

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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]

Overview

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.

Causes

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

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

Intrahepatic

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

Posthepatic

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

References

  1. ↑ 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. ↑ 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.
  5. ↑ 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. ↑ 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. ↑ 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. ↑ 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. ↑ 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. ↑ 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.
  15. ↑ 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. ↑ 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. ↑ 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. ↑ 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. ↑ 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. ↑ 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.
  21. ↑ 21.0 21.1 Campia U, Cardillo C, Panza JA (2004). "Ethnic differences in the vasoconstrictor activity of endogenous endothelin-1 in hypertensive patients". Circulation. 109 (25): 3191–5. doi:10.1161/01.CIR.0000130590.24107.D3. PMID 15148269.
  22. ↑ Mandel H, Szargel R, Labay V, Elpeleg O, Saada A, Shalata A, Anbinder Y, Berkowitz D, Hartman C, Barak M, Eriksson S, Cohen N (2001). "The deoxyguanosine kinase gene is mutated in individuals with depleted hepatocerebral mitochondrial DNA". Nat. Genet. 29 (3): 337–41. doi:10.1038/ng746. PMID 11687800.
  23. ↑ Blackburn MR, Lee CG, Young HW, Zhu Z, Chunn JL, Kang MJ, Banerjee SK, Elias JA (2003). "Adenosine mediates IL-13-induced inflammation and remodeling in the lung and interacts in an IL-13-adenosine amplification pathway". J. Clin. Invest. 112 (3): 332–44. doi:10.1172/JCI16815. PMC 166289. PMID 12897202.
  24. ↑ Kotani, Kohei; Kawabe, Joji; Morikawa, Hiroyasu; Akahoshi, Tomohiko; Hashizume, Makoto; Shiomi, Susumu (2015). "Comprehensive Screening of Gene Function and Networks by DNA Microarray Analysis in Japanese Patients with Idiopathic Portal Hypertension". Mediators of Inflammation. 2015: 1–10. doi:10.1155/2015/349215. ISSN 0962-9351.
  25. ↑ Chu FF, Esworthy RS, Doroshow JH, Doan K, Liu XF (1992). "Expression of plasma glutathione peroxidase in human liver in addition to kidney, heart, lung, and breast in humans and rodents". Blood. 79 (12): 3233–8. PMID 1339300.
  26. ↑ Yokomizo T, Izumi T, Chang K, Takuwa Y, Shimizu T (1997). "A G-protein-coupled receptor for leukotriene B4 that mediates chemotaxis". Nature. 387 (6633): 620–4. doi:10.1038/42506. PMID 9177352.
  27. ↑ Lopez MJ, Wong SK, Kishimoto I, Dubois S, Mach V, Friesen J, Garbers DL, Beuve A (1995). "Salt-resistant hypertension in mice lacking the guanylyl cyclase-A receptor for atrial natriuretic peptide". Nature. 378 (6552): 65–8. doi:10.1038/378065a0. PMID 7477288.
  28. ↑ Aruffo A, Stamenkovic I, Melnick M, Underhill CB, Seed B (1990). "CD44 is the principal cell surface receptor for hyaluronate". Cell. 61 (7): 1303–13. PMID 1694723.
  29. ↑ Derynck R, Akhurst RJ, Balmain A (2001). "TGF-beta signaling in tumor suppression and cancer progression". Nat. Genet. 29 (2): 117–29. doi:10.1038/ng1001-117. PMID 11586292.
  30. ↑ Cole SP, Bhardwaj G, Gerlach JH, Mackie JE, Grant CE, Almquist KC, Stewart AJ, Kurz EU, Duncan AM, Deeley RG (1992). "Overexpression of a transporter gene in a multidrug-resistant human lung cancer cell line". Science. 258 (5088): 1650–4. PMID 1360704.

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