MCID: HPT009
MIFTS: 49

Hepatopulmonary Syndrome malady

Liver diseases category

Summaries for Hepatopulmonary Syndrome

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63Wikipedia, 32MalaCards
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Wikipedia:63 In medicine, hepatopulmonary syndrome is a syndrome of shortness of breath and hypoxemia (low oxygen... more...

MalaCards: Hepatopulmonary Syndrome is related to hypertension and vascular disease. An important gene associated with Hepatopulmonary Syndrome is HMOX1 (heme oxygenase (decycling) 1), and among its related pathways is Bile secretion. The compounds oxygen and cholesterol have been mentioned in the context of this disorder. Affiliated tissues include liver, lung and endothelial, and related mouse phenotypes are homeostasis/metabolism and cardiovascular system.

Aliases & Classifications for Hepatopulmonary Syndrome

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8Disease Ontology, 10DISEASES, 44Novoseek, 60UMLS, 34MeSH, 56SNOMED-CT
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Liver diseases


Aliases & Descriptions:

hepatopulmonary syndrome 8 10 44 60


External Ids:

Disease Ontology8 DOID:900
SNOMED-CT56 371067004
MeSH34 D020065

Related Diseases for Hepatopulmonary Syndrome

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17GeneCards, 18GeneDecks
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Diseases related to Hepatopulmonary Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 52)
idRelated DiseaseScoreTop Affiliating Genes
1hypertension31.2KCNA5, NOX1, ALB
2vascular disease30.2ACVRL1, BHMT, NOX1, HMOX1
3hepatitis b30.2ALB
4biliary atresia30.2SLC10A2, SLC10A1
5arteriovenous malformation30.0ACVRL1
6cholestasis30.0ALB, SLC10A2, NR0B2, SLC10A1
7liver disease10.6
8liver cirrhosis10.6
9portal hypertension10.6
10hepatitis10.5
11hepatitis a10.3
12sarcoidosis10.3
13image syndrome10.3
14n syndrome10.2
15hepatitis c10.1
16adult syndrome10.1
17histiocytosis10.1
18hypopituitarism10.1
19langerhans-cell histiocytosis10.1
20cardiac tamponade10.0
21cystic fibrosis10.0
22dyskeratosis congenita10.0
23primary biliary cirrhosis10.0
24good syndrome10.0
25granulomatous hepatitis10.0
26fatty liver disease10.0
27hepatorenal syndrome10.0
28brain disease10.0
29ischemia10.0
30polycythemia10.0
31pulmonary embolism10.0
32respiratory failure10.0
33gaucher disease type 110.0
34congenital hepatic fibrosis10.0
35galactosemia10.0ALB
36pancreatitis10.0ALB, CELA1
37atherosclerosis10.0NOX1, ACVRL1, SLC10A2, HMOX1, PI3, ALB
38hepatocellular carcinoma10.0BHMT, ALB
39adenoma10.0NOX1, DUOX2, CAV2, DUOX1
40congenital hypothyroidism10.0DUOX2, DUOX1
41adenocarcinoma10.0RARG, CAV2, HMOX1, NOX1
42chronic granulomatous disease10.0DUOX1, DUOX2
43obesity10.0HMOX1, NR0B2
44sepsis10.0PI3, ALB, HMOX1, C4A
45hypercholesterolemia10.0SLC10A2
46diarrhea10.0ALB
47ulcerative colitis10.0ALB
48primary hyperoxaluria10.0NOX1, C4A, HMOX1, ALB
49uremia10.0ALB
50crohn's disease10.0ALB

Graphical network of the top 20 diseases related to Hepatopulmonary Syndrome:



Diseases related to hepatopulmonary syndrome

Clinical Features for Hepatopulmonary Syndrome

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Drugs & Therapeutics for Hepatopulmonary Syndrome

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Hepatopulmonary Syndrome

Drug clinical trials:

Search ClinicalTrials for Hepatopulmonary Syndrome

Search NIH Clinical Center for Hepatopulmonary Syndrome

Search CenterWatch for Hepatopulmonary Syndrome

Genetic Tests for Hepatopulmonary Syndrome

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Anatomical Context for Hepatopulmonary Syndrome

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32MalaCards
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MalaCards organs/tissues related to Hepatopulmonary Syndrome:

32
Liver, Lung, Endothelial, Brain, Smooth muscle, Bone, Skin, Testes, Monocytes, Bone marrow

Animal Models for Hepatopulmonary Syndrome or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Hepatopulmonary Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537610.5ALB, KCNA5, CAV2, CAV3, NR0B2, BHMT
2MP:000538510.5KCNA5, CAV2, CAV3, HTR2B, GDF2, RARG
3MP:000536910.5KCNA5, CAV2, CAV3, HTR2B, HMOX1, NOX1
4MP:000537810.4HTR2B, NR0B2, BHMT, DUOX2, RARG, HMOX1
5MP:000537010.4NR0B2, BHMT, HMOX1, SLC10A2, ACVRL1, ALB
6MP:000538110.2NR0B2, RARG, SLC10A2, NOX1, ACVRL1, ALB

Publications for Hepatopulmonary Syndrome

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50PubMed
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Articles related to Hepatopulmonary Syndrome:

(show top 50)    (show all 390)
idTitleAuthorsYear
1
Pulse Oximetry Is Insufficient for Timely Diagnosis of Hepatopulmonary Syndrome in Children with Liver Cirrhosis. (24321540)
2013
2
The use of transtracheal oxygen therapy in the management of severe hepatopulmonary syndrome after liver transplantation. (24182809)
2013
3
Hepatopulmonary syndrome in children with cirrhotic and non-cirrhotic portal hypertension: a single-center experience. (21792618)
2012
4
Hepatopulmonary syndrome associated with iron overload in sickle cell anaemia. (22753840)
2012
5
Cyanosis revealing hepatopulmonary syndrome in a child with dyskeratosis congenita. (19953657)
2010
6
(99m)Technetium-macroaggregated albumin perfusion lung scan versus contrast enhanced echocardiography in the diagnosis of the hepatopulmonary syndrome in children with chronic liver disease. (20101183)
2010
7
Improved survival after liver transplantation in patients with hepatopulmonary syndrome. (19775311)
2010
8
Attenuation of experimental hepatopulmonary syndrome in endothelin B receptor-deficient rats. (19196949)
2009
9
Prospective evaluation of postoperative outcome after liver transplantation in hepatopulmonary syndrome patients. (19951576)
2009
10
Hepatopulmonary syndrome: an update. (20011928)
2009
11
Successful treatment of severe hepatopulmonary syndrome with a sequential use of TIPS placement and liver transplantation. (19221539)
2009
12
CT findings in unilateral hepatopulmonary syndrome after the Fontan operation. (19189098)
2009
13
Prevalence of hepatopulmonary syndrome in children. (18310172)
2008
14
Mcp-1, eNOS, tPA and PAI-1 gene polymorphism and correlation of genotypes and phenotypes in hepatopulmonary syndrome. (17934860)
2008
15
The hepatopulmonary syndrome. (18724400)
2008
16
Value of contrast echocardiography for the diagnosis of hepatopulmonary syndrome. (17011830)
2007
17
Clinical controversies surrounding the diagnosis and treatment of hepatopulmonary syndrome. (17464272)
2007
18
Pediatric hepatopulmonary syndrome is seen with polysplenia/interrupted inferior vena cava and without cirrhosis. (17457897)
2007
19
Modulation of pulmonary endothelial endothelin B receptor expression and signaling: implications for experimental hepatopulmonary syndrome. (17337507)
2007
20
Hepatopulmonary syndrome can show spontaneous resolution: possible mechanism of portopulmonary hypertension overlap? (16423214)
2006
21
Echocardiographic detection of intrapulmonary shunting in a patient with hepatopulmonary syndrome: case report and review of the literature. (16412185)
2006
22
The pulmonary involvement in portal hypertension: portopulmonary hypertension and hepatopulmonary syndrome. (16393630)
2006
23
Hepatopulmonary syndrome increases the postoperative mortality rate following liver transplantation: a prospective study in 90 patients. (16686767)
2006
24
Is there any medical therapeutic option in hepatopulmonary syndrome? A case report. (15833681)
2005
25
Development of hepatopulmonary syndrome and portopulmonary hypertension in a paediatric liver transplant patient. (15667626)
2005
26
Liver transplantation in high-risk patients: hepatopulmonary syndrome and portopulmonary hypertension. (16386564)
2005
27
Arteriovenous malformations and hepatopulmonary syndrome. (15234848)
2004
28
Hepatopulmonary syndrome: role of nitric oxide and clinical aspects. (15191196)
2004
29
Hepatopulmonary syndrome in HIV-hepatitis C virus coinfection: a case report and review of the literature. (15307020)
2004
30
Effect on dyspnoea and hypoxaemia of inhaled N(G)-nitro-L-arginine methyl ester in hepatopulmonary syndrome. (12853200)
2003
31
Improvement of hepatopulmonary syndrome after transjugular intrahepatic portasystemic shunting: case report and review of literature. (12654059)
2003
32
Hepatopulmonary syndrome. (12421031)
2002
33
Regulation of heme oxygenase-1 by nitric oxide during hepatopulmonary syndrome. (12114196)
2002
34
Methylene blue improves the hepatopulmonary syndrome. (11529704)
2001
35
Cardiopulmonary effects of nitric oxide inhalation and methylene blue injection in hepatopulmonary syndrome. (11497151)
2001
36
Successful liver transplantation in a child with biliary atresia and hepatopulmonary syndrome. (11480250)
2001
37
The hepatopulmonary syndrome: a case report and review of the literature. (11061024)
2000
38
Rehabilitating patients with hepatopulmonary syndrome using living-related orthotopic liver transplant: a case report. (11083360)
2000
39
Pulmonary infarction after liver transplant and previous hepatopulmonary syndrome. (10963521)
2000
40
Late recurrence and rapid evolution of severe hepatopulmonary syndrome after liver transplantation. (10477847)
1999
41
Hepatopulmonary syndrome associated with cardiorespiratory disease. (10365816)
1999
42
Hepatopulmonary syndrome. (10407994)
1999
43
Hypoxemia and liver cirrhosis (hepatopulmonary syndrome) in eight patients: comparison of the central and peripheral pulmonary vasculature. (10228541)
1999
44
Endothelin-1 stimulation of endothelial nitric oxide synthase in the pathogenesis of hepatopulmonary syndrome. (10564099)
1999
45
Hepatopulmonary syndrome]. (10544613)
1999
46
Hepatopulmonary syndrome and liver transplantation: a review of the peroperative management of seven paediatric cases. (9483600)
1998
47
The hepatopulmonary syndrome. (15562676)
1997
48
Elevated exhaled nitric oxide in patients with hepatopulmonary syndrome. (8620957)
1995
49
Reversibility of hepatopulmonary syndrome evidenced by serial pulmonary perfusion scan. (8440418)
1993
50
Hepatopulmonary syndrome: an evolving perspective in the era of liver transplantation. (2403962)
1990

Genetic Variations for Hepatopulmonary Syndrome

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Expression for genes affiliated with Hepatopulmonary Syndrome

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Hepatopulmonary Syndrome

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Pathways for genes affiliated with Hepatopulmonary Syndrome

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29KEGG
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Pathways related to Hepatopulmonary Syndrome according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.5SLC10A1, SLC10A2, NR0B2

Compounds for genes affiliated with Hepatopulmonary Syndrome

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Sources:
44Novoseek, 24HMDB, 28IUPHAR, 11DrugBank, 49PharmGKB, 59Tocris Bioscience
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Compounds related to Hepatopulmonary Syndrome according to GeneCards/GeneDecks:

(show all 20)
idCompoundScoreTop Affiliating Genes
1oxygen44 2411.8NOX1, KCNA5, HTR2B, C4A, PI3, DUOX1
2cholesterol44 28 11 2413.8CAV2, CAV3, NR0B2, SLC10A2, SLC10A1, ALB
3glycochenodeoxycholic acid44 1111.8SLC10A2, SLC10A1
4nadph44 2411.8NOX1, HMOX1, DUOX2, DUOX1, HTR2B
5chenodeoxycholic acid44 28 11 2413.8SLC10A1, SLC10A2, NR0B2
6calcium44 49 11 2413.8KCNA5, CAV3, HTR2B, PI3, NR0B2, DUOX1
7superoxide44 2411.7PI3, DUOX1, DUOX2, HMOX1, NOX1
8nitric oxide44 11 2412.7CAV2, CAV3, HTR2B, PI3, HMOX1, NOX1
9sodium44 2411.7CAV3, DUOX2, SLC10A2, SLC10A1, ALB
101,3,7,12-Tetrahydroxycholan-24-oic acid2410.7SLC10A2, SLC10A1
111,3,12-Trihydroxycholan-24-oic acid2410.7SLC10A2, SLC10A1
12(3a,5b,7a)-23-Carboxy-7-hydroxy-24-norcholan-3-yl-b-D-Glucopyranosiduronic acid2410.7SLC10A2, SLC10A1
13h2o24410.6NOX1, HMOX1, DUOX2, DUOX1, KCNA5
14Pitavastatin1110.6SLC10A1, ALB
15serine4410.6ACVRL1, SLC10A2, RARG, BHMT, PI3, C4A
16Liotrix1110.5SLC10A1, ALB
17auranofin44 59 28 1113.5ALB, HMOX1
18cyclosporine49 1111.5SLC10A1, SLC10A2
19Liothyronine11 2411.4ALB, SLC10A1
20acitretin44 1111.2ALB, RARG

GO Terms for genes affiliated with Hepatopulmonary Syndrome

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16Gene Ontology
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Cellular components related to Hepatopulmonary Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1membrane raftGO:04512110.5CAV3, CAV2, KCNA5
2caveolaGO:00590110.4HMOX1, CAV3, CAV2, KCNA5
3protein complexGO:04323410.2CAV2, NR0B2, BHMT, ALB

Biological processes related to Hepatopulmonary Syndrome according to GeneCards/GeneDecks:

(show all 16)
idNameGO IDScoreTop Affiliating Genes
1bile acid and bile salt transportGO:01572110.8SLC10A2, SLC10A1, ALB
2positive regulation of endothelial cell proliferationGO:00193810.8ACVRL1, GDF2, HTR2B, CAV2
3negative regulation of endothelial cell proliferationGO:00193710.7ACVRL1, GDF2, CAV2
4bile acid metabolic processGO:00820610.7ALB, SLC10A1, SLC10A2
5cuticle developmentGO:04233510.7DUOX1, DUOX2
6caveola assemblyGO:07083610.7CAV3, CAV2
7negative regulation of DNA biosynthetic processGO:200027910.7ACVRL1, GDF2
8regulation of blood pressureGO:00821710.7HMOX1, NOX1, ACVRL1
9positive regulation of endothelial cell differentiationGO:04560310.6ACVRL1, GDF2
10hormone biosynthetic processGO:04244610.6DUOX2, DUOX1
11negative regulation of blood vessel endothelial cell migrationGO:04353710.6GDF2, ACVRL1
12superoxide anion generationGO:04255410.5DUOX1, NOX1
13negative regulation of endothelial cell migrationGO:01059610.5GDF2, ACVRL1
14hydrogen peroxide catabolic processGO:04274410.5DUOX2, DUOX1
15thyroid hormone generationGO:00659010.4DUOX1, DUOX2
16oxidation-reduction processGO:05511410.1NOX1, DUOX2, DUOX1

Molecular functions related to Hepatopulmonary Syndrome according to GeneCards/GeneDecks:

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1potassium channel inhibitor activityGO:01987010.6CAV3, KCNA5
2bile acid:sodium symporter activityGO:00850810.6SLC10A1, SLC10A2
3NAD(P)H oxidase activityGO:01617410.5DUOX2, DUOX1
4retinoid X receptor bindingGO:04696510.5RARG, NR0B2
5peroxidase activityGO:00460110.5DUOX2, DUOX1
6protein complex bindingGO:03240310.4CAV3, NR0B2, BHMT
7heme bindingGO:02003710.2HMOX1, DUOX2, DUOX1

Products for genes affiliated with Hepatopulmonary Syndrome

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  • Antibodies
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Sources for Hepatopulmonary Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet