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, Monocytes, Bone marrow, Testes, Bone, Skin, Smooth muscle

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
Are the spider angiomas skin markers of hepatopulmonary syndrome? (24322187)
2013
2
Recurrence of hepatopulmonary syndrome post-orthotopic liver transplantation in a patient with noncirrhotic portal hypertension. (23908097)
2013
3
Parenchymal alterations in cirrhotic livers in patients with hepatopulmonary syndrome or portopulmonary hypertension. (23463612)
2013
4
Pentoxifylline for slow to resolve hepatopulmonary syndrome post liver transplantation: helpful or unnecessary? (23650789)
2013
5
TNF-I+ neutralization improves experimental hepatopulmonary syndrome in rats. (22672643)
2012
6
Hepatopulmonary syndrome: monitoring at your fingertip. (21448697)
2011
7
Hepatopulmonary syndrome: prevalence and predictors in Egyptian cirrhotic patients. (21922852)
2011
8
Serial pulse oximetry in hepatopulmonary syndrome. (21327708)
2011
9
Extracorporeal membrane oxygenation as a bridge to liver transplantation for acute respiratory distress syndrome-induced life-threatening hypoxaemia aggravated by hepatopulmonary syndrome. (21958549)
2011
10
Pulmonary artery embolotherapy in a patient with type I hepatopulmonary syndrome after liver transplantation. (20592935)
2010
11
Successful resolution of severe hepatopulmonary syndrome following liver transplantation. (19930139)
2010
12
Long-term outcome and management of hepatopulmonary syndrome in children. (19686444)
2010
13
High values of intrathoracic blood volume in hepatopulmonary syndrome. (19485877)
2009
14
Findings of hepatopulmonary syndrome on breath-hold perfusion SPECT-CT fusion images. (19396512)
2009
15
Hepatopulmonary syndrome in patients with Schistosoma mansoni periportal fibrosis. (19524079)
2009
16
Pulmonary angiogenesis in a rat model of hepatopulmonary syndrome. (19109954)
2009
17
Pilot study of pentoxifylline in hepatopulmonary syndrome. (18668653)
2008
18
Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation. (18280268)
2008
19
The hepatopulmonary syndrome. (18724401)
2008
20
Should we screen for hepatopulmonary syndrome in liver transplant candidates? (17256775)
2007
21
Left atrial volume: a novel predictor of hepatopulmonary syndrome. (17433020)
2007
22
Utility of pulse oximetry screening for hepatopulmonary syndrome. (17392034)
2007
23
Evaluation of intrapulmonary vascular dilatations with high-resolution computed thorax tomography in patients with hepatopulmonary syndrome. (16340638)
2006
24
Portopulmonary hypertension and hepatopulmonary syndrome, and liver transplantation. (16832207)
2006
25
Pregnancy exacerbating hepatopulmonary syndrome. (16449147)
2006
26
The importance of predicting the prognosis in patients with hepatopulmonary syndrome: a simple scoring system. (16647473)
2006
27
Natural history of hepatopulmonary syndrome: Impact of liver transplantation. (15828054)
2005
28
Congenital absence of portal vein presenting as hepatopulmonary syndrome. (15670231)
2005
29
Rapid recurrence of nonalcoholic fatty liver disease after transplantation in a child with hypopituitarism and hepatopulmonary syndrome. (15690545)
2005
30
The effects of serum estrogen levels on hypoxemia and blood nitric oxide levels in experimental hepatopulmonary syndrome. (16099204)
2005
31
Case 63: hepatopulmonary syndrome. (14519870)
2003
32
Hepatopulmonary syndrome: a good relationship gone bad. (14517808)
2003
33
Novel presentation and approach to management of hepatopulmonary syndrome with use of antimicrobial agents. (11317264)
2001
34
Hepatopulmonary syndrome associated with autoimmune liver cirrhosis. (11422898)
2001
35
Hepatopulmonary syndrome in noncirrhotic portal hypertension. (10836927)
2000
36
Hepatopulmonary syndrome: recent literature (1997 to 1999) and implications for liver transplantation. (10915189)
2000
37
Pulmonary hypertension following hepatopulmonary syndrome in a patient with cirrhosis. (10453952)
1999
38
Unexpected myocardial uptake on perfusion lung scan in the hepatopulmonary syndrome. (10439188)
1999
39
Reversal of hypoxemia by inhaled nitric oxide in children with severe hepatopulmonary syndrome, type 1, during and after liver transplantation. (9484767)
1998
40
Use of macroaggregated albumin lung perfusion scan to diagnose hepatopulmonary syndrome: a new approach. (9453490)
1998
41
Common bile duct ligation in the rat: a model of intrapulmonary vasodilatation and hepatopulmonary syndrome. (9142908)
1997
42
Formation of a national database on pulmonary hypertension and hepatopulmonary syndrome in chronic liver disease. (9286915)
1997
43
Hepatopulmonary syndrome]. (9549117)
1997
44
Hepatopulmonary syndrome associated with schistosomal liver disease. (9286482)
1997
45
The hepatopulmonary syndrome: radiologic findings in 10 patients. (8633451)
1996
46
Diagnostic utility of contrast echocardiography and lung perfusion scan in patients with hepatopulmonary syndrome. (7557096)
1995
47
Transjugular intrahepatic portosystemic shunt improves oxygenation in hepatopulmonary syndrome. (7657128)
1995
48
The hepatopulmonary syndrome masquerading as pulmonary Langerhans-cell histiocytosis. Hepatopulmonary Syndrome Study Group. (8198345)
1994
49
Hepatopulmonary syndrome: qualification of the defect. (1539585)
1992
50
Hepatopulmonary syndrome. (2225941)
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

Search GEO for disease gene expression data for Hepatopulmonary Syndrome.

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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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