MCID: HPT009
MIFTS: 38

Hepatopulmonary Syndrome malady

Liver diseases category

Aliases & Classifications for Hepatopulmonary Syndrome

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Hepatopulmonary Syndrome, Aliases & Descriptions:

Name: Hepatopulmonary Syndrome 9 11 43 60


Classifications:

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


External Ids:

Disease Ontology9 DOID:900
SNOMED-CT55 371067004
MeSH33 D020065

Summaries for Hepatopulmonary Syndrome

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

MalaCards based summary: Hepatopulmonary Syndrome is related to cholestasis and cystic fibrosis. An important gene associated with Hepatopulmonary Syndrome is HMOX1 (heme oxygenase (decycling) 1). The compounds auranofin and Bilirubin have been mentioned in the context of this disorder. Affiliated tissues include liver, lung and endothelial, and related mouse phenotypes are muscle and cardiovascular system.

Related Diseases for Hepatopulmonary Syndrome

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Diseases related to Hepatopulmonary Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 48)
idRelated DiseaseScoreTop Affiliating Genes
1cholestasis30.3ALB, NR0B2
2cystic fibrosis30.0PI3, CELA1, ALB, HMOX1
3liver disease10.7
4portal hypertension10.6
5hepatitis10.6
6liver cirrhosis10.5
7pulmonary hypertension10.4
8endotheliitis10.4
9budd-chiari syndrome10.4
10bilirubin metabolic disorder10.3HMOX1, ALB
11congenital hepatic fibrosis10.3
12cork-handlers' disease10.3ALB, HMOX1
13diabetes mellitus, insulin-dependent10.3HMOX1, ALB
14hepatitis c virus10.2
15hepatitis b10.2
16hepatitis c10.2
17langerhans-cell histiocytosis10.2
18biliary atresia10.2
19histiocytosis10.2
20hypopituitarism10.2
21hypoxia10.2
22long qt syndrome10.2CAV3, KCNA5
23hyperhomocysteinemia10.1BHMT, ALB
24pancreatitis10.1ALB, CELA1
25pulmonary function10.1
26common variable immunodeficiency10.1
27primary biliary cirrhosis10.1
28dyskeratosis congenita10.1
29fatty liver disease10.1
30cardiac tamponade10.1
31hepatorenal syndrome10.1
32hepatitis a10.1
33arteriovenous malformation10.1
34visceral leishmaniasis10.1
35ischemia10.1
36leishmaniasis10.1
37polycythemia10.1
38respiratory failure10.1
39acute respiratory distress syndrome10.1
40congenital extrahepatic portosystemic shunt10.1
41nodular regenerative hyperplasia10.1
42cirrhotic cardiomyopathy10.1
43chronic kidney failure10.0ALB, BHMT, HMOX1
44iga glomerulonephritis10.0C4A, ALB
45atherosclerosis10.0HMOX1, ALB, PI3
46sudden infant death syndrome10.0C4A, CAV3
47nephritis9.9C4A, ALB
48primary hyperoxaluria9.9C4A, HMOX1, ALB

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



Diseases related to hepatopulmonary syndrome

Symptoms for Hepatopulmonary Syndrome

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Drugs & Therapeutics for Hepatopulmonary Syndrome

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Drug clinical trials:

Search ClinicalTrials for Hepatopulmonary Syndrome

Search NIH Clinical Center for Hepatopulmonary Syndrome

Genetic Tests for Hepatopulmonary Syndrome

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Anatomical Context for Hepatopulmonary Syndrome

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MalaCards organs/tissues related to Hepatopulmonary Syndrome:

31
Liver, Lung, Endothelial, Brain, Smooth muscle, Bone marrow, Bone, Skin, Testes, Monocytes

Animal Models for Hepatopulmonary Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Hepatopulmonary Syndrome:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053698.6HMOX1, ALB, CAV3, HTR2B, KCNA5
2MP:00053858.5HMOX1, CAV3, HTR2B, KCNA5, GDF2
3MP:00053708.5ALB, NR0B2, BHMT, HMOX1
4MP:00053767.8KCNA5, CAV3, ALB, NR0B2, BHMT, HMOX1

Publications for Hepatopulmonary Syndrome

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Articles related to Hepatopulmonary Syndrome:

(show top 50)    (show all 422)
idTitleAuthorsYear
1
Hepatopulmonary syndrome and portopulmonary hypertension: recent knowledge in pathogenesis and overview of clinical assessment. (25627425)
2015
2
Variability of Oxygenation in Possible Hepatopulmonary Syndrome: Effects of Requiring Two Abnormal Arterial Blood Gas Results for Diagnosis. (25586084)
2015
3
Aggravation of hepatopulmonary syndrome after sildenafil treatment in a patient with coexisting portopulmonary hypertension. (25653708)
2015
4
MiR-206 controls the phenotypic modulation of pulmonary arterial smooth muscle cells induced by serum from rats with hepatopulmonary syndrome by regulating the target gene, annexin A2. (25427750)
2014
5
Successful resolution of very severe hepatopulmonary syndrome following adult-to-adult living donor liver transplantation: Report of two cases. (25355534)
2014
6
Report of seven children with hepatopulmonary syndrome. (25599789)
2014
7
Are the spider angiomas skin markers of hepatopulmonary syndrome? (24322187)
2013
8
Pharmacological treatment for hepatopulmonary syndrome. (24102057)
2013
9
Detection of hepatopulmonary syndrome in patients with liver cirrhosis using 3D contrast echocardiography. (22560819)
2012
10
Effects of caffeic acid phenethyl ester (CAPE) on hepatopulmonary syndrome. (21046212)
2011
11
Physiopathological, clinical and therapeutic aspects of hepatopulmonary syndrome. (20589345)
2010
12
Sarcoidosis complicated by cirrhosis and hepatopulmonary syndrome. (18437252)
2008
13
Current concepts in the management of hepatopulmonary syndrome. (19183751)
2008
14
Pentoxifylline therapy for hepatopulmonary syndrome: a pilot study. (18779471)
2008
15
Value of contrast echocardiography for the diagnosis of hepatopulmonary syndrome. (17011830)
2007
16
Successful liver retransplantation for recurrent hepatopulmonary syndrome. (17976130)
2007
17
Hepatopulmonary syndrome. (17722431)
2007
18
Clinical controversies surrounding the diagnosis and treatment of hepatopulmonary syndrome. (17464272)
2007
19
Congenital portocaval fistula associated with hepatopulmonary syndrome: ligation vs liver transplantation. (16481229)
2006
20
Liver transplantation of a child with child a cirrhosis and severe hepatopulmonary syndrome. (16797324)
2006
21
Effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome. (16688838)
2006
22
Hepatopulmonary syndrome can show spontaneous resolution: possible mechanism of portopulmonary hypertension overlap? (16423214)
2006
23
Natural history of hepatopulmonary syndrome: Impact of liver transplantation. (15828054)
2005
24
Estrogen promotes intrapulmonary vasodilatation in hepatopulmonary syndrome. (16024287)
2005
25
Congenital absence of portal vein presenting as hepatopulmonary syndrome. (15670231)
2005
26
The hepatopulmonary syndrome: NO way out? (15176675)
2004
27
Case report of unchanged tacrolimus clearance in a hypoxemic pediatric liver transplant recipient with hepatopulmonary syndrome. (15502939)
2004
28
Severe polycythemia as the first clinical presentation of hepatopulmonary syndrome. (12811222)
2003
29
Liver transplantation for hepatopulmonary syndrome: a ten-year experience in Paris, France. (12792501)
2003
30
Hepatopulmonary syndrome in noncirrhotic portal hypertensive patients. (12757170)
2003
31
Case 63: hepatopulmonary syndrome. (14519870)
2003
32
Hepatopulmonary syndrome. (12421031)
2002
33
Biliary cyst fluid from common bile duct-ligated rats stimulates endothelial nitric oxide synthase in pulmonary artery endothelial cells: a potential role in hepatopulmonary syndrome. (11230754)
2001
34
Methylene blue improves the hepatopulmonary syndrome. (11529704)
2001
35
Prevalence of hepatopulmonary syndrome in cirrhosis and extrahepatic portal venous obstruction. (11774955)
2001
36
Hepatopulmonary Syndrome and Portopulmonary Hypertension. (11696280)
2001
37
Hepatopulmonary syndrome in noncirrhotic portal hypertension. (10836927)
2000
38
Hepatopulmonary Syndrome and Liver Transplantation. (10648589)
2000
39
Portal hypertension and hepatopulmonary syndrome in a middle-aged man with hepatitis B infection. (11076405)
2000
40
Hepatopulmonary syndrome: a prospective study of relationships between severity of liver disease, PaO(2) response to 100% oxygen, and brain uptake after (99m)Tc MAA lung scanning. (10988181)
2000
41
Hepatopulmonary syndrome in Gaucher disease with right-to-left shunt: evaluation and measurement using Tc-99m MAA. (10069725)
1999
42
Late recurrence and rapid evolution of severe hepatopulmonary syndrome after liver transplantation. (10477847)
1999
43
Localizing intrapulmonary shunt in hepatopulmonary syndrome by transesophageal echocardiography. (10506859)
1999
44
Pulmonary hypertension following hepatopulmonary syndrome in a patient with cirrhosis. (10453952)
1999
45
Hepatopulmonary syndrome associated with sarcoidosis of the liver: case report. (9879271)
1998
46
Severe pulmonary hypertension and amelioration of hepatopulmonary syndrome after liver transplantation. (9516572)
1998
47
Diagnosis of hepatopulmonary syndrome with contrast transthoracic echocardiography and histological confirmation. (9766826)
1998
48
Hepatopulmonary syndrome: the paradigm of liver-induced hypoxaemia. (9395754)
1997
49
Elevated exhaled nitric oxide in patients with hepatopulmonary syndrome. (8620957)
1995
50
The hepatopulmonary syndrome masquerading as pulmonary Langerhans-cell histiocytosis. Hepatopulmonary Syndrome Study Group. (8198345)
1994

Variations for Hepatopulmonary Syndrome

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Expression for genes affiliated with Hepatopulmonary Syndrome

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Search GEO for disease gene expression data for Hepatopulmonary Syndrome.

Pathways for genes affiliated with Hepatopulmonary Syndrome

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Compounds for genes affiliated with Hepatopulmonary Syndrome

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Compounds related to Hepatopulmonary Syndrome according to GeneCards Suite gene sharing:

(show all 19)
idCompoundScoreTop Affiliating Genes
1auranofin28 43 59 1213.2HMOX1, ALB
2Bilirubin2410.2ALB, HMOX1
3isoflurane49 43 1212.2HMOX1, ALB
4propofol43 49 1212.1HMOX1, ALB
5clomipramine43 49 1212.1HTR2B, ALB
6fluoxetine43 49 28 1213.0ALB, HTR2B
7polyethylene glycol439.9HMOX1, ALB
8olanzapine43 49 28 24 1213.9HTR2B, ALB
9vitamin b6439.8BHMT, ALB
10salicylic acid43 2810.8HMOX1, ALB
11vitamin b12439.7BHMT, ALB
12histamine43 28 2411.6PI3, ALB, HMOX1
13fatty acid439.1ALB, CAV3, PI3
14nitric oxide43 24 1211.0PI3, HTR2B, CAV3, HMOX1
15glucose439.0GDF2, CAV3, ALB, NR0B2
16cysteine439.0HMOX1, BHMT, ALB, PI3
17oxygen43 249.4PI3, KCNA5, HTR2B, HMOX1, C4A
18lipid438.4PI3, CAV3, ALB, NR0B2
19calcium43 49 24 1210.9NR0B2, ALB, CAV3, HTR2B, CELA1, KCNA5

GO Terms for genes affiliated with Hepatopulmonary Syndrome

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Cellular components related to Hepatopulmonary Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1Z discGO:00300189.5CAV3, KCNA5
2caveolaGO:00059019.2HMOX1, CAV3, KCNA5
3protein complexGO:00432348.8BHMT, NR0B2, ALB

Biological processes related to Hepatopulmonary Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of angiogenesisGO:00457669.8HMOX1, GDF2
2negative regulation of sequence-specific DNA binding transcription factor activityGO:00434339.8HMOX1, NR0B2
3cellular iron ion homeostasisGO:00068799.7GDF2, HMOX1
4regulation of membrane potentialGO:00423919.5KCNA5, CAV3
5positive regulation of endothelial cell proliferationGO:00019389.5HTR2B, GDF2

Molecular functions related to Hepatopulmonary Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1drug bindingGO:00081449.8ALB, HTR2B
2potassium channel inhibitor activityGO:00198709.5CAV3, KCNA5
3endopeptidase inhibitor activityGO:00048668.8C4A, PI3
4protein complex bindingGO:00324038.8BHMT, NR0B2, CAV3

Products for genes affiliated with Hepatopulmonary Syndrome

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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
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet