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MCID: HPT009
MIFTS: 48

Hepatopulmonary Syndrome

Categories: Gastrointestinal diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Hepatopulmonary Syndrome

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MalaCards integrated aliases for Hepatopulmonary Syndrome:

Name: Hepatopulmonary Syndrome 12 20 54 44 15 17 70

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Gastrointestinal diseases
See all MalaCards categories (disease lists)


External Ids:

Disease Ontology 12 DOID:900
ICD9CM 34 573.5
MeSH 44 D020065
SNOMED-CT 67 371067004
ICD10 32 K76.81
UMLS 70 C0600452
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Summaries for Hepatopulmonary Syndrome

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GARD : 20 Hepatopulmonary syndrome (HPS) is a lung disease characterized by widening of arteries and veins (dilatation) in the lungs in people who have chronic liver disease. Because of the dilated vases, the workload of the heart increases and the blood pumped to the body does not have enough oxygen, leading to a decreased level of oxygen in the blood (hypoxemia). The normal diameter of the lung vessels ranges between 8 and 15 ?m whereas when in HPS rises to between 15 and 500 ?m. While many people with HPS don't have any obvious problems, the main reported symptom is shortness of breath (dyspnea) that is worse when standing or sitting up, and is relieved when lying down (platypnea). Symptoms related to chronic liver disease (generally cirrhosis ) include small red spots on the skin ( spider angiomas ) and abnormal vascular dilatations. Some other symptoms that have been described are infections in the brain (brain abscesses), brain bleeding and an increased number of red blood cells in the blood (polycythemia). There is currently no effective medication for HPS. Oxygen therapy may improve the breathing in some cases. Liver transplant is an efficient treatment which improves the symptoms, even in severe cases.

MalaCards based summary : Hepatopulmonary Syndrome is related to hepatic encephalopathy and varicose veins, and has symptoms including hemoptysis An important gene associated with Hepatopulmonary Syndrome is ALB (Albumin), and among its related pathways/superpathways are ERK Signaling and PEDF Induced Signaling. The drugs Norfloxacin and Letrozole have been mentioned in the context of this disorder. Affiliated tissues include liver, brain and lung, and related phenotypes are cardiovascular system and homeostasis/metabolism

Wikipedia : 73 In medicine, hepatopulmonary syndrome is a syndrome of shortness of breath and hypoxemia (low oxygen... more...

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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 top 50) (show all 410)
# Related Disease Score Top Affiliating Genes
1 hepatic encephalopathy 30.6 TNF HMOX1 ALB
2 varicose veins 30.5 VWF VEGFA TNF
3 schistosomiasis 30.3 TNF CCL2 ALB
4 non-alcoholic steatohepatitis 30.3 TNF CCL2 ALB
5 interstitial lung disease 30.2 TNF HMOX1 EDN1 CCL2 ALB
6 respiratory failure 30.2 VEGFA TNF EDN1 CCL2 ALB
7 liver cirrhosis 30.1 VEGFA TNF EDNRA EDN1 ALB AKT1
8 granulomatous hepatitis 30.1 TNF ALB
9 pneumonia 30.0 TNF HMOX1 CCL2 ALB
10 obstructive jaundice 30.0 TNF EDN1 ALB
11 thrombotic microangiopathy 30.0 VWF VEGFA
12 lung disease 30.0 VEGFA TNF HMOX1 EDN1 CCL2 ALB
13 juvenile rheumatoid arthritis 30.0 TNF CCL2 ALB
14 pulmonary hypertension, primary, 1 29.9 ENG BMPR2 ACVRL1
15 patent foramen ovale 29.9 VWF EDNRA EDN1 ALB
16 idiopathic interstitial pneumonia 29.8 VEGFA EDN1 CCL2
17 bilirubin metabolic disorder 29.8 VEGFA TNF HMOX1 ALB
18 visceral leishmaniasis 29.8 TNF NOS2 ALB
19 portal hypertension 29.7 VWF VEGFA TNF NOS3 EDNRB EDNRA
20 pulmonary edema 29.7 TNF NOS3 NOS2 HMOX1 EDN1
21 pulmonary arteriovenous malformation 29.5 ENG BMPR2 ACVRL1
22 end stage renal disease 29.5 VEGFA TNF NOS3 CCL2 ALB
23 arteriovenous malformation 29.3 VEGFA TNF ENG EDN1 BMPR2 ACVRL1
24 atrial heart septal defect 29.3 EDN1 BMPR2 ALB
25 pulmonary fibrosis, idiopathic 29.3 VEGFA TNF EDN1 CCL2 ALB AKT1
26 sleep apnea 29.3 VEGFA TNF NOS3 EDN1 CCL2 ALB
27 telangiectasis 29.2 GDF2 ENG BMPR2 ACVRL1 VEGFA
28 hemangioma 28.9 VWF VEGFA ENG AKT1
29 hereditary hemorrhagic telangiectasia 28.5 VWF VEGFA NOS3 GDF2 ENG BMPR2
30 body mass index quantitative trait locus 11 28.4 VEGFA TNF NOS3 HMOX1 EDNRA CCL2
31 stroke, ischemic 28.0 VWF TNF NOS3 ENG EDNRB EDNRA
32 vascular disease 27.5 VWF VEGFA TNF NOS3 NOS2 HMOX1
33 pulmonary hypertension 27.1 VWF VEGFA TNF NOS3 NOS2 HMOX1
34 heart disease 26.2 VWF VEGFA TNF NOS3 NOS2 HMOX1
35 pulmonary arterial hypertension associated with portal hypertension 10.7
36 cyanosis, transient neonatal 10.7
37 liver disease 10.6
38 biliary atresia 10.5
39 platelet membrane fluidity 10.4 NOS3 NOS2
40 immuno-osseous dysplasia 10.4 NOS2 ALB
41 near-fatal asthma 10.3 TNF CCL2
42 spotted fever rickettsiosis 10.3 TNF NOS2
43 meningovascular neurosyphilis 10.3 TNF ALB
44 palladium allergic contact dermatitis 10.3 TNF CCL2
45 critical illness polyneuropathy 10.3 TNF ALB
46 jejunoileitis 10.3 TNF ALB
47 renal hypertension 10.3 NOS3 EDN1 ALB
48 gastrointestinal tuberculosis 10.3 TNF ALB
49 aspiration pneumonitis 10.3 TNF ALB
50 chronic thromboembolic pulmonary hypertension 10.3 EDN1 BMPR2

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



Diseases related to Hepatopulmonary Syndrome
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Symptoms & Phenotypes for Hepatopulmonary Syndrome

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UMLS symptoms related to Hepatopulmonary Syndrome:


hemoptysis

MGI Mouse Phenotypes related to Hepatopulmonary Syndrome:

46 (show all 20)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.47 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
2 homeostasis/metabolism MP:0005376 10.39 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
3 growth/size/body region MP:0005378 10.35 ACVRL1 AKT1 BMPR2 EDN1 EDNRA EDNRB
4 hematopoietic system MP:0005397 10.34 ACVRL1 AKT1 BMPR2 EDNRA EDNRB ENG
5 cellular MP:0005384 10.32 AKT1 ALB BMPR2 EDNRA EDNRB HMOX1
6 digestive/alimentary MP:0005381 10.31 ACVRL1 ALB EDN1 EDNRA EDNRB ENG
7 immune system MP:0005387 10.31 AKT1 ALB BMPR2 CCL2 EDNRA EDNRB
8 mortality/aging MP:0010768 10.28 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
9 embryo MP:0005380 10.26 ACVRL1 AKT1 BMPR2 EDN1 EDNRA EDNRB
10 endocrine/exocrine gland MP:0005379 10.25 AKT1 ALB EDN1 EDNRA EDNRB HMOX1
11 craniofacial MP:0005382 10.23 ACVRL1 EDN1 EDNRA EDNRB ENG HMOX1
12 muscle MP:0005369 10.22 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
13 nervous system MP:0003631 10.1 ACVRL1 AKT1 BMPR2 EDN1 EDNRA EDNRB
14 integument MP:0010771 10.08 AKT1 BMPR2 EDNRA EDNRB ENG NOS2
15 liver/biliary system MP:0005370 10.06 ACVRL1 AKT1 ALB HMOX1 NOS2 NOS3
16 normal MP:0002873 9.91 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
17 neoplasm MP:0002006 9.85 AKT1 ALB EDNRB NOS2 TNF VEGFA
18 renal/urinary system MP:0005367 9.85 ACVRL1 ALB BMPR2 EDN1 EDNRA EDNRB
19 respiratory system MP:0005388 9.7 ACVRL1 AKT1 BMPR2 EDNRA EDNRB ENG
20 skeleton MP:0005390 9.32 AKT1 BMPR2 EDN1 EDNRA EDNRB GDF2
Sources

Drugs & Therapeutics for Hepatopulmonary Syndrome

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Drugs for Hepatopulmonary Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 36)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Norfloxacin Approved Phase 2 70458-96-7 4539
2
Letrozole Approved, Investigational Phase 2 112809-51-5 3902
3
Bosentan Approved, Investigational Phase 2 147536-97-8 104865
4
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
5
Iodine Approved, Investigational Phase 2 7553-56-2 807
6
Cadexomer iodine Experimental Phase 2 94820-09-4
7 Cytochrome P-450 Enzyme Inhibitors Phase 2
8 Anti-Bacterial Agents Phase 2
9 Anti-Infective Agents Phase 2
10 Hormone Antagonists Phase 2
11 Hormones Phase 2
12 Estrogens Phase 2
13 Estrogen Receptor Antagonists Phase 2
14 Estrogen Antagonists Phase 2
15 Aromatase Inhibitors Phase 2
16 Liver Extracts Phase 2
17 Antihypertensive Agents Phase 2
18 Endothelin Receptor Antagonists Phase 2
19 Protein Kinase Inhibitors Phase 2
20
Midodrine Approved Phase 1 133163-28-7, 42794-76-3 4195
21
Pentoxifylline Approved, Investigational Phase 1 6493-05-6 4740
22 Adrenergic alpha-Agonists Phase 1
23 Vasoconstrictor Agents Phase 1
24 Sympathomimetics Phase 1
25 Neurotransmitter Agents Phase 1
26 Adrenergic Agents Phase 1
27 Adrenergic Agonists Phase 1
28 Protective Agents Phase 1
29 Vasodilator Agents Phase 1
30 Radiation-Protective Agents Phase 1
31 Antioxidants Phase 1
32 Phosphodiesterase Inhibitors Phase 1
33 Platelet Aggregation Inhibitors Phase 1
34
Rifaximin Approved, Investigational 80621-81-4 6436173 46783403
35
Polygeline Experimental 66455-30-9
36 Gastrointestinal Agents

Interventional clinical trials:

(show all 12)
# Name Status NCT ID Phase Drugs
1 A Pilot Study of Norfloxacin for Hepatopulmonary Syndrome Completed NCT00362752 Phase 2 Norfloxacin;Placebo
2 Letrozole in Patients With Hepatopulmonary Syndrome Recruiting NCT04577001 Phase 2 Letrozole;Placebo
3 Bosentan for Treatment of Hepatopulmonary Syndrome in Patients With Liver Cirrhosis - a Prospective Double Blind Randomized Controlled Clinical Study Terminated NCT01518595 Phase 2 bosentan;Placebo
4 Sorafenib in Patients With Hepatopulmonary Syndrome: A Double-Blind Randomized Clinical Trial Terminated NCT02021929 Phase 2 Sorafenib;Placebo
5 A Phase 1 Proof-of-concept Clinical Trial Evaluating the Safety and Tolerability of Midodrine in Hepatopulmonary Syndrome Completed NCT03600870 Phase 1 Midodrine
6 Open Label Single Arm Pilot Study of Pentoxifylline in Advanced Hepatopulmonary Syndrome Terminated NCT00593658 Phase 1 pentoxifylline
7 Evaluation of Two Methods for the Echocardiographic Assessment of Intrapulmonary Vasodilatation in Patients With Liver Cirrhosis: Agitated Saline Versus Polygelatine Unknown status NCT00343122
8 Hepatopulmonary Syndrome and Postoperative Complications After Liver Transplantation : A Case-control Study. Unknown status NCT03092401
9 Investigation of the Prevalence of Hepatopulmonary Syndrome in Cirrhosis Patients Caused by Hepatitis B in Western China Unknown status NCT03435406
10 Efficacy of Pentoxifylline and Rifaximin Combination in Pentoxifylline Refractory Clinical and Subclinical Hepatopulmonary Syndrome Unknown status NCT01676597 pentoxifylline and rifaximin;Pentoxifylline and placebo
11 Effect of Transjugular Intrahepatic Portosystemic Shunt on Oxygenation in Cirrhotic Patients With Hepatopulmonary Syndrome Completed NCT02148536
12 Design and Rationale for a Randomized Controlled Crossover Trial of Supine Versus Upright Exercise in Patients With Hepatopulmonary Syndrome and Orthodeoxia Recruiting NCT04004104

Search NIH Clinical Center for Hepatopulmonary Syndrome

Cochrane evidence based reviews: hepatopulmonary syndrome

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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:

40
Liver, Brain, Lung, Endothelial, Bone Marrow, Smooth Muscle, Heart
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Publications for Hepatopulmonary Syndrome

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Articles related to Hepatopulmonary Syndrome:

(show top 50) (show all 1076)
# Title Authors PMID Year
1
Cyanosis revealing hepatopulmonary syndrome in a child with dyskeratosis congenita. 61 54
19953657 2010
2
Deleterious effect of nitric oxide inhibition in chronic hepatopulmonary syndrome. 61 54
17353701 2007
3
Reversal of severe hepato-pulmonary syndrome in congenital hepatic fibrosis after living-related liver transplantation. 61 54
17558074 2007
4
Recent advances in hepatopulmonary syndrome. 54 61
16323392 2005
5
Carboxyhemoglobin levels in cirrhotic patients with and without hepatopulmonary syndrome. 54 61
15685544 2005
6
[Significance of lung perfusion scanning with technetium labeled macroaggregated albumin and pulmonary function assay for diagnosis of early hepatopulmonary syndrome]. 61 54
11782297 2001
7
Pulmonary hypertension following hepatopulmonary syndrome in a patient with cirrhosis. 61 54
10453952 1999
8
Use of macroaggregated albumin lung perfusion scan to diagnose hepatopulmonary syndrome: a new approach. 61 54
9453490 1998
9
[Hepatopulmonary syndrome]. 54 61
9549117 1997
10
Tea polyphenols and Levofloxacin alleviate the lung injury of hepatopulmonary syndrome in common bile duct ligation rats through Endotoxin -TNF signaling. 61
33516071 2021
11
Transportal Technetium-99m Labeled Macroaggregated Albumin Scintigraphy to Quantify Occult Intrahepatic Microvascular Portosystemic Shunting. 61
33664925 2021
12
Hepatopulmonary syndrome presenting as unexplained dyspnea after ventricular septal defect closure. 61
31452074 2021
13
Combined lung and liver transplantation for noncirrhotic portal hypertension with severe hepatopulmonary syndrome in a patient with dyskeratosis congenita. 61
32777145 2021
14
Long-term outcome of liver transplantation for congenital extrahepatic portosystemic shunt. 61
33749116 2021
15
Analogies and differences between cirrhotic cardiomyopathy and hepatopulmonary syndrome. 61
33174630 2021
16
Transjugular intrahepatic portosystemic shunt creation may be associated with hyperplastic hepatic nodular lesions in the long term: an analysis of 18 pediatric and young adult patients. 61
33783576 2021
17
Hepatopulmonary Syndrome and Multiple Arteriovenous Fistulas in a Child with Niemann-Pick Disease. 61
33734872 2021
18
Non-invasive assessment of intrapulmonary shunt and ventilation to perfusion ratio in children with hepatopulmonary syndrome before and after liver transplantation. 61
33780759 2021
19
Reply to Comment on: Lung Perfusion Imaging with Technetium-99m-macroaggregated Albumin should be Combined with Contrast-enhanced Echocardiography for the Diagnosis of Hepatopulmonary Syndrome. 61
33586414 2021
20
Impact of hypoxemia on pediatric liver transplantation for hepatopulmonary syndrome. 61
33590638 2021
21
CX3CR1 regulates angiogenesis and activation of pro-angiogenic factors and triggers macrophage accumulation in experimental hepatopulmonary syndrome model. 61
32980177 2021
22
50 Years Ago in TheJournalofPediatrics: Hepatopulmonary Syndrome: Transplantation Is the Big Difference. 61
33487230 2021
23
Pulmonary Manifestations of Gastrointestinal, Pancreatic, and Liver Diseases in Children. 61
33228942 2021
24
Pulmonary abnormalities in liver disease: relevance to transplantation and outcome. 61
33636019 2021
25
Longterm Outcome of Liver Transplantation for Congenital Extrahepatic Portosystemic Shunt. 61
32463947 2021
26
Clinical outcomes and risk factors of hepatopulmonary syndrome in children. 61
33603173 2021
27
Severe hepatopulmonary syndrome with hypoxemia refractory to liver transplant: Recovery after 67 days of ECMO support. 61
33478326 2021
28
Indicators Of Hepatopulmonary Syndrome In Patients With Portal Hypertension. Its Various Aetiologies, Clinical Presentations And Outcome. 61
33774947 2021
29
Congenital portosystemic shunt occlusion with an Amplatzer PFO occlusion device: a case report. 61
33427962 2021
30
Recent advances in the approach to hepatopulmonary syndrome and portopulmonary hypertension. 61
33639700 2021
31
Liver epigenome changes in patients with hepatopulmonary syndrome: A pilot study. 61
33630849 2021
32
High-flow nasal cannula oxygen therapy: Alternative respiratory therapy for severe post-transplant hypoxemia in children with hepatopulmonary syndrome. 61
33099865 2020
33
[Splenic tyrosine kinase promotes pulmonary angiogenesis in rats with hepatopulmonary syndrome]. 61
33349837 2020
34
Cardiovascular disorders in patients with congenital portosystemic shunts: 23 years of experience in a tertiary referral centre. 61
33281106 2020
35
Modeling Outcomes in Children With Biliary Atresia With Native Liver After 2 Years of Age. 61
33305153 2020
36
Pediatric liver transplantation in severe hepatopulmonary syndrome and use of inhaled nitric oxide for post-transplant hypoxemia-a single center experience. 61
32881212 2020
37
Hepatopulmonary Syndrome in an Adolescent With Insidious Hypoxia and Small Intrahepatic Portal Venous Shunts: Posttransplant Benefit From Sildenafil. 61
32813578 2020
38
Hepatocellular carcinoma with tumor thrombus extends to the right atrium and portal vein: A case report. 61
33312435 2020
39
Noninvasive imaging assessment of portal hypertension. 61
32926209 2020
40
Correlation between hepatopulmonary syndrome and oxygen saturation pulse oximetry in cirrhotic patients. 61
33295413 2020
41
A novel application of pulmonary transit time to differentiate between benign and malignant pulmonary nodules using myocardial contrast echocardiography. 61
33231789 2020
42
Lung Perfusion Imaging with Technetium-99m Macroaggregated Albumin should be Combined with Contrast-enhanced Echocardiography for the Diagnosis of Hepatopulmonary Syndrome 61
33094580 2020
43
Therapeutic coil embolization of dominant shunt in hepatopulmonary syndrome enhances post-liver transplant respiratory recovery. 61
32436643 2020
44
Diagnosis of Hepatopulmonary Syndrome in a Large Integrated Health System. 61
33007510 2020
45
[Role of hepatic venous pressure gradient in the overall management process of portal hypertension]. 61
33053970 2020
46
Hepatopulmonary Syndrome and Portopulmonary Hypertension: Current Status and Implications for Liver Transplantation. 61
32905452 2020
47
Ligation of patent ductus venosus in a child with pulmonary arterial hypertension and hypersplenism: A case report. 61
32846835 2020
48
Endothelin-1 level as a predictor of hepatopulmonary syndrome in liver cirrhosis. 61
32567294 2020
49
Prevalence and determinants of hepatopulmonary syndrome in decompensated chronic liver disease. 61
32839954 2020
50
Platypnea-Orthodeoxia Syndrome Caused by an Intracardiac Shunt. 61
33472229 2020
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Variations for Hepatopulmonary Syndrome

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Expression for Hepatopulmonary Syndrome

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Search GEO for disease gene expression data for Hepatopulmonary Syndrome.
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Pathways for Hepatopulmonary Syndrome

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

(show all 40)
# Super pathways Score Top Affiliating Genes
1
ERK Signaling 63
Show member pathways
 13.74 VEGFA TNF NOS2 GDF2 CCL2 BMPR2
2
PEDF Induced Signaling 63
Show member pathways
 13.49 TNF NOS3 NOS2 GDF2 EDNRB EDNRA
3
Akt Signaling 63
Show member pathways
 13.33 TNF NOS3 GDF2 CCL2 BMPR2 AKT1
4
Cytokine Signaling in Immune system 65
Show member pathways
 13.28 VWF VEGFA TNF NOS2 HMOX1 CCL2
5
PAK Pathway 63
Show member pathways
 13.23 TNF GDF2 CCL2 BMPR2 AKT1 ACVRL1
6
PI3K-Akt signaling pathway 36
Show member pathways
 12.91 VWF VEGFA TNF NOS3 AKT1
7
Pathways in cancer 36
12.73 VEGFA NOS2 HMOX1 EDNRB EDNRA EDN1
8
Relaxin signaling pathway 36
Show member pathways
 12.58 VEGFA NOS3 NOS2 EDNRB EDN1 AKT1
9
Immune response IL-23 signaling pathway 23
Show member pathways
 12.52 TNF NOS3 NOS2 CCL2 AKT1
10
cGMP-PKG signaling pathway 36
Show member pathways
 12.47 NOS3 EDNRB EDNRA AKT1
11
VEGF Signaling 66
Show member pathways
 12.42 VEGFA NOS3 NOS2 AKT1
12
Development VEGF signaling via VEGFR2 - generic cascades 23
Show member pathways
 12.32 VEGFA NOS3 NOS2 CCL2 AKT1
13
Calcium signaling pathway 36
12.3 VEGFA NOS3 NOS2 EDNRB EDNRA
14
Colorectal Cancer Metastasis 63
12.12 VEGFA TNF NOS2 AKT1
15
TNF signaling pathway 36
11.91 TNF EDN1 CCL2 AKT1
16
Insulin resistance 36
Show member pathways
 11.89 TNF NOS3 AKT1
17
Interleukin-4 and 13 signaling 65
11.86 VEGFA TNF NOS2 HMOX1 CCL2 AKT1
18
TGF-beta Signaling Pathways 64
11.82 ENG AKT1 ACVRL1
19
Rheumatoid arthritis 36
11.8 VEGFA TNF CCL2
20
Signaling events mediated by VEGFR1 and VEGFR2 1
Show member pathways
 11.79 VEGFA NOS3 AKT1
21
HIF-1 signaling pathway 36
11.77 VEGFA NOS3 NOS2 HMOX1 EDN1 AKT1
22
Angiogenesis (CST) 4
11.72 VWF NOS3 HMOX1 ENG CCL2
23
AGE/RAGE pathway 1
11.63 NOS3 NOS2 AKT1
24
AGE-RAGE signaling pathway in diabetic complications 36
11.63 VEGFA TNF NOS3 EDN1 CCL2 AKT1
25
Photodynamic therapy-induced NF-kB survival signaling 1
Show member pathways
 11.61 VEGFA TNF NOS2
26
Lung fibrosis 1
11.6 TNF HMOX1 EDN1 CCL2
27
HIF1Alpha Pathway 63
11.53 VEGFA NOS3 EDN1
28
Hepatitis C and Hepatocellular Carcinoma 1
11.5 VEGFA NOS2 AKT1
29
Endothelins 1
11.5 EDNRB EDNRA EDN1 AKT1
30
MSP-RON Signaling 63
11.47 TNF NOS2 CCL2
31
Fluid shear stress and atherosclerosis 36
11.37 VEGFA TNF NOS3 HMOX1 EDN1 CCL2
32
Photodynamic therapy-induced HIF-1 survival signaling 1
11.34 VEGFA NOS2 EDN1
33
Validated transcriptional targets of AP1 family members Fra1 and Fra2 1
11.29 NOS3 HMOX1 CCL2
34
Endothelin Pathways 1
11.27 NOS3 EDNRB EDNRA EDN1
35
HIF-1-alpha transcription factor network 1
11.25 VEGFA NOS2 HMOX1 ENG EDN1 AKT1
36
Prostaglandin Synthesis and Regulation 1
11.23 EDNRB EDNRA EDN1
37
ALK1 signaling events 1
Show member pathways
 11.17 GDF2 BMPR2 ACVRL1
38
Angiogenesis (WikiPathways) 1
11.1 VEGFA NOS3 AKT1
39
NO-dependent CFTR activation (normal and CF) 23
10.78 NOS3 NOS2
40
Effects of Nitric Oxide 1
10.55 NOS3 NOS2
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GO Terms for Hepatopulmonary Syndrome

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

# Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 9.72 VEGFA TNF ENG BMPR2 ACVRL1
2 caveola GO:0005901 9.43 NOS3 HMOX1 BMPR2
3 platelet alpha granule lumen GO:0031093 9.33 VWF VEGFA ALB
4 extracellular space GO:0005615 9.32 VWF VEGFA TNF HMOX1 GDF2 ENG
5 Weibel-Palade body GO:0033093 8.96 VWF EDN1

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

(show top 50) (show all 73)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 10.18 VEGFA TNF GDF2 ENG EDN1 BMPR2
2 positive regulation of gene expression GO:0010628 10.09 VEGFA TNF NOS3 GDF2 ENG AKT1
3 heart development GO:0007507 10.03 ENG EDNRA EDN1 ACVRL1
4 in utero embryonic development GO:0001701 10 VEGFA EDNRA EDN1 ACVRL1
5 regulation of cell proliferation GO:0042127 10 TNF NOS2 ENG BMPR2
6 response to lipopolysaccharide GO:0032496 9.97 NOS3 NOS2 EDNRB EDN1
7 cellular response to lipopolysaccharide GO:0071222 9.97 TNF NOS2 EDNRB CCL2
8 cytokine-mediated signaling pathway GO:0019221 9.97 VEGFA TNF NOS2 HMOX1 CCL2 AKT1
9 positive regulation of protein phosphorylation GO:0001934 9.96 VEGFA TNF ENG EDNRB AKT1
10 cellular response to hypoxia GO:0071456 9.93 VEGFA HMOX1 EDN1 AKT1
11 cellular response to interferon-gamma GO:0071346 9.92 NOS2 EDN1 CCL2
12 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.91 VEGFA TNF AKT1
13 BMP signaling pathway GO:0030509 9.9 GDF2 ENG BMPR2 ACVRL1
14 cellular response to organic cyclic compound GO:0071407 9.89 TNF CCL2 AKT1
15 negative regulation of cysteine-type endopeptidase activity involved in apoptotic process GO:0043154 9.89 VEGFA TNF AKT1
16 cellular response to growth factor stimulus GO:0071363 9.89 BMPR2 AKT1 ACVRL1
17 vasculogenesis GO:0001570 9.88 VEGFA GDF2 ENG
18 angiogenesis GO:0001525 9.88 VEGFA HMOX1 GDF2 ENG CCL2 ACVRL1
19 positive regulation of MAP kinase activity GO:0043406 9.87 VEGFA TNF EDN1
20 negative regulation of gene expression GO:0010629 9.87 VEGFA TNF NOS2 ENG EDN1 AKT1
21 response to hormone GO:0009725 9.86 NOS3 NOS2 AKT1
22 sensory perception of pain GO:0019233 9.86 EDNRB EDN1 CCL2
23 positive regulation of blood vessel endothelial cell migration GO:0043536 9.85 VEGFA NOS3 AKT1
24 negative regulation of blood vessel endothelial cell migration GO:0043537 9.84 TNF GDF2 ACVRL1
25 positive regulation of endothelial cell migration GO:0010595 9.84 VEGFA EDN1 BMPR2 AKT1
26 positive regulation of nitric oxide biosynthetic process GO:0045429 9.83 TNF EDN1 AKT1
27 protein kinase B signaling GO:0043491 9.83 TNF CCL2 AKT1
28 positive regulation of smooth muscle cell proliferation GO:0048661 9.83 TNF HMOX1 EDN1 AKT1
29 cellular response to BMP stimulus GO:0071773 9.82 GDF2 BMPR2 ACVRL1
30 blood vessel remodeling GO:0001974 9.81 NOS3 BMPR2 ACVRL1
31 lipopolysaccharide-mediated signaling pathway GO:0031663 9.81 TNF CCL2 AKT1
32 positive regulation of pathway-restricted SMAD protein phosphorylation GO:0010862 9.81 GDF2 ENG BMPR2 ACVRL1
33 blood vessel morphogenesis GO:0048514 9.79 GDF2 EDN1 ACVRL1
34 activin receptor signaling pathway GO:0032924 9.78 GDF2 BMPR2 ACVRL1
35 vasoconstriction GO:0042310 9.77 EDNRB EDNRA EDN1
36 positive regulation of endothelial cell proliferation GO:0001938 9.77 VEGFA GDF2 BMPR2 AKT1 ACVRL1
37 endocardial cushion morphogenesis GO:0003203 9.76 NOS3 ENG ACVRL1
38 negative regulation of endothelial cell proliferation GO:0001937 9.76 TNF GDF2 ENG ACVRL1
39 enteric nervous system development GO:0048484 9.75 EDNRB EDNRA
40 negative regulation of macroautophagy GO:0016242 9.75 HMOX1 AKT1
41 macrophage chemotaxis GO:0048246 9.74 EDNRB CCL2
42 positive regulation of renal sodium excretion GO:0035815 9.74 EDNRB EDN1
43 positive regulation of urine volume GO:0035810 9.74 EDNRB EDN1
44 lymphangiogenesis GO:0001946 9.74 BMPR2 ACVRL1
45 response to transforming growth factor beta GO:0071559 9.74 ENG EDN1
46 nitric oxide biosynthetic process GO:0006809 9.74 NOS3 NOS2 AKT1
47 artery smooth muscle contraction GO:0014824 9.73 EDNRA EDN1
48 positive regulation of guanylate cyclase activity GO:0031284 9.73 NOS3 NOS2
49 artery development GO:0060840 9.73 BMPR2 ACVRL1
50 maternal placenta development GO:0001893 9.73 BMPR2 AKT1

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

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 10.41 VWF VEGFA TNF NOS3 NOS2 HMOX1
2 identical protein binding GO:0042802 10 VWF VEGFA TNF HMOX1 ENG ALB
3 activin binding GO:0048185 9.52 ENG ACVRL1
4 BMP binding GO:0036122 9.49 ENG BMPR2
5 transmembrane receptor protein serine/threonine kinase activity GO:0004675 9.48 BMPR2 ACVRL1
6 arginine binding GO:0034618 9.43 NOS3 NOS2
7 BMP receptor activity GO:0098821 9.4 BMPR2 ACVRL1
8 NADPH-hemoprotein reductase activity GO:0003958 9.37 NOS3 NOS2
9 cytokine activity GO:0005125 9.35 VEGFA TNF GDF2 EDN1 CCL2
10 tetrahydrobiopterin binding GO:0034617 9.32 NOS3 NOS2
11 nitric-oxide synthase activity GO:0004517 9.26 NOS3 NOS2
12 endothelin receptor activity GO:0004962 9.16 EDNRB EDNRA
13 transforming growth factor beta-activated receptor activity GO:0005024 8.8 ENG BMPR2 ACVRL1
Sources

Sources for Hepatopulmonary Syndrome

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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