MCID: HPT009
MIFTS: 49

Hepatopulmonary Syndrome

Categories: Endocrine diseases, Gastrointestinal diseases, Rare diseases

Aliases & Classifications for Hepatopulmonary Syndrome

MalaCards integrated aliases for Hepatopulmonary Syndrome:

Name: Hepatopulmonary Syndrome 12 52 54 43 15 17 71

Classifications:



External Ids:

Disease Ontology 12 DOID:900
ICD9CM 34 573.5
MeSH 43 D020065
ICD10 32 K76.81
UMLS 71 C0600452

Summaries for Hepatopulmonary Syndrome

NIH Rare Diseases : 52 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 idiopathic interstitial pneumonia and thrombotic microangiopathy, and has symptoms including hemoptysis An important gene associated with Hepatopulmonary Syndrome is ALB (Albumin), and among its related pathways/superpathways are PEDF Induced Signaling and Akt Signaling. The drugs Atorvastatin and Hypolipidemic Agents have been mentioned in the context of this disorder. Affiliated tissues include liver, lung and brain, and related phenotypes are cardiovascular system and homeostasis/metabolism

Wikipedia : 74 In medicine, hepatopulmonary syndrome is a syndrome of shortness of breath and hypoxemia (low oxygen... more...

Related Diseases for Hepatopulmonary Syndrome

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

(show top 50) (show all 361)
# Related Disease Score Top Affiliating Genes
1 idiopathic interstitial pneumonia 30.4 VEGFA EDN1 CCL2
2 thrombotic microangiopathy 30.3 VWF VEGFA
3 patent foramen ovale 30.2 VWF EDNRA EDN1 ALB
4 varicose veins 30.1 VWF VEGFA TNF CCL2
5 obstructive jaundice 30.0 TNF EDN1 ALB
6 visceral leishmaniasis 30.0 TNF NOS2 ALB
7 schistosomiasis 29.9 TNF CCL2 ALB
8 interstitial lung disease 29.8 TNF HMOX1 EDN1 CCL2 ALB
9 lung disease 29.8 VEGFA TNF HMOX1 EDN1 CCL2 ALB
10 pulmonary arteriovenous malformation 29.8 ENG ACVRL1
11 ischemia 29.8 VEGFA NOS3 NOS2 HMOX1 EDN1
12 portal hypertension 29.6 VWF VEGFA TNF NOS3 EDNRB EDNRA
13 pulmonary disease, chronic obstructive 29.6 TNF HMOX1 CCL2 ALB
14 pulmonary edema 29.6 TNF NOS3 NOS2 HMOX1 EDN1
15 juvenile rheumatoid arthritis 29.5 TNF CCL2 ALB
16 leishmaniasis 29.5 TNF NOS2 CCL2 ALB
17 pulmonary hypertension, primary, 1 29.4 ENG EDN1 BMPR2 ACVRL1
18 pneumonia 29.1 VEGFA TNF HMOX1 CCL2 ALB
19 arteriovenous malformation 29.1 VEGFA TNF ENG EDN1 BMPR2 ACVRL1
20 telangiectasis 29.0 VEGFA GDF2 ENG BMPR2 ACVRL1
21 sleep apnea 28.9 VEGFA TNF NOS3 EDN1 CCL2 ALB
22 hemangioma 28.9 VWF VEGFA ENG AKT1
23 pulmonary fibrosis, idiopathic 28.8 VEGFA TNF EDN1 CCL2 ALB AKT1
24 hereditary hemorrhagic telangiectasia 28.2 VWF VEGFA NOS3 GDF2 ENG BMPR2
25 myocardial infarction 26.8 VWF VEGFA TNF NOS3 HMOX1 ENG
26 pulmonary hypertension 26.7 VWF VEGFA TNF NOS3 NOS2 HMOX1
27 vascular disease 26.6 VWF VEGFA TNF NOS3 NOS2 HMOX1
28 heart disease 26.0 VWF VEGFA TNF NOS3 NOS2 HMOX1
29 liver cirrhosis 10.9
30 liver disease 10.8
31 pulmonary arterial hypertension associated with portal hypertension 10.7
32 cyanosis, transient neonatal 10.7
33 platelet membrane fluidity 10.6 NOS3 NOS2
34 biliary atresia 10.5
35 immuno-osseous dysplasia 10.5 NOS2 ALB
36 hemifacial spasm 10.5 VEGFA NOS3
37 koro 10.5 EDNRA ALB
38 pulmonary arterial hypertension associated with connective tissue disease 10.4 EDNRB EDNRA
39 acroosteolysis 10.4 VEGFA ALB
40 night blindness, congenital stationary, type 1h 10.4 EDNRB EDN1
41 bullous impetigo 10.4 EDNRB EDNRA
42 renovascular hypertension 10.4 NOS3 HMOX1 EDN1
43 pediatric angiosarcoma 10.4 VWF VEGFA
44 sexual disorder 10.4 NOS3 EDN1 ALB
45 renal hypertension 10.4 NOS3 EDN1 ALB
46 chronic thromboembolic pulmonary hypertension 10.4 EDN1 BMPR2
47 acute poststreptococcal glomerulonephritis 10.4 EDNRB EDN1 ALB
48 varicocele 10.3 NOS3 NOS2 ALB
49 respiratory failure 10.3
50 chronic mountain sickness 10.3 VEGFA NOS3 EDN1

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



Diseases related to Hepatopulmonary Syndrome

Symptoms & Phenotypes for Hepatopulmonary Syndrome

UMLS symptoms related to Hepatopulmonary Syndrome:


hemoptysis

MGI Mouse Phenotypes related to Hepatopulmonary Syndrome:

45 (show all 22)
# 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 10.4 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
3 growth/size/body region MP:0005378 10.36 ACVRL1 AKT1 BMPR2 EDN1 EDNRA EDNRB
4 behavior/neurological MP:0005386 10.35 ACVRL1 AKT1 BMPR2 EDNRA EDNRB ENG
5 digestive/alimentary MP:0005381 10.35 ACVRL1 ALB EDN1 EDNRA EDNRB ENG
6 cellular MP:0005384 10.33 AKT1 ALB BMPR2 EDNRA EDNRB HMOX1
7 immune system MP:0005387 10.32 AKT1 ALB BMPR2 CCL2 EDNRA EDNRB
8 hematopoietic system MP:0005397 10.31 ACVRL1 AKT1 BMPR2 EDNRA EDNRB ENG
9 endocrine/exocrine gland MP:0005379 10.3 AKT1 ALB EDN1 EDNRA EDNRB HMOX1
10 mortality/aging MP:0010768 10.3 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
11 muscle MP:0005369 10.25 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
12 embryo MP:0005380 10.22 ACVRL1 AKT1 BMPR2 EDN1 EDNRA EDNRB
13 craniofacial MP:0005382 10.19 ACVRL1 EDN1 EDNRA EDNRB ENG NOS2
14 nervous system MP:0003631 10.15 ACVRL1 AKT1 BMPR2 EDN1 EDNRA EDNRB
15 liver/biliary system MP:0005370 10.13 ACVRL1 AKT1 ALB HMOX1 NOS2 NOS3
16 integument MP:0010771 10.1 AKT1 BMPR2 EDNRA EDNRB ENG NOS2
17 normal MP:0002873 10.02 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
18 renal/urinary system MP:0005367 9.96 ACVRL1 ALB BMPR2 EDN1 EDNRA EDNRB
19 respiratory system MP:0005388 9.9 ACVRL1 AKT1 BMPR2 EDNRA EDNRB ENG
20 neoplasm MP:0002006 9.88 AKT1 ALB EDNRB NOS2 TNF VEGFA
21 skeleton MP:0005390 9.65 AKT1 BMPR2 EDN1 EDNRA EDNRB GDF2
22 vision/eye MP:0005391 9.23 BMPR2 EDNRA EDNRB GDF2 NOS2 NOS3

Drugs & Therapeutics for Hepatopulmonary Syndrome

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

(show all 39)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Atorvastatin Approved Phase 4 134523-00-5 60823
2 Hypolipidemic Agents Phase 4
3 Anticholesteremic Agents Phase 4
4 Antimetabolites Phase 4
5 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 4
6 Lipid Regulating Agents Phase 4
7
Norfloxacin Approved Phase 2 70458-96-7 4539
8
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
9
Iodine Approved, Investigational Phase 2 7553-56-2 807
10
Bosentan Approved, Investigational Phase 2 147536-97-8 104865
11 Anti-Infective Agents Phase 2
12 Anti-Bacterial Agents Phase 2
13 Cytochrome P-450 Enzyme Inhibitors Phase 2
14 Topoisomerase Inhibitors Phase 2
15 Cytochrome P-450 CYP1A2 Inhibitors Phase 2
16 Liver Extracts Phase 2
17 Protein Kinase Inhibitors Phase 2
18 cadexomer iodine Phase 2
19 Endothelin Receptor Antagonists Phase 2
20 Antihypertensive Agents Phase 2
21
Midodrine Approved Phase 1 133163-28-7, 42794-76-3 4195
22
Pentoxifylline Approved, Investigational Phase 1 6493-05-6 4740
23 Neurotransmitter Agents Phase 1
24 Adrenergic Agonists Phase 1
25 Sympathomimetics Phase 1
26 Vasoconstrictor Agents Phase 1
27 Autonomic Agents Phase 1
28 Adrenergic Agents Phase 1
29 Platelet Aggregation Inhibitors Phase 1
30 Protective Agents Phase 1
31 Vasodilator Agents Phase 1
32 Free Radical Scavengers Phase 1
33 Radiation-Protective Agents Phase 1
34 Phosphodiesterase Inhibitors Phase 1
35 Antioxidants Phase 1
36
Rifaximin Approved, Investigational 80621-81-4 6436173 46783403
37 Hematinics
38 Gastrointestinal Agents
39 Polygeline

Interventional clinical trials:

(show all 13)
# Name Status NCT ID Phase Drugs
1 Statin for Preventing Hepatocellular Carcinoma Recurrence After Curative Treatment: a Double-blind Randomized Placebo-controlled Trial Recruiting NCT03024684 Phase 4 Atorvastatin;Placebo Oral Tablet
2 A Pilot Study of Norfloxacin for Hepatopulmonary Syndrome Completed NCT00362752 Phase 2 Norfloxacin;Placebo
3 Sorafenib in Patients With Hepatopulmonary Syndrome: A Double-Blind Randomized Clinical Trial Terminated NCT02021929 Phase 2 Sorafenib;Placebo
4 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
5 A Phase 1 Proof-of-concept Clinical Trial Evaluating the Safety and Tolerability of Midodrine in Hepatopulmonary Syndrome Recruiting NCT03600870 Phase 1 Midodrine
6 Open Label Single Arm Pilot Study of Pentoxifylline in Advanced Hepatopulmonary Syndrome Terminated NCT00593658 Phase 1 pentoxifylline
7 Hepatopulmonary Syndrome and Postoperative Complications After Liver Transplantation : A Case-control Study. Unknown status NCT03092401
8 Efficacy of Pentoxifylline and Rifaximin Combination in Pentoxifylline Refractory Clinical and Subclinical Hepatopulmonary Syndrome Unknown status NCT01676597 pentoxifylline and rifaximin;Pentoxifylline and placebo
9 Evaluation of Two Methods for the Echocardiographic Assessment of Intrapulmonary Vasodilatation in Patients With Liver Cirrhosis: Agitated Saline Versus Polygelatine Unknown status NCT00343122
10 Effect of Transjugular Intrahepatic Portosystemic Shunt on Oxygenation in Cirrhotic Patients With Hepatopulmonary Syndrome Completed NCT02148536
11 Investigation of the Prevalence of Hepatopulmonary Syndrome in Cirrhosis Patients Caused by Hepatitis B in Western China Recruiting NCT03435406
12 Physiological Effects of Supine Versus Upright Exercise in Patients With Hepatopulmonary Syndrome (HPS) and Orthodeoxia Recruiting NCT04004104
13 Vascular Liver Disease Evaluation, Follow Up and Non-Invasive Diagnostics Program Recruiting NCT03717792

Search NIH Clinical Center for Hepatopulmonary Syndrome

Cochrane evidence based reviews: hepatopulmonary syndrome

Genetic Tests for Hepatopulmonary Syndrome

Anatomical Context for Hepatopulmonary Syndrome

MalaCards organs/tissues related to Hepatopulmonary Syndrome:

40
Liver, Lung, Brain, Heart, Skin, Endothelial, Bone

Publications for Hepatopulmonary Syndrome

Articles related to Hepatopulmonary Syndrome:

(show top 50) (show all 1009)
# Title Authors PMID Year
1
Cyanosis revealing hepatopulmonary syndrome in a child with dyskeratosis congenita. 54 61
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. 54 61
17558074 2007
4
Recent advances in hepatopulmonary syndrome. 54 61
16323392 2005
5
Carboxyhemoglobin levels in cirrhotic patients with and without hepatopulmonary syndrome. 61 54
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]. 61 54
9549117 1997
10
Hepatopulmonary Syndrome Mimics Pulmonary Embolism on Pulmonary Ventilation/Perfusion SPECT/CT Study. 61
31789915 2020
11
Epigenome-wide base-resolution profiling of DNA methylation in chorionic villi of fetuses with Down syndrome by methyl-capture sequencing. 61
31801612 2019
12
Value of Contrast Transesophageal Echocardiography in the Detection of Intrapulmonary Vascular Dilatations in Hepatosplenic Schistosomiasis. 61
31596323 2019
13
Pulmonary Complications of Portal Hypertension. 61
31563218 2019
14
Pulmonary Hypertension Post Liver-Kidney Transplant. 61
31675782 2019
15
Hepatocyte-derived exosomal MiR-194 activates PMVECs and promotes angiogenesis in hepatopulmonary syndrome. 61
31700002 2019
16
High-Dose Oral Garlic is Associated with a Decrease in Intrapulmonary Shunting in Severe Hepatopulmonary Syndrome. 61
31701077 2019
17
Platypnoea and orthodeoxia in the hepatopulmonary syndrome. 61
31776152 2019
18
Does Biliodigestive Anastomosis Have Any Effect on the Reversal of Hepatopulmonary Syndrome in a Biliary Cirrhosis Experimental Model? 61
31076984 2019
19
MiR145-5p inhibits proliferation of PMVECs via PAI-1 in experimental hepatopulmonary syndrome rat pulmonary microvascular hyperplasia. 61
31649116 2019
20
Underrated Cause of Hypoxemia and Stroke in Patient with Cirrhosis. 61
31656945 2019
21
Vascular syndromes in liver cirrhosis. 61
30980261 2019
22
A high-dose rapamycin treatment alleviates hepatopulmonary syndrome in cirrhotic rats. 61
31567652 2019
23
Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes. 61
31549332 2019
24
Reversal of life-threatening hepatopulmonary syndrome in Gaucher disease by imiglucerase enzyme replacement therapy. 61
31309038 2019
25
[Analysis of clinical features and targeted drug therapy of portopulmonary hypertension]. 61
31550806 2019
26
Platypnoea-orthodeoxia syndrome: beware of investigations undertaken supine. 61
31147400 2019
27
CX3CR1 participates in pulmonary angiogenesis in experimental hepatopulmonary syndrome mice through inhibiting AKT/ERK signaling pathway and regulating NO/NOS release. 61
31378907 2019
28
Dyspnoea, clubbing, cirrhosis, and bubbles in both sides of the heart suggests hepatopulmonary syndrome. 61
31402029 2019
29
Multiple recurrent intracerebral hemorrhages and hepatopulmonary syndrome. 61
31583193 2019
30
Loss of cell polarity regulated by PTEN/Cdc42 enrolled in the process of Hepatopulmonary Syndrome. 61
31144461 2019
31
Sorafenib in Hepatopulmonary Syndrome: A Randomized, Double-Blind, Placebo-Controlled Trial. 61
30816637 2019
32
Translational Research in Hepatopulmonary Syndrome: Lessons Learned Despite Negative Results. 61
31206237 2019
33
Fatty Liver and Autoimmune Hepatitis: Two Forms of Liver Involvement in Lipodystrophies. 61
31559327 2019
34
Hepatopulmonary Syndrome in a Thirteen Year Old Boy: A Case Report. 61
31579635 2019
35
Hepatopulmonary syndrome presenting as unexplained dyspnea after ventricular septal defect closure. 61
31452074 2019
36
Current status of hepatopulmonary syndrome in liver transplantation in Japan: a Japanese multicenter analysis. 61
31069999 2019
37
A Role for Alveolar Exhaled Nitric Oxide Measurement in the Diagnosis of Hepatopulmonary Syndrome. 61
31306341 2019
38
Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study. 61
31211875 2019
39
Pulmonary Hypertension that Developed During Treatment for Hepatopulmonary Syndrome and Pulmonary Arteriovenous Malformation. 61
30799344 2019
40
Changes in arterial oxygenation after portal decompression in Budd-Chiari syndrome patients with hepatopulmonary syndrome. 61
30506220 2019
41
Krüppel-like factor 6 mediates pulmonary angiogenesis in rat experimental hepatopulmonary syndrome and is aggravated by bone morphogenetic protein 9. 61
31189661 2019
42
Melatonin effects on pulmonary tissue in the experimental model of Hepatopulmonary Syndrome. 61
31166552 2019
43
Breathlessness in liver disease: A case of hepatopulmonary syndrome . 61
31092521 2019
44
Time-dependent reversal of significant intrapulmonary shunt after liver transplantation. 61
29502364 2019
45
Cyclooxygenase-2 regulates HPS patient serum induced-directional collective HPMVEC migration via PKC/Rac signaling pathway. 61
30660713 2019
46
Ischemic Cholangiopathy: Insight From an Explant Liver Allograft Affected by Severe Post-transplant Hypoxemia Due to Hepatopulmonary Syndrome: A Case Report. 61
30979439 2019
47
Type I congenital extrahepatic portosystemic shunt treated by orthotopic liver transplantation: A case report. 61
31024963 2019
48
Abernethy malformation type 2: varied presentation, management and outcome. 61
30262201 2019
49
Successful liver transplantation in common variable immune deficiency with reversal of hepatopulmonary syndrome. 61
30948390 2019
50
Extracorporeal Membrane Oxygenation in a Pediatric Patient with Hepatopulmonary Syndrome and Interrupted Inferior Vena Cava After Living Related Liver Donation. 61
29613889 2019

Variations for Hepatopulmonary Syndrome

Expression for Hepatopulmonary Syndrome

Search GEO for disease gene expression data for Hepatopulmonary Syndrome.

Pathways for Hepatopulmonary Syndrome

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

(show all 36)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.51 TNF NOS3 NOS2 GDF2 EDNRB EDNRA
2
Show member pathways
13.35 TNF NOS3 GDF2 CCL2 BMPR2 AKT1
3
Show member pathways
13.29 VWF VEGFA TNF NOS2 HMOX1 CCL2
4 12.73 VEGFA NOS2 HMOX1 EDNRB EDNRA EDN1
5
Show member pathways
12.58 VEGFA NOS3 NOS2 EDNRB EDN1 AKT1
6
Show member pathways
12.52 TNF NOS3 NOS2 CCL2 AKT1
7
Show member pathways
12.31 VEGFA NOS3 NOS2 CCL2 AKT1
8
Show member pathways
12.22 NOS3 NOS2 EDNRB EDNRA
9
Show member pathways
12.16 TNF NOS2 HMOX1 EDN1
10 12.12 VEGFA TNF NOS2 AKT1
11 11.91 TNF EDN1 CCL2 AKT1
12 11.86 VEGFA TNF NOS2 HMOX1 CCL2 AKT1
13 11.82 ENG AKT1 ACVRL1
14 11.81 VEGFA TNF CCL2
15
Show member pathways
11.8 VEGFA NOS3 AKT1
16 11.77 VEGFA NOS3 NOS2 HMOX1 EDN1 AKT1
17 11.72 VWF NOS3 HMOX1 ENG CCL2
18 11.68 TNF NOS2 CCL2
19 11.64 NOS3 NOS2 AKT1
20 11.63 VEGFA TNF NOS3 EDN1 CCL2 AKT1
21
Show member pathways
11.61 VEGFA TNF NOS2
22 11.6 TNF HMOX1 EDN1 CCL2
23 11.53 VEGFA NOS3 EDN1
24 11.5 VEGFA NOS2 AKT1
25 11.5 EDNRB EDNRA EDN1 AKT1
26 11.46 TNF NOS2 CCL2
27 11.37 VEGFA TNF NOS3 HMOX1 EDN1 CCL2
28 11.33 VEGFA NOS2 EDN1
29 11.28 NOS3 HMOX1 CCL2
30 11.25 VEGFA NOS2 HMOX1 ENG EDN1 AKT1
31 11.24 NOS3 EDNRB EDNRA EDN1
32 11.22 EDNRB EDNRA EDN1
33
Show member pathways
11.16 GDF2 BMPR2 ACVRL1
34 11.09 VEGFA NOS3 AKT1
35 10.78 NOS3 NOS2
36 10.55 NOS3 NOS2

GO Terms for Hepatopulmonary Syndrome

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.61 VEGFA TNF HMOX1 GDF2 ENG EDN1
2 caveola GO:0005901 9.5 NOS3 HMOX1 BMPR2
3 platelet alpha granule lumen GO:0031093 9.43 VWF VEGFA ALB
4 cell GO:0005623 9.23 NOS3 NOS2 HMOX1 GDF2 EDNRB EDNRA
5 Weibel-Palade body GO:0033093 9.16 VWF EDN1

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

(show top 50) (show all 71)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 10.19 VEGFA TNF GDF2 ENG EDN1 BMPR2
2 heart development GO:0007507 10.02 ENG EDNRA EDN1 ACVRL1
3 positive regulation of gene expression GO:0010628 10.02 VEGFA TNF NR0B2 NOS3 GDF2 ENG
4 in utero embryonic development GO:0001701 10 VEGFA NOS3 EDNRA EDN1 ACVRL1
5 cellular response to lipopolysaccharide GO:0071222 9.97 TNF NOS2 EDNRB CCL2
6 response to lipopolysaccharide GO:0032496 9.97 NOS3 NOS2 EDNRB EDN1
7 cytokine-mediated signaling pathway GO:0019221 9.97 VEGFA TNF NOS2 HMOX1 CCL2 AKT1
8 positive regulation of protein phosphorylation GO:0001934 9.96 VEGFA TNF ENG EDNRB AKT1
9 cellular response to hypoxia GO:0071456 9.93 VEGFA HMOX1 EDN1 AKT1
10 cellular response to interferon-gamma GO:0071346 9.91 NOS2 EDN1 CCL2
11 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.91 VEGFA TNF AKT1
12 BMP signaling pathway GO:0030509 9.91 GDF2 ENG BMPR2 ACVRL1
13 cellular response to growth factor stimulus GO:0071363 9.89 BMPR2 AKT1 ACVRL1
14 cellular response to organic cyclic compound GO:0071407 9.88 TNF CCL2 AKT1
15 positive regulation of MAP kinase activity GO:0043406 9.87 VEGFA TNF EDN1
16 vasculogenesis GO:0001570 9.87 VEGFA GDF2 ENG
17 response to hormone GO:0009725 9.87 NOS3 NOS2 AKT1
18 sensory perception of pain GO:0019233 9.85 EDNRB EDN1 CCL2
19 positive regulation of endothelial cell migration GO:0010595 9.85 VEGFA EDN1 BMPR2
20 positive regulation of blood vessel endothelial cell migration GO:0043536 9.85 VEGFA NOS3 AKT1
21 response to hypoxia GO:0001666 9.85 VEGFA NOS2 HMOX1 EDNRA EDN1 ACVRL1
22 positive regulation of nitric oxide biosynthetic process GO:0045429 9.84 TNF EDN1 AKT1
23 protein kinase B signaling GO:0043491 9.83 TNF CCL2 AKT1
24 positive regulation of smooth muscle cell proliferation GO:0048661 9.83 TNF HMOX1 EDN1 AKT1
25 negative regulation of endothelial cell proliferation GO:0001937 9.82 TNF GDF2 ACVRL1
26 cellular response to BMP stimulus GO:0071773 9.81 GDF2 BMPR2 ACVRL1
27 positive regulation of pathway-restricted SMAD protein phosphorylation GO:0010862 9.81 GDF2 ENG BMPR2 ACVRL1
28 blood vessel remodeling GO:0001974 9.8 NOS3 BMPR2 ACVRL1
29 positive regulation of endothelial cell proliferation GO:0001938 9.8 VEGFA GDF2 BMPR2 AKT1 ACVRL1
30 positive regulation of angiogenesis GO:0045766 9.8 VEGFA NOS3 HMOX1 GDF2 ENG ACVRL1
31 angiogenesis GO:0001525 9.8 VEGFA NOS3 HMOX1 GDF2 ENG CCL2
32 blood vessel morphogenesis GO:0048514 9.79 GDF2 EDN1 ACVRL1
33 activin receptor signaling pathway GO:0032924 9.76 GDF2 BMPR2 ACVRL1
34 positive regulation of BMP signaling pathway GO:0030513 9.76 GDF2 ENG BMPR2 ACVRL1
35 endocardial cushion morphogenesis GO:0003203 9.75 NOS3 ENG ACVRL1
36 macrophage chemotaxis GO:0048246 9.74 EDNRB CCL2
37 positive regulation of renal sodium excretion GO:0035815 9.74 EDNRB EDN1
38 positive regulation of urine volume GO:0035810 9.74 EDNRB EDN1
39 negative regulation of macroautophagy GO:0016242 9.74 HMOX1 AKT1
40 enteric nervous system development GO:0048484 9.74 EDNRB EDNRA
41 lymphangiogenesis GO:0001946 9.74 BMPR2 ACVRL1
42 vasoconstriction GO:0042310 9.74 EDNRB EDNRA EDN1
43 response to transforming growth factor beta GO:0071559 9.73 ENG EDN1
44 positive regulation of chemokine biosynthetic process GO:0045080 9.73 TNF HMOX1
45 positive regulation of guanylate cyclase activity GO:0031284 9.73 NOS3 NOS2
46 maternal placenta development GO:0001893 9.73 BMPR2 AKT1
47 cofactor metabolic process GO:0051186 9.73 NOS3 AKT1
48 lipopolysaccharide-mediated signaling pathway GO:0031663 9.73 TNF NOS3 CCL2 AKT1
49 artery smooth muscle contraction GO:0014824 9.72 EDNRA EDN1
50 response to fluid shear stress GO:0034405 9.72 NOS3 AKT1

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

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.89 VWF VEGFA TNF NR0B2 NOS3 NOS2
2 protein homodimerization activity GO:0042803 9.7 VWF VEGFA NR0B2 NOS2 HMOX1 ENG
3 transmembrane receptor protein serine/threonine kinase activity GO:0004675 9.51 BMPR2 ACVRL1
4 BMP binding GO:0036122 9.49 ENG BMPR2
5 arginine binding GO:0034618 9.48 NOS3 NOS2
6 transforming growth factor beta-activated receptor activity GO:0005024 9.46 BMPR2 ACVRL1
7 BMP receptor activity GO:0098821 9.43 BMPR2 ACVRL1
8 NADPH-hemoprotein reductase activity GO:0003958 9.4 NOS3 NOS2
9 tetrahydrobiopterin binding GO:0034617 9.37 NOS3 NOS2
10 nitric-oxide synthase activity GO:0004517 9.32 NOS3 NOS2
11 endothelin receptor activity GO:0004962 9.26 EDNRB EDNRA
12 cytokine activity GO:0005125 9.02 VEGFA TNF GDF2 EDN1 CCL2

Sources for Hepatopulmonary Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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