MCID: HPT009
MIFTS: 48

Hepatopulmonary Syndrome

Categories: 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
SNOMED-CT 67 371067004
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 thrombotic microangiopathy and patent foramen ovale, 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 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 367)
# Related Disease Score Top Affiliating Genes
1 thrombotic microangiopathy 30.3 VWF VEGFA
2 patent foramen ovale 30.2 VWF EDNRA EDN1 ALB
3 visceral leishmaniasis 30.2 TNF NOS2 ALB
4 pulmonary arteriovenous malformation 30.0 ENG ACVRL1
5 end stage renal disease 30.0 VEGFA TNF NOS3 ALB
6 varicose veins 30.0 VWF VEGFA TNF CCL2
7 pulmonary edema 29.8 TNF NOS3 NOS2 HMOX1 EDN1
8 ischemia 29.8 VEGFA NOS3 NOS2 HMOX1 EDN1
9 pulmonary hypertension, primary, 1 29.8 ENG EDN1 BMPR2 ACVRL1
10 interstitial lung disease 29.7 TNF HMOX1 EDN1 CCL2 ALB
11 schistosomiasis 29.7 VEGFA TNF CCL2 ALB
12 lung disease 29.7 VEGFA TNF HMOX1 EDN1 CCL2 ALB
13 portal hypertension 29.6 VWF VEGFA TNF NOS3 NOS2 EDNRB
14 arteriovenous malformation 29.5 VEGFA TNF ENG EDN1 BMPR2 ACVRL1
15 pneumonia 29.5 TNF HMOX1 CCL2 ALB
16 idiopathic interstitial pneumonia 29.5 VEGFA EDN1 CCL2
17 telangiectasis 29.4 VEGFA GDF2 ENG BMPR2 ACVRL1
18 obstructive jaundice 29.4 TNF EDN1 CCL2 ALB
19 leishmaniasis 29.3 TNF NOS2 CCL2 ALB
20 pulmonary disease, chronic obstructive 29.2 TNF HMOX1 CCL2 ALB
21 hemangioma 29.1 VWF VEGFA ENG AKT1
22 sleep apnea 28.8 VEGFA TNF NOS3 EDN1 CCL2 ALB
23 pulmonary fibrosis, idiopathic 28.7 VEGFA TNF EDN1 CCL2 ALB AKT1
24 juvenile rheumatoid arthritis 28.7 TNF CCL2 ALB
25 hereditary hemorrhagic telangiectasia 28.6 VWF VEGFA NOS3 GDF2 ENG BMPR2
26 myocardial infarction 26.9 VWF VEGFA TNF NOS3 HMOX1 ENG
27 vascular disease 26.8 VWF VEGFA TNF NOS3 NOS2 HMOX1
28 pulmonary hypertension 26.3 VWF VEGFA TNF NOS3 NOS2 HMOX1
29 heart disease 26.2 VWF VEGFA TNF NOS3 NOS2 HMOX1
30 liver cirrhosis 10.9
31 liver disease 10.8
32 pulmonary arterial hypertension associated with portal hypertension 10.7
33 cyanosis, transient neonatal 10.7
34 platelet membrane fluidity 10.5 NOS3 NOS2
35 biliary atresia 10.5
36 hemifacial spasm 10.5 VEGFA NOS3
37 immuno-osseous dysplasia 10.5 NOS2 ALB
38 spotted fever rickettsiosis 10.4 TNF NOS2
39 koro 10.4 EDNRA ALB
40 pulmonary arterial hypertension associated with connective tissue disease 10.4 EDNRB EDNRA
41 renovascular hypertension 10.4 NOS3 HMOX1 EDN1
42 hemorrhoid 10.4 TNF NOS2 ALB
43 renal hypertension 10.4 NOS3 EDN1 ALB
44 hypertensive encephalopathy 10.4 VEGFA NOS3 ALB
45 bullous impetigo 10.4 EDNRB EDNRA
46 sexual disorder 10.4 NOS3 EDN1 ALB
47 acute poststreptococcal glomerulonephritis 10.4 EDNRB EDN1 ALB
48 hemorrhagic cystitis 10.4 TNF HMOX1 ALB
49 cerebral artery occlusion 10.4 TNF NOS3 NOS2
50 respiratory failure 10.3

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 21)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.49 ACVRL1 AKT1 ALB BMPR2 COL18A1 EDN1
2 homeostasis/metabolism MP:0005376 10.41 ACVRL1 AKT1 ALB BMPR2 COL18A1 EDN1
3 behavior/neurological MP:0005386 10.38 ACVRL1 AKT1 BMPR2 COL18A1 EDNRA EDNRB
4 cellular MP:0005384 10.36 AKT1 ALB BMPR2 COL18A1 EDNRA EDNRB
5 digestive/alimentary MP:0005381 10.35 ACVRL1 ALB EDN1 EDNRA EDNRB ENG
6 growth/size/body region MP:0005378 10.35 ACVRL1 AKT1 BMPR2 EDN1 EDNRA EDNRB
7 immune system MP:0005387 10.31 AKT1 ALB BMPR2 CCL2 EDNRA EDNRB
8 hematopoietic system MP:0005397 10.3 ACVRL1 AKT1 BMPR2 EDNRA EDNRB ENG
9 mortality/aging MP:0010768 10.3 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
10 endocrine/exocrine gland MP:0005379 10.29 AKT1 ALB EDN1 EDNRA EDNRB HMOX1
11 muscle MP:0005369 10.25 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
12 craniofacial MP:0005382 10.23 ACVRL1 COL18A1 EDN1 EDNRA EDNRB ENG
13 embryo MP:0005380 10.21 ACVRL1 AKT1 BMPR2 EDN1 EDNRA EDNRB
14 nervous system MP:0003631 10.18 ACVRL1 AKT1 BMPR2 COL18A1 EDN1 EDNRA
15 liver/biliary system MP:0005370 10.13 ACVRL1 AKT1 ALB HMOX1 NOS2 NOS3
16 normal MP:0002873 10.02 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRA
17 renal/urinary system MP:0005367 10 ACVRL1 ALB BMPR2 COL18A1 EDN1 EDNRA
18 neoplasm MP:0002006 9.95 AKT1 ALB COL18A1 EDNRB NOS2 TNF
19 respiratory system MP:0005388 9.9 ACVRL1 AKT1 BMPR2 EDNRA EDNRB ENG
20 skeleton MP:0005390 9.7 AKT1 BMPR2 COL18A1 EDN1 EDNRA EDNRB
21 vision/eye MP:0005391 9.28 BMPR2 COL18A1 EDNRA EDNRB GDF2 NOS2

Drugs & Therapeutics for Hepatopulmonary Syndrome

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

(show all 35)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Atorvastatin Approved Phase 4 134523-00-5 60823
2 Hypolipidemic Agents Phase 4
3 Lipid Regulating Agents Phase 4
4 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 4
5 Anticholesteremic Agents Phase 4
6 Antimetabolites 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 Liver Extracts Phase 2
15 Protein Kinase Inhibitors Phase 2
16 cadexomer iodine Phase 2
17 Antihypertensive Agents Phase 2
18 Endothelin Receptor Antagonists Phase 2
19
Midodrine Approved Phase 1 133163-28-7, 42794-76-3 4195
20
Pentoxifylline Approved, Investigational Phase 1 6493-05-6 4740
21 Adrenergic alpha-Agonists Phase 1
22 Adrenergic Agents Phase 1
23 Vasoconstrictor Agents Phase 1
24 Adrenergic Agonists Phase 1
25 Sympathomimetics Phase 1
26 Neurotransmitter Agents Phase 1
27 Phosphodiesterase Inhibitors Phase 1
28 Antioxidants Phase 1
29 Vasodilator Agents Phase 1
30 Platelet Aggregation Inhibitors Phase 1
31 Protective Agents Phase 1
32 Radiation-Protective Agents Phase 1
33
Rifaximin Approved, Investigational 80621-81-4 6436173 46783403
34 Gastrointestinal Agents
35 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 Investigation of the Prevalence of Hepatopulmonary Syndrome in Cirrhosis Patients Caused by Hepatitis B in Western China Unknown status NCT03435406
8 Hepatopulmonary Syndrome and Postoperative Complications After Liver Transplantation : A Case-control Study. Unknown status NCT03092401
9 Efficacy of Pentoxifylline and Rifaximin Combination in Pentoxifylline Refractory Clinical and Subclinical Hepatopulmonary Syndrome Unknown status NCT01676597 pentoxifylline and rifaximin;Pentoxifylline and placebo
10 Evaluation of Two Methods for the Echocardiographic Assessment of Intrapulmonary Vasodilatation in Patients With Liver Cirrhosis: Agitated Saline Versus Polygelatine Unknown status NCT00343122
11 Effect of Transjugular Intrahepatic Portosystemic Shunt on Oxygenation in Cirrhotic Patients With Hepatopulmonary Syndrome Completed NCT02148536
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 1037)
# 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. 54 61
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. 61 54
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. 54 61
9453490 1998
9
[Hepatopulmonary syndrome]. 54 61
9549117 1997
10
Endothelin-1 level as a predictor of hepatopulmonary syndrome in liver cirrhosis. 61
32567294 2020
11
Hepatopulmonary Syndrome and Portopulmonary Hypertension: Management in Liver Transplantation in the Horizon 2020. 61
32278579 2020
12
Persistent hypoxaemia following pneumonia unmasking hepatopulmonary syndrome. 61
32513770 2020
13
Hepatopulmonary syndrome revealed via echocardiography in the upright position. 61
32463571 2020
14
Therapeutic coil embolization of dominant shunt in hepatopulmonary syndrome enhances post-liver transplant respiratory recovery. 61
32436643 2020
15
Reduced circulating BMP10 and BMP9 and elevated endoglin are associated with disease severity, decompensation and pulmonary vascular syndromes in patients with cirrhosis. 61
32454407 2020
16
Living-donor liver transplantation for Abernethy malformation - case report and review of literature. 61
32457268 2020
17
Successful liver transplantation in short telomere syndromes without bone marrow failure due to DKC1 mutation. 61
32166868 2020
18
Long-term Outcomes and Health Perceptions in Pediatric-onset Portal Hypertension Complicated by Varices. 61
31990868 2020
19
Cancer spectrum and outcomes in the Mendelian short telomere syndromes. 61
32076714 2020
20
Pulmonary manifestations of chronic liver disease: a comprehensive review. 61
32382226 2020
21
Chronic active Epstein-Barr virus infection with intrapulmonary shunting: A case report. 61
31983614 2020
22
Long-term outcome of liver transplantation for congenital extrahepatic portosystemic shunt. 61
32463947 2020
23
Plasma perfusion combined with plasma exchange in chronic hepatitis B-related acute-on-chronic liver failure patients. 61
32472309 2020
24
[Diagnosis and therapeutic strategies for hepatopulmonary syndrome]. 61
32536053 2020
25
Comparison of Two Diagnostic Criteria for Hepatopulmonary Syndrome-High Prevalence in Biliary Atresia. 61
31939865 2020
26
Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes. 61
31549332 2020
27
Diosmin enhances the anti-angiogenic activity of sildenafil and pentoxifylline against hepatopulmonary syndrome via regulation of TNF-α/VEGF, IGF-1/PI3K/AKT, and FGF-1/ANG-2 signaling pathways. 61
32050083 2020
28
Resources Utilization After Liver Transplantation in Patients With and Without Hepatopulmonary Syndrome: Cleveland Clinic Experience. 61
32309631 2020
29
A Role for Alveolar Exhaled Nitric Oxide Measurement in the Diagnosis of Hepatopulmonary Syndrome. 61
31306341 2020
30
Hepatopulmonary Syndrome: A New Name for Old Complexities. 61
31931611 2020
31
Hepatopulmonary Syndrome. 61
32130817 2020
32
Technetium-99m-labeled macroaggregated albumin lung perfusion scan for diagnosis of hepatopulmonary syndrome: A prospective study comparing brain uptake and whole-body uptake. 61
32205999 2020
33
Natural history, risk factors, and outcome of hepatopulmonary syndrome in pediatric liver diseases. 61
32189246 2020
34
Hepatopulmonary Syndrome and Portopulmonary Hypertension: The Pulmonary Vascular Enigmas of Liver Disease. 61
32140210 2020
35
Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study. 61
31211875 2020
36
Hepatopulmonary Syndrome with Right-to-left Shunt in Cirrhotic Patients Using Macro-Aggregated Albumin Lung Perfusion Scan: Comparison with Contrast Echocardiography and Association with Clinical Data 61
32079381 2020
37
Hepatopulmonary Syndrome Mimics Pulmonary Embolism on Pulmonary Ventilation/Perfusion SPECT/CT Study. 61
31789915 2020
38
Pulmonary capillary hemangiomatosis or hepatopulmonary syndrome in a patient with calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia syndrome? 61
31868804 2020
39
A high-dose rapamycin treatment alleviates hepatopulmonary syndrome in cirrhotic rats. 61
31567652 2020
40
Hospital-Acquired Conditions after Liver Transplantation. 61
32077412 2020
41
Impact of liver damage on blood-borne variables and pulmonary hemodynamic responses to hypoxia and hyperoxia in anesthetized rats. 61
31931715 2020
42
Kidney and lung tissue modifications after BDL-induced liver injury in mice are associated with increased expression of IGFBPrP1 and activation of the NF-κB inflammation pathway. 61
32211099 2020
43
Epigenome-wide base-resolution profiling of DNA methylation in chorionic villi of fetuses with Down syndrome by methyl-capture sequencing. 61
31801612 2019
44
Value of Contrast Transesophageal Echocardiography in the Detection of Intrapulmonary Vascular Dilatations in Hepatosplenic Schistosomiasis. 61
31596323 2019
45
Pulmonary Complications of Portal Hypertension. 61
31563218 2019
46
Pulmonary Hypertension Post Liver-Kidney Transplant. 61
31675782 2019
47
Hepatocyte-derived exosomal MiR-194 activates PMVECs and promotes angiogenesis in hepatopulmonary syndrome. 61
31700002 2019
48
High-Dose Oral Garlic is Associated with a Decrease in Intrapulmonary Shunting in Severe Hepatopulmonary Syndrome. 61
31701077 2019
49
Does Biliodigestive Anastomosis Have Any Effect on the Reversal of Hepatopulmonary Syndrome in a Biliary Cirrhosis Experimental Model? 61
31076984 2019
50
Platypnoea and orthodeoxia in the hepatopulmonary syndrome. 61
31776152 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 35)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.75 VEGFA TNF NOS2 GDF2 COL18A1 CCL2
2
Show member pathways
13.51 TNF NOS3 NOS2 GDF2 EDNRB EDNRA
3
Show member pathways
13.34 TNF NOS3 GDF2 CCL2 BMPR2 AKT1
4
Show member pathways
13.3 VWF VEGFA TNF NOS2 HMOX1 CCL2
5 12.73 VEGFA NOS2 HMOX1 EDNRB EDNRA EDN1
6
Show member pathways
12.58 VEGFA NOS3 NOS2 EDNRB EDN1 AKT1
7
Show member pathways
12.52 TNF NOS3 NOS2 CCL2 AKT1
8
Show member pathways
12.31 VEGFA NOS3 NOS2 CCL2 AKT1
9
Show member pathways
12.22 NOS3 NOS2 EDNRB EDNRA
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 11.8 VEGFA TNF CCL2
15
Show member pathways
11.79 VEGFA NOS3 AKT1
16 11.77 VEGFA NOS3 NOS2 HMOX1 EDN1 AKT1
17 11.72 VWF NOS3 HMOX1 ENG CCL2
18 11.63 NOS3 NOS2 AKT1
19 11.63 VEGFA TNF NOS3 EDN1 CCL2 AKT1
20
Show member pathways
11.61 VEGFA TNF NOS2
21 11.6 TNF HMOX1 EDN1 CCL2
22 11.53 VEGFA NOS3 EDN1
23 11.5 EDNRB EDNRA EDN1 AKT1
24 11.49 VEGFA NOS2 AKT1
25 11.46 TNF NOS2 CCL2
26 11.37 VEGFA TNF NOS3 HMOX1 EDN1 CCL2
27 11.33 VEGFA NOS2 EDN1
28 11.28 NOS3 HMOX1 CCL2
29 11.25 VEGFA NOS2 HMOX1 ENG EDN1 AKT1
30 11.24 NOS3 EDNRB EDNRA EDN1
31 11.22 EDNRB EDNRA EDN1
32
Show member pathways
11.16 GDF2 BMPR2 ACVRL1
33 11.09 VEGFA NOS3 AKT1
34 10.78 NOS3 NOS2
35 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 cell GO:0005623 9.56 NOS3 NOS2 HMOX1 GDF2 EDNRB EDNRA
2 caveola GO:0005901 9.5 NOS3 HMOX1 BMPR2
3 platelet alpha granule lumen GO:0031093 9.43 VWF VEGFA ALB
4 extracellular space GO:0005615 9.32 VEGFA TNF HMOX1 GDF2 ENG EDN1
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 69)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 10.21 VEGFA TNF GDF2 ENG EDN1 BMPR2
2 positive regulation of cell proliferation GO:0008284 10.14 VEGFA EDNRB EDN1 COL18A1 AKT1
3 positive regulation of gene expression GO:0010628 10.04 VEGFA TNF NR0B2 NOS3 GDF2 ENG
4 heart development GO:0007507 10.03 ENG EDNRA EDN1 ACVRL1
5 in utero embryonic development GO:0001701 10 VEGFA NOS3 EDNRA EDN1 ACVRL1
6 cytokine-mediated signaling pathway GO:0019221 10 VEGFA TNF NOS2 HMOX1 CCL2 AKT1
7 cellular response to lipopolysaccharide GO:0071222 9.98 TNF NOS2 EDNRB CCL2
8 response to lipopolysaccharide GO:0032496 9.97 NOS3 NOS2 EDNRB EDN1
9 positive regulation of protein phosphorylation GO:0001934 9.97 VEGFA TNF ENG EDNRB AKT1
10 cellular response to hypoxia GO:0071456 9.93 VEGFA HMOX1 EDN1 AKT1
11 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.92 VEGFA TNF AKT1
12 negative regulation of cysteine-type endopeptidase activity involved in apoptotic process GO:0043154 9.9 VEGFA TNF AKT1
13 BMP signaling pathway GO:0030509 9.9 GDF2 ENG BMPR2 ACVRL1
14 cellular response to growth factor stimulus GO:0071363 9.89 BMPR2 AKT1 ACVRL1
15 cellular response to organic cyclic compound GO:0071407 9.89 TNF CCL2 AKT1
16 vasculogenesis GO:0001570 9.88 VEGFA GDF2 ENG
17 positive regulation of MAP kinase activity GO:0043406 9.88 VEGFA TNF EDN1
18 response to hypoxia GO:0001666 9.88 VEGFA NOS2 HMOX1 EDNRA EDN1 ACVRL1
19 positive regulation of endothelial cell migration GO:0010595 9.87 VEGFA EDN1 BMPR2
20 response to hormone GO:0009725 9.87 NOS3 NOS2 AKT1
21 sensory perception of pain GO:0019233 9.85 EDNRB EDN1 CCL2
22 positive regulation of blood vessel endothelial cell migration GO:0043536 9.85 VEGFA NOS3 AKT1
23 negative regulation of blood vessel endothelial cell migration GO:0043537 9.84 TNF GDF2 ACVRL1
24 positive regulation of nitric oxide biosynthetic process GO:0045429 9.84 TNF EDN1 AKT1
25 positive regulation of smooth muscle cell proliferation GO:0048661 9.84 TNF HMOX1 EDN1 AKT1
26 negative regulation of endothelial cell proliferation GO:0001937 9.83 TNF GDF2 ACVRL1
27 protein kinase B signaling GO:0043491 9.82 TNF CCL2 AKT1
28 cellular response to BMP stimulus GO:0071773 9.82 GDF2 BMPR2 ACVRL1
29 positive regulation of pathway-restricted SMAD protein phosphorylation GO:0010862 9.81 GDF2 ENG BMPR2 ACVRL1
30 blood vessel remodeling GO:0001974 9.8 NOS3 BMPR2 ACVRL1
31 blood vessel morphogenesis GO:0048514 9.8 GDF2 EDN1 ACVRL1
32 positive regulation of angiogenesis GO:0045766 9.8 VEGFA NOS3 HMOX1 GDF2 ENG ACVRL1
33 activin receptor signaling pathway GO:0032924 9.77 GDF2 BMPR2 ACVRL1
34 positive regulation of endothelial cell proliferation GO:0001938 9.77 VEGFA GDF2 BMPR2 AKT1 ACVRL1
35 endocardial cushion morphogenesis GO:0003203 9.76 NOS3 ENG ACVRL1
36 positive regulation of BMP signaling pathway GO:0030513 9.76 GDF2 ENG BMPR2 ACVRL1
37 negative regulation of gene expression GO:0010629 9.76 VEGFA TNF NR0B2 NOS2 ENG EDN1
38 vasoconstriction GO:0042310 9.75 EDNRB EDNRA EDN1
39 positive regulation of chemokine biosynthetic process GO:0045080 9.74 TNF HMOX1
40 lymphangiogenesis GO:0001946 9.74 BMPR2 ACVRL1
41 response to transforming growth factor beta GO:0071559 9.74 ENG EDN1
42 cofactor metabolic process GO:0051186 9.73 NOS3 AKT1
43 positive regulation of guanylate cyclase activity GO:0031284 9.73 NOS3 NOS2
44 maternal placenta development GO:0001893 9.73 BMPR2 AKT1
45 artery smooth muscle contraction GO:0014824 9.73 EDNRA EDN1
46 negative regulation of DNA biosynthetic process GO:2000279 9.73 GDF2 BMPR2 ACVRL1
47 lipopolysaccharide-mediated signaling pathway GO:0031663 9.73 TNF NOS3 CCL2 AKT1
48 artery development GO:0060840 9.72 BMPR2 ACVRL1
49 response to fluid shear stress GO:0034405 9.72 NOS3 AKT1
50 regulation of glucose transmembrane transport GO:0010827 9.72 EDNRA EDN1

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

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 10.02 VWF VEGFA TNF HMOX1 ENG ALB
2 protein binding GO:0005515 9.91 VWF VEGFA TNF NR0B2 NOS3 NOS2
3 protein homodimerization activity GO:0042803 9.88 VEGFA NR0B2 NOS2 HMOX1 ENG AKT1
4 cytokine activity GO:0005125 9.55 VEGFA TNF GDF2 EDN1 CCL2
5 BMP binding GO:0036122 9.52 ENG BMPR2
6 transmembrane receptor protein serine/threonine kinase activity GO:0004675 9.51 BMPR2 ACVRL1
7 arginine binding GO:0034618 9.49 NOS3 NOS2
8 BMP receptor activity GO:0098821 9.46 BMPR2 ACVRL1
9 NADPH-hemoprotein reductase activity GO:0003958 9.43 NOS3 NOS2
10 tetrahydrobiopterin binding GO:0034617 9.37 NOS3 NOS2
11 nitric-oxide synthase activity GO:0004517 9.32 NOS3 NOS2
12 endothelin receptor activity GO:0004962 9.26 EDNRB EDNRA
13 transforming growth factor beta-activated receptor activity GO:0005024 8.8 ENG BMPR2 ACVRL1

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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