MCID: HPT009
MIFTS: 52

Hepatopulmonary Syndrome

Categories: Endocrine diseases, Gastrointestinal diseases, Rare diseases

Aliases & Classifications for Hepatopulmonary Syndrome

MalaCards integrated aliases for Hepatopulmonary Syndrome:

Name: Hepatopulmonary Syndrome 12 53 55 44 15 73

Classifications:



External Ids:

Disease Ontology 12 DOID:900
ICD10 33 K76.81
ICD9CM 35 573.5
MeSH 44 D020065
UMLS 73 C0600452

Summaries for Hepatopulmonary Syndrome

NIH Rare Diseases : 53 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 μm whereas when in HPS rises to between 15 and 500 μm. 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 visceral leishmaniasis and ischemia, 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 Cytokine Signaling in Immune system. The drugs Dobutamine and Norepinephrine 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 : 76 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 143)
# Related Disease Score Top Affiliating Genes
1 visceral leishmaniasis 29.9 ALB NOS2 TNF
2 ischemia 29.5 EDN1 HMOX1 NOS2 NOS3 VEGFA
3 telangiectasis 29.5 ACVRL1 BMPR2 VEGFA
4 arteriovenous malformation 29.5 ACVRL1 BMPR2 VEGFA
5 portal hypertension 29.5 ALB BMPR2 EDN1 NOS3 TNF VEGFA
6 pulmonary hypertension 29.3 ACVRL1 BMPR2 EDN1 EDNRB NOS2 NOS3
7 heart disease 28.7 ALB BMPR2 EDN1 NOS3 TNF VEGFA
8 liver disease 10.5
9 liver cirrhosis 10.4
10 hepatitis 10.3
11 platelet membrane fluidity 10.2 NOS2 NOS3
12 biliary atresia 10.2
13 perinephritis 10.2 EDN1 EDNRB
14 severe nonproliferative diabetic retinopathy 10.2 ALB VEGFA
15 hemifacial spasm 10.2 NOS3 VEGFA
16 patellar tendinitis 10.2 NOS3 VEGFA
17 budd-chiari syndrome 10.2
18 coronary artery vasospasm 10.2 EDN1 NOS3
19 chronic graft versus host disease 10.1 ALB TNF
20 acroosteolysis 10.1 ALB VEGFA
21 idiopathic achalasia 10.1 NOS2 TNF
22 acute chest syndrome 10.1 EDN1 HMOX1 NOS3
23 renovascular hypertension 10.1 EDN1 HMOX1 NOS3
24 chronic myocardial ischemia 10.1 NOS3 VEGFA
25 impotence 10.1 EDN1 NOS2 NOS3
26 cerebral artery occlusion 10.1 NOS2 TNF
27 orthostatic intolerance 10.1 EDN1 NOS2 NOS3
28 jejunoileitis 10.1 ALB NOS3 TNF
29 eclampsia 10.1 ALB EDN1 TNF
30 critical limb ischemia 10.1 TNF VEGFA
31 dystrophinopathies 10.1 NOS2 TNF
32 kashin-beck disease 10.1 TNF VEGFA
33 arthus reaction 10.1 HMOX1 NOS2 TNF
34 acute poststreptococcal glomerulonephritis 10.1 EDN1 EDNRB
35 brachydactyly, type e2 10.1 EDN1 NOS3
36 background diabetic retinopathy 10.1 ALB VEGFA
37 obstructive jaundice 10.1 ALB EDN1 TNF
38 proteasome-associated autoinflammatory syndrome 1 10.1 ALB NOS2 TNF
39 chronic mountain sickness 10.1 EDN1 NOS3 VEGFA
40 microvascular complications of diabetes 5 10.1 ALB NOS3 VEGFA
41 chronic thromboembolic pulmonary hypertension 10.1 BMPR2 EDN1
42 sleep apnea 10.1 EDN1 NOS3 TNF
43 brain edema 10.1 ALB HMOX1 VEGFA
44 acute mountain sickness 10.1 EDN1 NOS3 VEGFA
45 hypopituitarism 10.1
46 congenital hepatic fibrosis 10.1
47 kidney disease 10.1 ALB EDN1 HMOX1 NOS3
48 central nervous system disease 10.0 HMOX1 NOS2 TNF
49 eales disease 10.0 NOS2 TNF VEGFA
50 crohn's colitis 10.0 NOS2 TNF

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:

46 (show all 21)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.33 ACVRL1 AKT1 BMPR2 EDN1 EDNRB HMOX1
2 homeostasis/metabolism MP:0005376 10.32 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRB
3 cellular MP:0005384 10.28 AKT1 ALB BMPR2 EDNRB HMOX1 NOS2
4 growth/size/body region MP:0005378 10.28 ACVRL1 AKT1 BMPR2 EDN1 EDNRB HMOX1
5 digestive/alimentary MP:0005381 10.27 ACVRL1 ALB EDN1 EDNRB NOS2 NOS3
6 behavior/neurological MP:0005386 10.25 ACVRL1 AKT1 BMPR2 EDNRB HMOX1 NOS2
7 endocrine/exocrine gland MP:0005379 10.25 AKT1 ALB EDN1 EDNRB HMOX1 NOS2
8 mortality/aging MP:0010768 10.23 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRB
9 hematopoietic system MP:0005397 10.22 ACVRL1 AKT1 BMPR2 EDNRB HMOX1 NOS2
10 muscle MP:0005369 10.17 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRB
11 immune system MP:0005387 10.15 AKT1 BMPR2 EDNRB HMOX1 NOS2 NOS3
12 liver/biliary system MP:0005370 10.13 ACVRL1 AKT1 ALB HMOX1 NOS2 NOS3
13 embryo MP:0005380 10.1 ACVRL1 AKT1 BMPR2 EDN1 EDNRB TNF
14 nervous system MP:0003631 10.07 ACVRL1 AKT1 BMPR2 EDN1 EDNRB NOS2
15 craniofacial MP:0005382 10.05 ACVRL1 EDN1 EDNRB NOS2 TNF VEGFA
16 normal MP:0002873 9.92 ACVRL1 AKT1 ALB BMPR2 EDN1 NOS3
17 renal/urinary system MP:0005367 9.91 ACVRL1 ALB BMPR2 EDN1 EDNRB HMOX1
18 neoplasm MP:0002006 9.88 AKT1 ALB EDNRB NOS2 TNF VEGFA
19 reproductive system MP:0005389 9.7 AKT1 HMOX1 NOS2 NOS3 NR0B2 TNF
20 respiratory system MP:0005388 9.61 ACVRL1 AKT1 BMPR2 EDNRB HMOX1 NOS2
21 skeleton MP:0005390 9.23 AKT1 BMPR2 EDN1 EDNRB NOS2 NOS3

Drugs & Therapeutics for Hepatopulmonary Syndrome

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

(show top 50) (show all 53)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dobutamine Approved Phase 2 34368-04-2 36811
2
Norepinephrine Approved Phase 2 51-41-2 439260
3
Norfloxacin Approved Phase 2 70458-96-7 4539
4
Nicotinamide Approved, Investigational Phase 2 98-92-0 936
5
Iodine Approved, Investigational Phase 2 7553-56-2 807
6
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
7
Bosentan Approved, Investigational Phase 2 147536-97-8 104865
8
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
9
Niacin Approved, Investigational, Nutraceutical Phase 2 59-67-6 938
10 Liver Extracts Phase 2,Phase 1
11 Anti-Bacterial Agents Phase 2,Not Applicable
12
Bilirubin Phase 2 635-65-4 5280352
13 Antibiotics, Antitubercular Phase 2,Not Applicable
14 Vasoconstrictor Agents Phase 2,Phase 1
15 Anti-Infective Agents Phase 2,Not Applicable
16 Cytochrome P-450 Enzyme Inhibitors Phase 2
17 Cytochrome P-450 CYP1A2 Inhibitors Phase 2
18 Topoisomerase Inhibitors Phase 2
19 Vitamin B9 Phase 2
20 Vitamins Phase 2
21 Trace Elements Phase 2
22 Vitamin B3 Phase 2
23 Protein Kinase Inhibitors Phase 2
24 Micronutrients Phase 2
25 cadexomer iodine Phase 2
26 Nicotinic Acids Phase 2
27 Vitamin B Complex Phase 2
28 Folate Phase 2
29 Antihypertensive Agents Phase 2
30 Endothelin Receptor Antagonists Phase 2
31
Pentoxifylline Approved, Investigational Phase 1,Not Applicable 6493-05-6 4740
32
Midodrine Approved Phase 1 133163-28-7, 42794-76-3 4195
33 Vasodilator Agents Phase 1,Not Applicable
34 Antioxidants Phase 1,Not Applicable
35 Radiation-Protective Agents Phase 1,Not Applicable
36 Phosphodiesterase Inhibitors Phase 1,Not Applicable
37 Free Radical Scavengers Phase 1,Not Applicable
38 Protective Agents Phase 1,Not Applicable
39 Platelet Aggregation Inhibitors Phase 1,Not Applicable
40 Sympathomimetics Phase 1
41 Adrenergic Agonists Phase 1
42 Autonomic Agents Phase 1
43 Adrenergic alpha-Agonists Phase 1
44 Neurotransmitter Agents Phase 1
45 Peripheral Nervous System Agents Phase 1
46 Adrenergic Agents Phase 1
47
Rifaximin Approved, Investigational Not Applicable 80621-81-4 6436173
48 rifamycin SV Not Applicable
49 Gastrointestinal Agents Not Applicable
50 Nucleic Acid Synthesis Inhibitors Not Applicable

Interventional clinical trials:

(show all 18)
# Name Status NCT ID Phase Drugs
1 Molecular Adsorbent Recirculating System (MARS®) in Hypoxic Hepatitis Unknown status NCT01690845 Phase 2
2 A Pilot Study of Norfloxacin for Hepatopulmonary Syndrome Completed NCT00362752 Phase 2 Norfloxacin;Placebo
3 Sorafenib for Hepatopulmonary Syndrome Terminated NCT02021929 Phase 2 Sorafenib;Placebo
4 Bosentan for Treatment of Hepatopulmonary Syndrome in Patients With Liver Cirrhosis Terminated NCT01518595 Phase 2 bosentan;Placebo
5 Midodrine in Hepatopumonary Syndrome Not yet recruiting NCT03600870 Phase 1 Midodrine
6 Pilot Study of Pentoxifylline for Hepatopulmonary Syndrome Terminated NCT00593658 Phase 1 pentoxifylline
7 Efficacy of Pentoxifylline and Rifaximin Combination in Pentoxifylline Refractory Clinical and Subclinical Hepatopulmonary Syndrome Unknown status NCT01676597 Not Applicable pentoxifylline and rifaximin;Pentoxifylline and placebo
8 Echocardiographic Assessment of Intrapulmonary Vasodilatation: Agitated Saline Versus Polygeline Unknown status NCT00343122
9 Efficacy of Rifaximin in Treatment of Cirrhotic Gastroesophageal Variceal Bleeding Unknown status NCT02964195 Not Applicable Rifaximin
10 Effect of Transjugular Intrahepatic Portosystemic Shunt on Oxygenation in Cirrhotic Patients With Hepatopulmonary Syndrome Completed NCT02148536
11 Rifaximin in Patients With Gastroesophageal Variceal Bleeding Completed NCT02991612 Not Applicable Rifaximin
12 Prevalence and Predictive Factors of Portal Vein Thrombosis in Patients With Cirrhosis Completed NCT02069457
13 Portal Vein Thrombosis Relevance on Liver Cirrhosis: Italian Venous Thrombotic Events Registry Completed NCT01470547
14 Investigation of the Prevalence of Hepatopulmonary Syndrome in Cirrhosis Patients Caused by Hepatitis B in Western China Recruiting NCT03435406
15 MRI Biomarkers in as Predictor of Clinical Endpoints in Pediatric Autoimmune Liver Disease Recruiting NCT03178630
16 Hepatopulmonary Syndrome and Postoperative Complications After Liver Transplantation : A Case-control Study Active, not recruiting NCT03092401
17 Skin Sympathetic Nerve Activity and Sympathetic Tone Enrolling by invitation NCT03243448
18 The Effect of Rifaximin on Portal Vein Thrombosis Not yet recruiting NCT03631147 Not Applicable Rifaximin

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:

41
Liver, Lung, Brain, Skin, Heart, Endothelial, Kidney

Publications for Hepatopulmonary Syndrome

Articles related to Hepatopulmonary Syndrome:

(show top 50) (show all 551)
# Title Authors Year
1
Prevalence and characteristics of hepatopulmonary syndrome in children with cirrhosis in southern Brazil. ( 30045096 )
2019
2
Hepatopulmonary syndrome-attributed extreme hypoxemia and polycythemia revealing liver cirrhosis. ( 30269997 )
2019
3
Placental Growth Factor: New Treatment Target Bringing Hope in Hepatopulmonary Syndrome. ( 29356037 )
2018
4
Review article: Update on current and emergent data on hepatopulmonary syndrome. ( 29599604 )
2018
5
Corrigendum to miR144-3p inhibits PMVECs excessive proliferation in angiogenesis of hepatopulmonary syndrome via Tie2 [Exp. Cell Res. 365 (2018) 24-32]. ( 29656831 )
2018
6
Hepatopulmonary Syndrome and Diffuse Interstitial Lung Disease: An Unusual Combination. ( 29588084 )
2018
7
Hepatopulmonary syndrome. ( 29483331 )
2018
8
Potential clinical targets in hepatopulmonary syndrome: lessons from experimental models. ( 29729196 )
2018
9
Hormone-replacement Therapy for Hepatopulmonary Syndrome and NASH Associated with Hypopituitarism. ( 29910217 )
2018
10
Hepatopulmonary Syndrome and Portopulmonary Hypertension in Children: Recent Advances in Diagnosis and Management. ( 29514741 )
2018
11
Impact of Bacterial Translocation on Hepatopulmonary Syndrome: A Prospective Observational Study. ( 29192374 )
2018
12
Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome. ( 29716638 )
2018
13
miR144-3p inhibits PMVECs excessive proliferation in angiogenesis of hepatopulmonary syndrome via Tie2. ( 29453975 )
2018
14
Hepatopulmonary syndrome. ( 29960651 )
2018
15
Epstein-Barr virus hepatitis can cause transient hepatopulmonary syndrome. ( 29977758 )
2018
16
Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report. ( 29609450 )
2018
17
Extracorporeal Membrane Oxygenation in a Pediatric Patient with Hepatopulmonary Syndrome and Interrupted Inferior Vena Cava After Living Related Liver Donation. ( 29613889 )
2018
18
Pulmonary transit time derived from pulmonary angiography for the diagnosis of hepatopulmonary syndrome. ( 29573542 )
2018
19
Changes in arterial oxygenation after portal decompression in Budd-Chiari syndrome patients with hepatopulmonary syndrome. ( 30506220 )
2018
20
Hepatopulmonary Syndrome in Children: A 20-Year Review of Presenting Symptoms, Clinical Progression, and Transplant Outcome. ( 30066494 )
2018
21
Pulse Oximetry is Insensitive for Detection of Hepatopulmonary Syndrome in Patients evaluated for Liver Transplantation. ( 30070715 )
2018
22
A case report of hepatopulmonary syndrome in hereditary hemorrhagic telangiectasia (HHT): Not all right-to-left shunting in HHT is due to pulmonary arteriovenous malformations. ( 30095617 )
2018
23
Multifocal stroke with proliferation of small cerebral arteries in hepatopulmonary syndrome. ( 30105173 )
2018
24
Evidence for an Association Between Intrahepatic Vascular Changes and the Development of Hepatopulmonary Syndrome. ( 30292761 )
2018
25
Correlations of MMP-2 and MMP-9 gene polymorphisms with the risk of hepatopulmonary syndrome in cirrhotic patients: A case-control study. ( 30392570 )
2018
26
Cardiac involvement in patients with end-stage liver disease, beyond the hepatopulmonary syndrome. ( 30409693 )
2018
27
Outcome of liver transplantation for hepatopulmonary syndrome: a Eurotransplant experience. ( 30487203 )
2018
28
Late complications of biliary atresia: hepatopulmonary syndrome and portopulmonary hypertension. ( 28983709 )
2017
29
Selective cyclooxygenase inhibition by SC-560 improves hepatopulmonary syndrome in cirrhotic rats. ( 28632747 )
2017
30
Pioglitazone decrease intrapulmonary shunt in biliary cirrhotic rats with hepatopulmonary syndrome. ( 28954708 )
2017
31
Ventilatory support and hospital stay after liver transplant in cirrhotic patients with hepatopulmonary syndrome. ( 29091154 )
2017
32
Differential diagnosis of hepatopulmonary syndrome (HPS): Portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT). ( 28759737 )
2017
33
Dual organ beneficial effects of metformin in cirrhotic rats with hepatopulmonary syndrome. ( 28705726 )
2017
34
Hepatopulmonary syndrome with large pulmonary arteriovenous malformations: CT findings with emphasis on its association with a mosaic pattern of the lung parenchyma. ( 28480722 )
2017
35
Live donor liver transplantation for a child presented with severe hepatopulmonary syndrome and nodular liver lesions due to Abernethy malformation. ( 28078819 )
2017
36
Annexin A2-modulated proliferation of pulmonary arterial smooth muscle cells depends on caveolae and caveolin-1 in hepatopulmonary syndrome. ( 28729092 )
2017
37
Unexplained cyanosis caused by hepatopulmonary syndrome in a girl with APECED syndrome. ( 28222032 )
2017
38
Dyspnoea, cyanosis and digital clubbing in a 28-year-old patient as a result of hepatopulmonary syndrome. ( 29288484 )
2017
39
Isolated Intrapulmonary Vascular Dilatations and the Risk of Developing Hepatopulmonary Syndrome in Liver Transplant Candidates. ( 28611257 )
2017
40
Successful venoarterial extracorporeal membrane oxygenation for prolonged hepatopulmonary syndrome following pediatric liver transplantation: A case report and review of the literature. ( 28833992 )
2017
41
Extracorporeal membrane oxygenation for post-transplant hypoxaemia following very severe hepatopulmonary syndrome. ( 29092970 )
2017
42
Clinical efficacy of transjugular intrahepatic portosystemic shunt in the treatment of hepatopulmonary syndrome. ( 29245324 )
2017
43
Hepatopulmonary syndrome is associated with the presence of hepatocellular carcinoma in patients with decompensated cirrhosis. ( 28243044 )
2017
44
Screening for Hepatopulmonary Syndrome in Cirrhotic Patients Using Technetium 99m-macroaggregated Albumin Perfusion Lung Scan (Tc-MAA): Diagnostic Approach and Clinical Correlations. ( 28961571 )
2017
45
Do Late Bubbles Correspond to Early Hepatopulmonary Syndrome? ( 28612753 )
2017
46
CXCR2 is involved in pulmonary intravascular macrophage accumulation and angiogenesis in a rat model of hepatopulmonary syndrome. ( 27879294 )
2017
47
Hepatopulmonary syndrome: which blood gas analysis criteria and position should we use for diagnosis? ( 28972388 )
2017
48
Severe Hepatopulmonary Syndrome in a Child with Caroli Syndrome. ( 28884036 )
2017
49
The utility of the macro-aggregated albumin lung perfusion scan in the diagnosis and prognosis of hepatopulmonary syndrome in cirrhotic patients candidates for liver transplantation. ( 28301945 )
2017
50
Persistent Unexplained Dyspnea: A Case of Hepatopulmonary Syndrome. ( 28948051 )
2017

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 39)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.48 ACVRL1 AKT1 BMPR2 EDN1 EDNRB NOS2
2
Show member pathways
13.21 AKT1 HMOX1 NOS2 TNF VEGFA VWF
3
Show member pathways
13.17 AKT1 ALB NOS2 NOS3 VEGFA VWF
4
Show member pathways
12.86 AKT1 NOS3 TNF VEGFA VWF
5
Show member pathways
12.8 AKT1 EDN1 EDNRB NOS3 TNF
6
Show member pathways
12.78 AKT1 EDN1 EDNRB NOS2 NOS3 VEGFA
7 12.68 AKT1 EDNRB HMOX1 NOS2 VEGFA
8
Show member pathways
12.47 AKT1 NOS2 NOS3 TNF
9
Show member pathways
12.47 AKT1 NOS3 TNF VEGFA
10
Show member pathways
12.28 AKT1 NOS2 NOS3 VEGFA
11
Show member pathways
12.05 AKT1 EDN1 TNF
12 12.04 AKT1 BMPR2 VEGFA
13
Show member pathways
12.04 EDN1 HMOX1 NOS2 TNF
14 12 AKT1 NOS2 TNF VEGFA
15 11.98 AKT1 NOS2 NOS3
16 11.93 AKT1 NOS3 VWF
17 11.91 AKT1 NOS3 TNF
18 11.87 AKT1 EDN1 TNF
19
Show member pathways
11.86 AKT1 NOS3 TNF
20 11.78 AKT1 HMOX1 NOS2 TNF VEGFA
21
Show member pathways
11.76 AKT1 NOS3 VEGFA
22 11.73 HMOX1 NOS3 VWF
23 11.65 AKT1 EDN1 NOS3 TNF VEGFA
24 11.58 AKT1 NOS2 NOS3
25 11.55 EDN1 HMOX1 TNF
26
Show member pathways
11.54 NOS2 TNF VEGFA
27 11.46 EDN1 NOS3 VEGFA
28 11.43 AKT1 EDN1 HMOX1 NOS2 NOS3 VEGFA
29 11.42 AKT1 EDN1 EDNRB
30 11.39 AKT1 NOS2 VEGFA
31 11.37 AKT1 EDN1 HMOX1 NOS2 VEGFA
32 11.31 AKT1 BMPR2 EDN1 HMOX1 NOS3 TNF
33 11.2 EDN1 NOS2 VEGFA
34 11.11 EDN1 EDNRB NOS3
35 11.06 NOS2 NOS3
36 10.95 HMOX1 TNF
37 10.92 AKT1 NOS3 VEGFA
38 10.76 NOS2 NOS3
39 10.5 NOS2 NOS3

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 caveola GO:0005901 9.33 BMPR2 HMOX1 NOS3
2 platelet alpha granule lumen GO:0031093 9.13 ALB VEGFA VWF
3 Weibel-Palade body GO:0033093 8.62 EDN1 VWF

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

(show top 50) (show all 62)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.95 AKT1 NOS3 NR0B2 TNF VEGFA
2 angiogenesis GO:0001525 9.94 ACVRL1 HMOX1 NOS3 VEGFA
3 in utero embryonic development GO:0001701 9.9 ACVRL1 EDN1 NOS3 VEGFA
4 platelet degranulation GO:0002576 9.88 ALB VEGFA VWF
5 positive regulation of DNA-binding transcription factor activity GO:0051091 9.87 AKT1 EDN1 TNF
6 positive regulation of protein phosphorylation GO:0001934 9.86 AKT1 EDNRB TNF VEGFA
7 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.85 AKT1 TNF VEGFA
8 response to lipopolysaccharide GO:0032496 9.83 EDN1 EDNRB NOS2 NOS3
9 positive regulation of angiogenesis GO:0045766 9.81 ACVRL1 HMOX1 NOS3 VEGFA
10 cytokine-mediated signaling pathway GO:0019221 9.8 AKT1 HMOX1 NOS2 TNF VEGFA
11 positive regulation of MAP kinase activity GO:0043406 9.79 EDN1 TNF VEGFA
12 response to hormone GO:0009725 9.78 AKT1 NOS2 NOS3
13 positive regulation of endothelial cell migration GO:0010595 9.77 BMPR2 EDN1 VEGFA
14 positive regulation of blood vessel endothelial cell migration GO:0043536 9.74 AKT1 NOS3 VEGFA
15 positive regulation of blood vessel endothelial cell proliferation involved in sprouting angiogenesis GO:1903589 9.73 HMOX1 VEGFA
16 protein import into nucleus, translocation GO:0000060 9.73 AKT1 TNF
17 cell migration involved in sprouting angiogenesis GO:0002042 9.73 AKT1 VEGFA
18 regulation of nitric-oxide synthase activity GO:0050999 9.73 AKT1 NOS3
19 cellular response to hypoxia GO:0071456 9.73 AKT1 EDN1 HMOX1 VEGFA
20 regulation of pH GO:0006885 9.72 EDN1 EDNRB
21 vasodilation GO:0042311 9.72 EDNRB NOS3
22 transmembrane receptor protein serine/threonine kinase signaling pathway GO:0007178 9.72 ACVRL1 BMPR2
23 nitric oxide mediated signal transduction GO:0007263 9.72 NOS2 NOS3
24 regulation of blood vessel size GO:0050880 9.72 EDN1 NOS3
25 activin receptor signaling pathway GO:0032924 9.71 ACVRL1 BMPR2
26 endocardial cushion morphogenesis GO:0003203 9.71 ACVRL1 NOS3
27 endothelial cell proliferation GO:0001935 9.71 BMPR2 HMOX1
28 vasoconstriction GO:0042310 9.71 EDN1 EDNRB
29 positive regulation of nitric oxide biosynthetic process GO:0045429 9.71 AKT1 EDN1 TNF
30 positive regulation of protein localization to cell surface GO:2000010 9.7 AKT1 TNF
31 negative regulation of macroautophagy GO:0016242 9.7 AKT1 HMOX1
32 positive regulation of renal sodium excretion GO:0035815 9.7 EDN1 EDNRB
33 positive regulation of urine volume GO:0035810 9.69 EDN1 EDNRB
34 negative regulation of DNA biosynthetic process GO:2000279 9.69 ACVRL1 BMPR2
35 lymphangiogenesis GO:0001946 9.68 ACVRL1 BMPR2
36 maternal placenta development GO:0001893 9.68 AKT1 BMPR2
37 cofactor metabolic process GO:0051186 9.68 AKT1 NOS3
38 positive regulation of guanylate cyclase activity GO:0031284 9.68 NOS2 NOS3
39 response to fluid shear stress GO:0034405 9.67 AKT1 NOS3
40 lipopolysaccharide-mediated signaling pathway GO:0031663 9.67 AKT1 NOS3 TNF
41 positive regulation of endothelial cell proliferation GO:0001938 9.67 ACVRL1 AKT1 BMPR2 VEGFA
42 artery development GO:0060840 9.66 ACVRL1 BMPR2
43 endothelial cell apoptotic process GO:0072577 9.66 BMPR2 TNF
44 positive regulation of chemokine biosynthetic process GO:0045080 9.65 HMOX1 TNF
45 retina vasculature development in camera-type eye GO:0061298 9.65 ACVRL1 BMPR2
46 arginine catabolic process GO:0006527 9.65 NOS2 NOS3
47 blood vessel remodeling GO:0001974 9.65 ACVRL1 BMPR2 NOS3
48 positive regulation of axon extension involved in axon guidance GO:0048842 9.64 BMPR2 VEGFA
49 epithelial fluid transport GO:0042045 9.63 EDN1 EDNRB
50 lymphatic endothelial cell differentiation GO:0060836 9.63 ACVRL1 BMPR2

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 heme binding GO:0020037 9.67 HMOX1 NOS2 NOS3
2 FMN binding GO:0010181 9.48 NOS2 NOS3
3 transforming growth factor beta-activated receptor activity GO:0005024 9.46 ACVRL1 BMPR2
4 transmembrane receptor protein serine/threonine kinase activity GO:0004675 9.43 ACVRL1 BMPR2
5 protein homodimerization activity GO:0042803 9.43 AKT1 HMOX1 NOS2 NR0B2 VEGFA VWF
6 arginine binding GO:0034618 9.4 NOS2 NOS3
7 BMP receptor activity GO:0098821 9.37 ACVRL1 BMPR2
8 NADPH-hemoprotein reductase activity GO:0003958 9.32 NOS2 NOS3
9 tetrahydrobiopterin binding GO:0034617 9.16 NOS2 NOS3
10 nitric-oxide synthase activity GO:0004517 8.62 NOS2 NOS3
11 protein binding GO:0005515 10.07 ACVRL1 AKT1 ALB BMPR2 EDN1 EDNRB

Sources for Hepatopulmonary Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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