MCID: HPT009
MIFTS: 53

Hepatopulmonary Syndrome

Categories: Rare diseases, Endocrine diseases, Gastrointestinal diseases, Liver 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
SNOMED-CT 68 371067004
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 Carvedilol and Vasodilator Agents have been mentioned in the context of this disorder. Affiliated tissues include liver, lung and brain, and related phenotypes are endocrine/exocrine gland 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 131)
# Related Disease Score Top Affiliating Genes
1 visceral leishmaniasis 29.8 ALB NOS2 TNF
2 ischemia 28.0 CAV1 EDN1 HMOX1 NOS2 NOS3 VEGFA
3 portal hypertension 27.0 ALB EDN1 EDNRA NOS3 TNF VEGFA
4 pulmonary hypertension 26.1 CAV1 EDN1 EDNRA EDNRB NOS2 NOS3
5 platelet membrane fluidity 10.8 NOS2 NOS3
6 coronary artery vasospasm 10.6 EDN1 NOS3
7 severe nonproliferative diabetic retinopathy 10.6 ALB VEGFA
8 hemifacial spasm 10.6 NOS3 VEGFA
9 acroosteolysis 10.5 ALB VEGFA
10 burns 10.5 ALB TNF
11 adrenal gland pheochromocytoma 10.5 AKT1 NOS3
12 hepatic vascular disease 10.5 ALB EDN1 NOS3
13 chronic myocardial ischemia 10.5 NOS3 VEGFA
14 impotence 10.4 EDN1 NOS2 NOS3
15 renovascular hypertension 10.4 EDN1 HMOX1 NOS3
16 orthostatic intolerance 10.4 EDN1 NOS2 NOS3
17 idiopathic achalasia 10.4 NOS2 TNF
18 background diabetic retinopathy 10.4 ALB VEGFA
19 cerebral artery occlusion 10.4 NOS3 TNF
20 liver disease 10.3
21 microvascular complications of diabetes 5 10.3 ALB NOS3 VEGFA
22 chronic mountain sickness 10.3 EDN1 NOS3 VEGFA
23 brain edema 10.3 ALB HMOX1 VEGFA
24 vasculogenic impotence 10.3 CAV1 NOS3
25 acute mountain sickness 10.3 EDN1 NOS3 VEGFA
26 hepatitis 10.3
27 dystrophinopathies 10.2 NOS2 TNF
28 obstructive jaundice 10.2 ALB EDN1 TNF
29 raynaud disease 10.2 EDN1 VWF
30 liver cirrhosis 10.2
31 eclampsia 10.2 ALB EDN1 TNF
32 urinary system disease 10.2 ALB EDN1 TNF
33 intestinal disease 10.2 ALB EDNRB TNF
34 infiltrating angiolipoma 10.2 VEGFA VWF
35 acute poststreptococcal glomerulonephritis 10.1 EDN1 EDNRB
36 gastrointestinal system disease 10.1 ALB EDNRB TNF
37 koro 10.1 ALB EDNRA
38 eales disease 10.1 NOS2 TNF VEGFA
39 endotheliitis 10.1
40 angina pectoris 10.1 EDN1 NOS3 VWF
41 biliary atresia 10.0
42 mandibulofacial dysostosis with alopecia 10.0 EDN1 EDNRA
43 bullous impetigo 10.0 EDNRA EDNRB
44 poems syndrome 10.0 TNF VEGFA
45 ritter's disease 10.0 EDNRA EDNRB
46 scarlet fever 10.0 EDNRA EDNRB
47 budd-chiari syndrome 10.0
48 eisenmenger syndrome 10.0 EDN1 VWF
49 intermittent claudication 9.9 ALB VEGFA VWF
50 angiodysplasia 9.9 VEGFA VWF

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 22)
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 10.34 ALB EDN1 AKT1 CAV1 VEGFA EDNRB
2 homeostasis/metabolism MP:0005376 10.34 ALB EDN1 AKT1 CAV1 VWF VEGFA
3 cardiovascular system MP:0005385 10.33 EDN1 AKT1 CAV1 VEGFA VWF HMOX1
4 cellular MP:0005384 10.32 ALB AKT1 CAV1 VEGFA VWF HMOX1
5 digestive/alimentary MP:0005381 10.31 ALB EDN1 CAV1 VWF VEGFA EDNRB
6 growth/size/body region MP:0005378 10.29 AKT1 CAV1 VEGFA TNF HMOX1 EDN1
7 behavior/neurological MP:0005386 10.25 AKT1 CAV1 VEGFA TNF EDNRA HMOX1
8 hematopoietic system MP:0005397 10.24 AKT1 CAV1 VEGFA TNF VWF EDNRA
9 mortality/aging MP:0010768 10.23 ALB EDN1 AKT1 CAV1 VEGFA VWF
10 immune system MP:0005387 10.21 AKT1 CAV1 VEGFA TNF VWF EDNRA
11 muscle MP:0005369 10.17 ALB EDN1 AKT1 CAV1 VEGFA EDNRB
12 liver/biliary system MP:0005370 10.13 ALB AKT1 CAV1 VEGFA HMOX1 TNF
13 adipose tissue MP:0005375 10.08 AKT1 CAV1 TNF HMOX1 NOS2 NOS3
14 nervous system MP:0003631 10.07 CAV1 AKT1 VEGFA TNF VWF EDNRA
15 craniofacial MP:0005382 10.05 VEGFA TNF EDNRA EDN1 EDNRB NOS2
16 integument MP:0010771 10.04 AKT1 CAV1 VEGFA TNF EDNRA EDNRB
17 neoplasm MP:0002006 9.95 ALB AKT1 CAV1 VEGFA TNF EDNRB
18 renal/urinary system MP:0005367 9.91 ALB EDN1 CAV1 VEGFA HMOX1 EDNRB
19 normal MP:0002873 9.87 AKT1 ALB VEGFA EDNRA EDN1 NR0B2
20 reproductive system MP:0005389 9.76 CAV1 AKT1 VEGFA TNF HMOX1 NR0B2
21 respiratory system MP:0005388 9.61 AKT1 CAV1 VEGFA HMOX1 EDNRB EDNRA
22 skeleton MP:0005390 9.28 AKT1 CAV1 VEGFA TNF EDN1 EDNRB

Drugs & Therapeutics for Hepatopulmonary Syndrome

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

(show top 50) (show all 51)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Carvedilol Approved, Investigational Phase 4 72956-09-3 2585
2 Vasodilator Agents Phase 4,Phase 1,Not Applicable
3 Adrenergic Agents Phase 4
4 Adrenergic alpha-1 Receptor Antagonists Phase 4
5 Adrenergic alpha-Antagonists Phase 4
6 Adrenergic Antagonists Phase 4
7 Adrenergic beta-Antagonists Phase 4
8 Neurotransmitter Agents Phase 4
9 Antihypertensive Agents Phase 4,Phase 2
10
Norfloxacin Approved Phase 2 70458-96-7 4539
11
Dobutamine Approved Phase 2 34368-04-2 36811
12
Norepinephrine Approved Phase 2 51-41-2 439260
13
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
14
Bosentan Approved, Investigational Phase 2 147536-97-8 104865
15
Iodine Approved, Investigational Phase 2 7553-56-2 807
16
Nicotinamide Approved, Investigational, Nutraceutical Phase 2 98-92-0 936
17
Niacin Approved, Investigational, Nutraceutical Phase 2 59-67-6 938
18
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
19 Anti-Bacterial Agents Phase 2,Not Applicable
20 Anti-Infective Agents Phase 2,Not Applicable
21 Liver Extracts Phase 2,Phase 1
22 Cytochrome P-450 CYP1A2 Inhibitors Phase 2
23 Cytochrome P-450 Enzyme Inhibitors Phase 2
24 Topoisomerase Inhibitors Phase 2
25
Bilirubin Phase 2 635-65-4 5280352
26 Vasoconstrictor Agents Phase 2
27 Antibiotics, Antitubercular Phase 2,Not Applicable
28 cadexomer iodine Phase 2
29 Micronutrients Phase 2
30 Nicotinic Acids Phase 2
31 Trace Elements Phase 2
32 Endothelin Receptor Antagonists Phase 2
33 Vitamin B Complex Phase 2
34 Vitamins Phase 2
35 Protein Kinase Inhibitors Phase 2
36 Folate Nutraceutical Phase 2
37 Vitamin B3 Nutraceutical Phase 2
38 Vitamin B9 Nutraceutical Phase 2
39
Pentoxifylline Approved, Investigational Phase 1,Not Applicable 6493-05-6 4740
40 Phosphodiesterase Inhibitors Phase 1,Not Applicable
41 Platelet Aggregation Inhibitors Phase 1,Not Applicable
42 Antioxidants Phase 1,Not Applicable
43 Protective Agents Phase 1,Not Applicable
44 Radiation-Protective Agents Phase 1,Not Applicable
45
Rifaximin Approved, Investigational Not Applicable 80621-81-4 6436173
46 rifamycin SV Not Applicable
47 Rifamycins Not Applicable
48 Gastrointestinal Agents Not Applicable
49 Nucleic Acid Synthesis Inhibitors Not Applicable
50 Polygeline

Interventional clinical trials:

(show all 17)
# Name Status NCT ID Phase Drugs
1 Early Precise Diagnosis and Intervention of CPT Based on a Noninvasive 3D-vHPS Recruiting NCT02925975 Phase 4 Carvedilol
2 A Pilot Study of Norfloxacin for Hepatopulmonary Syndrome Completed NCT00362752 Phase 2 Norfloxacin;Placebo
3 Molecular Adsorbent Recirculating System (MARS®) in Hypoxic Hepatitis Recruiting NCT01690845 Phase 2
4 Sorafenib for Hepatopulmonary Syndrome Terminated NCT02021929 Phase 2 Sorafenib;Placebo
5 Bosentan for Treatment of Hepatopulmonary Syndrome in Patients With Liver Cirrhosis Terminated NCT01518595 Phase 2 bosentan;Placebo
6 Pilot Study of Pentoxifylline for Hepatopulmonary Syndrome Terminated NCT00593658 Phase 1 pentoxifylline
7 Effect of Transjugular Intrahepatic Portosystemic Shunt on Oxygenation in Cirrhotic Patients With Hepatopulmonary Syndrome Unknown status NCT02148536
8 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
9 Echocardiographic Assessment of Intrapulmonary Vasodilatation: Agitated Saline Versus Polygeline Unknown status NCT00343122
10 Rifaximin in Patients With Gastroesophageal Variceal Bleeding Completed NCT02991612 Not Applicable Rifaximin
11 Prevalence and Predictive Factors of Portal Vein Thrombosis in Patients With Cirrhosis Completed NCT02069457
12 Portal Vein Thrombosis Relevance on Liver Cirrhosis: Italian Venous Thrombotic Events Registry Completed NCT01470547
13 Investigation of the Prevalence of Hepatopulmonary Syndrome in Cirrhosis Patients Caused by Hepatitis B in Western China Recruiting NCT03435406
14 Efficacy of Rifaximin in Treatment of Cirrhotic Gastroesophageal Variceal Bleeding Recruiting NCT02964195 Not Applicable Rifaximin
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

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, Smooth Muscle

Publications for Hepatopulmonary Syndrome

Articles related to Hepatopulmonary Syndrome:

(show top 50) (show all 517)
# Title Authors Year
1
Placental Growth Factor: New Treatment Target Bringing Hope in Hepatopulmonary Syndrome. ( 29356037 )
2018
2
Review article: Update on current and emergent data on hepatopulmonary syndrome. ( 29599604 )
2018
3
Corrigendum to miR144-3p inhibits PMVECs excessive proliferation in angiogenesis of hepatopulmonary syndrome via Tie2 [Exp. Cell Res. 365 (2018) 24-32]. ( 29656831 )
2018
4
Hepatopulmonary Syndrome and Diffuse Interstitial Lung Disease: An Unusual Combination. ( 29588084 )
2018
5
Hepatopulmonary syndrome. ( 29483331 )
2018
6
Potential clinical targets in hepatopulmonary syndrome: lessons from experimental models. ( 29729196 )
2018
7
Hormone-replacement Therapy for Hepatopulmonary Syndrome and NASH Associated with Hypopituitarism. ( 29910217 )
2018
8
Hepatopulmonary Syndrome and Portopulmonary Hypertension in Children: Recent Advances in Diagnosis and Management. ( 29514741 )
2018
9
Impact of Bacterial Translocation on Hepatopulmonary Syndrome: A Prospective Observational Study. ( 29192374 )
2018
10
Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome. ( 29716638 )
2018
11
miR144-3p inhibits PMVECs excessive proliferation in angiogenesis of hepatopulmonary syndrome via Tie2. ( 29453975 )
2018
12
Hepatopulmonary syndrome. ( 29960651 )
2018
13
Epstein-Barr virus hepatitis can cause transient hepatopulmonary syndrome. ( 29977758 )
2018
14
Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report. ( 29609450 )
2018
15
Extracorporeal Membrane Oxygenation in a Pediatric Patient with Hepatopulmonary Syndrome and Interrupted Inferior Vena Cava After Living Related Liver Donation. ( 29613889 )
2018
16
Pulmonary transit time derived from pulmonary angiography for the diagnosis of hepatopulmonary syndrome. ( 29573542 )
2018
17
Late complications of biliary atresia: hepatopulmonary syndrome and portopulmonary hypertension. ( 28983709 )
2017
18
Selective cyclooxygenase inhibition by SC-560 improves hepatopulmonary syndrome in cirrhotic rats. ( 28632747 )
2017
19
Pioglitazone decrease intrapulmonary shunt in biliary cirrhotic rats with hepatopulmonary syndrome. ( 28954708 )
2017
20
Ventilatory support and hospital stay after liver transplant in cirrhotic patients with hepatopulmonary syndrome. ( 29091154 )
2017
21
Differential diagnosis of hepatopulmonary syndrome (HPS): Portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT). ( 28759737 )
2017
22
Dual organ beneficial effects of metformin in cirrhotic rats with hepatopulmonary syndrome. ( 28705726 )
2017
23
Hepatopulmonary syndrome with large pulmonary arteriovenous malformations: CT findings with emphasis on its association with a mosaic pattern of the lung parenchyma. ( 28480722 )
2017
24
Live donor liver transplantation for a child presented with severe hepatopulmonary syndrome and nodular liver lesions due to Abernethy malformation. ( 28078819 )
2017
25
Annexin A2-modulated proliferation of pulmonary arterial smooth muscle cells depends on caveolae and caveolin-1 in hepatopulmonary syndrome. ( 28729092 )
2017
26
Unexplained cyanosis caused by hepatopulmonary syndrome in a girl with APECED syndrome. ( 28222032 )
2017
27
Dyspnoea, cyanosis and digital clubbing in a 28-year-old patient as a result of hepatopulmonary syndrome. ( 29288484 )
2017
28
Isolated Intrapulmonary Vascular Dilatations and the Risk of Developing Hepatopulmonary Syndrome in Liver Transplant Candidates. ( 28611257 )
2017
29
Successful venoarterial extracorporeal membrane oxygenation for prolonged hepatopulmonary syndrome following pediatric liver transplantation: A case report and review of the literature. ( 28833992 )
2017
30
Extracorporeal membrane oxygenation for post-transplant hypoxaemia following very severe hepatopulmonary syndrome. ( 29092970 )
2017
31
Clinical efficacy of transjugular intrahepatic portosystemic shunt in the treatment of hepatopulmonary syndrome. ( 29245324 )
2017
32
Hepatopulmonary syndrome is associated with the presence of hepatocellular carcinoma in patients with decompensated cirrhosis. ( 28243044 )
2017
33
Screening for Hepatopulmonary Syndrome in Cirrhotic Patients Using Technetium 99m-macroaggregated Albumin Perfusion Lung Scan (Tc-MAA): Diagnostic Approach and Clinical Correlations. ( 28961571 )
2017
34
Do Late Bubbles Correspond to Early Hepatopulmonary Syndrome? ( 28612753 )
2017
35
CXCR2 is involved in pulmonary intravascular macrophage accumulation and angiogenesis in a rat model of hepatopulmonary syndrome. ( 27879294 )
2017
36
Hepatopulmonary syndrome: which blood gas analysis criteria and position should we use for diagnosis? ( 28972388 )
2017
37
Severe Hepatopulmonary Syndrome in a Child with Caroli Syndrome. ( 28884036 )
2017
38
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
39
Persistent Unexplained Dyspnea: A Case of Hepatopulmonary Syndrome. ( 28948051 )
2017
40
Placental growth factor inhibition targets pulmonary angiogenesis and represents a novel therapy for hepatopulmonary syndrome in mice. ( 29023811 )
2017
41
The effects of pioglitazone in cirrhotic rats with hepatopulmonary syndrome. ( 28969989 )
2017
42
Onset ages of hepatopulmonary syndrome and pulmonary hypertension in patients with biliary atresia. ( 28871319 )
2017
43
Diammonium glycyrrhizinate alleviates hepatopulmonary syndrome via restoring superoxide dismutase 3 activity in rats. ( 28435094 )
2017
44
Cyclooxygenase-2 promotes pulmonary intravascular macrophage accumulation by exacerbating BMP signaling in rat experimental hepatopulmonary syndrome. ( 28642034 )
2017
45
Hepatopulmonary Syndrome and Portopulmonary Hypertension: Implications for Liver Transplantation. ( 29128026 )
2017
46
Development and Evaluation of an Online Resource for Hepatopulmonary Syndrome Patients and Caregivers: www.hpscare.com. ( 28302500 )
2017
47
Schistosomiasis and hepatopulmonary syndrome: the role of concomitant liver cirrhosis. ( 28591307 )
2017
48
Cryptogenic Cirrhosis and Hepatopulmonary Syndrome in a Boy with Hepatic Hemangioma in Botswana: A Case Report and Review of the Literature. ( 29410927 )
2017
49
The role of splenic reservoir monocytes in pulmonary vascular monocyte accumulation in experimental hepatopulmonary syndrome. ( 27029414 )
2016
50
Severe Hepatopulmonary Syndrome in an Adolescent Patient with Non-Cirrhotic Portal Fibrosis. ( 28008407 )
2016

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 46)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.57 AKT1 EDN1 EDNRA EDNRB NOS2 NOS3
2
Show member pathways
13.26 AKT1 HMOX1 NOS2 TNF VEGFA VWF
3
Show member pathways
13.13 AKT1 ALB CAV1 NOS2 NOS3 VEGFA
4
Show member pathways
12.94 AKT1 CAV1 NOS2 NOS3 VEGFA
5
Show member pathways
12.88 AKT1 EDN1 EDNRB NOS2 NOS3 VEGFA
6
Show member pathways
12.87 AKT1 NOS3 TNF VEGFA VWF
7
Show member pathways
12.79 AKT1 CAV1 EDN1 EDNRB NOS3 TNF
8 12.65 AKT1 EDNRA EDNRB HMOX1 NOS2 VEGFA
9
Show member pathways
12.63 AKT1 EDNRA EDNRB NOS3
10
Show member pathways
12.58 AKT1 EDN1 EDNRA VEGFA
11
Show member pathways
12.5 AKT1 NOS2 NOS3 TNF
12
Show member pathways
12.49 AKT1 NOS3 TNF VEGFA
13
Show member pathways
12.31 AKT1 NOS2 NOS3 VEGFA
14 12.2 AKT1 CAV1 TNF VEGFA
15
Show member pathways
12.15 EDNRA EDNRB NOS2 NOS3
16
Show member pathways
12.11 EDN1 HMOX1 NOS2 TNF
17 12.08 AKT1 NOS2 TNF VEGFA
18
Show member pathways
12.07 AKT1 EDN1 TNF
19 12 AKT1 NOS2 NOS3
20 11.94 AKT1 NOS3 VWF
21 11.92 AKT1 NOS3 TNF
22
Show member pathways
11.88 AKT1 NOS3 TNF
23 11.87 AKT1 EDN1 TNF
24 11.82 AKT1 HMOX1 NOS2 TNF VEGFA
25 11.78 AKT1 CAV1 NR0B2
26 11.76 HMOX1 NOS3 VWF
27
Show member pathways
11.7 AKT1 CAV1 NOS3
28
Show member pathways
11.7 AKT1 CAV1 NOS3 VEGFA
29 11.65 AKT1 EDN1 NOS3 TNF VEGFA
30 11.61 AKT1 NOS2 NOS3
31 11.59 EDN1 HMOX1 TNF
32
Show member pathways
11.58 NOS2 TNF VEGFA
33 11.51 EDN1 NOS3 VEGFA
34 11.47 AKT1 NOS2 VEGFA
35 11.43 AKT1 EDN1 EDNRA EDNRB
36 11.43 AKT1 EDN1 HMOX1 NOS2 NOS3 VEGFA
37 11.37 AKT1 EDN1 HMOX1 NOS2 VEGFA
38 11.31 AKT1 CAV1 EDN1 HMOX1 NOS3 TNF
39 11.27 EDN1 NOS2 VEGFA
40 11.15 EDN1 EDNRA EDNRB
41 11.15 EDN1 EDNRA EDNRB NOS3
42 11.08 NOS2 NOS3
43 11.03 AKT1 NOS3 VEGFA
44 10.96 HMOX1 TNF
45 10.76 NOS2 NOS3
46 10.53 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 CAV1 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 56)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.97 AKT1 CAV1 NR0B2 TNF VEGFA
2 angiogenesis GO:0001525 9.95 CAV1 HMOX1 NOS3 VEGFA
3 in utero embryonic development GO:0001701 9.92 EDN1 EDNRA NOS3 VEGFA
4 response to lipopolysaccharide GO:0032496 9.91 EDN1 EDNRB NOS2 NOS3
5 positive regulation of angiogenesis GO:0045766 9.89 HMOX1 NOS3 VEGFA
6 positive regulation of cytosolic calcium ion concentration GO:0007204 9.89 EDN1 EDNRA EDNRB
7 cytokine-mediated signaling pathway GO:0019221 9.89 AKT1 HMOX1 NOS2 TNF VEGFA
8 cellular response to lipopolysaccharide GO:0071222 9.88 EDNRB NOS2 TNF
9 positive regulation of protein phosphorylation GO:0001934 9.88 AKT1 EDNRB TNF VEGFA
10 platelet degranulation GO:0002576 9.87 ALB VEGFA VWF
11 positive regulation of DNA binding transcription factor activity GO:0051091 9.86 AKT1 EDN1 TNF
12 positive regulation of MAP kinase activity GO:0043406 9.82 EDN1 TNF VEGFA
13 response to hormone GO:0009725 9.8 AKT1 NOS2 NOS3
14 positive regulation of blood vessel endothelial cell migration GO:0043536 9.78 AKT1 NOS3 VEGFA
15 cellular response to hypoxia GO:0071456 9.78 AKT1 EDN1 HMOX1 VEGFA
16 positive regulation of nitric oxide biosynthetic process GO:0045429 9.77 AKT1 EDN1 TNF
17 negative regulation of gene expression GO:0010629 9.77 AKT1 NOS2 NR0B2 TNF VEGFA
18 lipopolysaccharide-mediated signaling pathway GO:0031663 9.73 AKT1 NOS3 TNF
19 membrane depolarization GO:0051899 9.72 CAV1 EDN1
20 positive regulation of protein complex assembly GO:0031334 9.72 TNF VEGFA
21 neural crest cell development GO:0014032 9.72 EDN1 EDNRA
22 vasodilation GO:0042311 9.71 EDNRB NOS3
23 regulation of pH GO:0006885 9.71 EDN1 EDNRB
24 positive regulation of blood vessel endothelial cell proliferation involved in sprouting angiogenesis GO:1903589 9.71 HMOX1 VEGFA
25 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.71 AKT1 CAV1 TNF VEGFA
26 cellular response to peptide hormone stimulus GO:0071375 9.7 CAV1 EDN1
27 regulation of blood vessel size GO:0050880 9.7 EDN1 NOS3
28 nitric oxide mediated signal transduction GO:0007263 9.7 NOS2 NOS3
29 positive regulation of protein localization to cell surface GO:2000010 9.69 AKT1 TNF
30 positive regulation of renal sodium excretion GO:0035815 9.69 EDN1 EDNRB
31 branching involved in blood vessel morphogenesis GO:0001569 9.69 EDN1 EDNRA VEGFA
32 negative regulation of macroautophagy GO:0016242 9.68 AKT1 HMOX1
33 positive regulation of urine volume GO:0035810 9.68 EDN1 EDNRB
34 positive regulation of guanylate cyclase activity GO:0031284 9.68 NOS2 NOS3
35 artery smooth muscle contraction GO:0014824 9.67 EDN1 EDNRA
36 cofactor metabolic process GO:0051186 9.67 AKT1 NOS3
37 enteric nervous system development GO:0048484 9.67 EDNRA EDNRB
38 response to fluid shear stress GO:0034405 9.66 AKT1 NOS3
39 positive regulation of chemokine biosynthetic process GO:0045080 9.66 HMOX1 TNF
40 arginine catabolic process GO:0006527 9.65 NOS2 NOS3
41 regulation of glucose transmembrane transport GO:0010827 9.65 EDN1 EDNRA
42 negative regulation of cellular protein metabolic process GO:0032269 9.64 EDN1 EDNRB
43 epithelial fluid transport GO:0042045 9.63 EDN1 EDNRB
44 regulation of systemic arterial blood pressure by endothelin GO:0003100 9.63 EDN1 NOS3
45 positive regulation of blood vessel diameter GO:0097755 9.63 HMOX1 NOS2 NOS3
46 regulation of nitric-oxide synthase activity GO:0050999 9.61 AKT1 CAV1 NOS3
47 vein smooth muscle contraction GO:0014826 9.6 EDN1 EDNRB
48 positive regulation of smooth muscle cell proliferation GO:0048661 9.56 AKT1 EDN1 HMOX1 TNF
49 regulation of the force of heart contraction by chemical signal GO:0003057 9.55 CAV1 NOS3
50 regulation of blood pressure GO:0008217 9.55 EDN1 EDNRA EDNRB HMOX1 NOS3

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.85 AKT1 ALB CAV1 TNF VEGFA VWF
2 protein homodimerization activity GO:0042803 9.73 AKT1 HMOX1 NOS2 NR0B2 VEGFA VWF
3 heme binding GO:0020037 9.65 HMOX1 NOS2 NOS3
4 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen GO:0016709 9.49 NOS2 NOS3
5 FMN binding GO:0010181 9.46 NOS2 NOS3
6 arginine binding GO:0034618 9.4 NOS2 NOS3
7 NADPH-hemoprotein reductase activity GO:0003958 9.32 NOS2 NOS3
8 tetrahydrobiopterin binding GO:0034617 9.16 NOS2 NOS3
9 nitric-oxide synthase activity GO:0004517 8.96 NOS2 NOS3
10 endothelin receptor activity GO:0004962 8.62 EDNRA EDNRB
11 protein binding GO:0005515 10.25 AKT1 ALB CAV1 EDN1 EDNRA 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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