MCID: HPT009
MIFTS: 53

Hepatopulmonary Syndrome

Categories: Rare diseases, Liver diseases, Gastrointestinal diseases

Aliases & Classifications for Hepatopulmonary Syndrome

MalaCards integrated aliases for Hepatopulmonary Syndrome:

Name: Hepatopulmonary Syndrome 12 49 51 41 14 69

Classifications:



External Ids:

Disease Ontology 12 DOID:900
ICD10 32 K76.81
ICD9CM 34 573.5
MeSH 41 D020065
SNOMED-CT 64 371067004
UMLS 69 C0600452

Summaries for Hepatopulmonary Syndrome

NIH Rare Diseases : 49 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. Last updated: 10/14/2017

MalaCards based summary : Hepatopulmonary Syndrome is related to visceral leishmaniasis and respiratory failure, 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 : 72 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 129)
# Related Disease Score Top Affiliating Genes
1 visceral leishmaniasis 29.7 ALB NOS2 TNF
2 respiratory failure 29.7 ALB EDN1 TNF
3 hereditary hemorrhagic telangiectasia 29.4 NOS3 VEGFA VWF
4 ischemia 28.6 CAV1 EDN1 HMOX1 NOS2 NOS3 VEGFA
5 portal hypertension 28.3 ALB EDN1 EDNRA NOS3 TNF VEGFA
6 pulmonary hypertension 27.5 CAV1 EDN1 EDNRA EDNRB NOS2 NOS3
7 platelet membrane fluidity 10.5 NOS2 NOS3
8 prinzmetal's variant angina 10.5 EDN1 NOS3
9 hemifacial spasm 10.4 NOS3 VEGFA
10 coronary artery vasospasm 10.4 EDN1 NOS3
11 liver disease 10.3
12 adrenal gland pheochromocytoma 10.3 AKT1 NOS3
13 tympanosclerosis 10.3 NOS2 TNF
14 hepatic vascular disease 10.3 ALB EDN1 NOS3
15 acroosteolysis 10.3 ALB VEGFA
16 burns 10.3 ALB TNF
17 impotence 10.3 EDN1 NOS2 NOS3
18 orthostatic intolerance 10.3 EDN1 NOS2 NOS3
19 chronic myocardial ischemia 10.3 NOS3 VEGFA
20 renovascular hypertension 10.3 EDN1 HMOX1 NOS3
21 hepatitis 10.3
22 critical limb ischemia 10.2 TNF VEGFA
23 idiopathic achalasia 10.2 NOS2 TNF
24 cerebral artery occlusion 10.2 NOS3 TNF
25 microvascular complications of diabetes 5 10.2 ALB NOS3 VEGFA
26 background diabetic retinopathy 10.2 ALB VEGFA
27 liver cirrhosis 10.2
28 brain edema 10.2 ALB HMOX1 VEGFA
29 chronic mountain sickness 10.2 EDN1 NOS3 VEGFA
30 acute mountain sickness 10.2 EDN1 NOS3 VEGFA
31 vasculogenic impotence 10.2 CAV1 NOS3
32 obstructive jaundice 10.2 ALB EDN1 TNF
33 sleep apnea 10.1 EDN1 NOS3 TNF
34 urinary system disease 10.1 ALB EDN1 TNF
35 eclampsia 10.1 ALB EDN1 TNF
36 intestinal disease 10.1 ALB EDNRB TNF
37 endotheliitis 10.1
38 gastrointestinal system disease 10.1 ALB EDNRB TNF
39 infiltrating angiolipoma 10.1 VEGFA VWF
40 atherosclerosis susceptibility 10.1 EDN1 NOS3 TNF
41 dystrophinopathies 10.1 NOS2 TNF
42 eales disease 10.1 NOS2 TNF VEGFA
43 koro 10.1 ALB EDNRA
44 central nervous system disease 10.1 HMOX1 NOS2 TNF
45 fibrosis of extraocular muscles, congenital, 1 10.1
46 cyanosis, transient neonatal 10.1
47 biliary atresia 10.0
48 bullous impetigo 10.0 EDNRA EDNRB
49 mandibulofacial dysostosis with alopecia 10.0 EDN1 EDNRA
50 myeloma, multiple 10.0 AKT1 ALB VEGFA

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:

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

Drugs & Therapeutics for Hepatopulmonary Syndrome

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

(show all 45)
# 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
3 Polygeline Phase 4
4 Liver Extracts Phase 4,Phase 2,Phase 1
5 Adrenergic Agents Phase 4
6 Adrenergic alpha-1 Receptor Antagonists Phase 4
7 Adrenergic alpha-Antagonists Phase 4
8 Adrenergic Antagonists Phase 4
9 Adrenergic beta-Antagonists Phase 4
10 Neurotransmitter Agents Phase 4
11 Antihypertensive Agents Phase 4,Phase 2
12
Norfloxacin Approved Phase 2 70458-96-7 4539
13
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
14
Iodine Approved, Investigational Phase 2 7553-56-2 807
15
Bosentan Approved, Investigational Phase 2 147536-97-8 104865
16
Niacin Approved, Investigational, Nutraceutical Phase 2 59-67-6 938
17
Nicotinamide Approved, Investigational, Nutraceutical Phase 2 98-92-0 936
18
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
19 Anti-Bacterial Agents Phase 2
20 Anti-Infective Agents Phase 2
21 Topoisomerase Inhibitors Phase 2
22 Cytochrome P-450 CYP1A2 Inhibitors Phase 2
23 Cytochrome P-450 Enzyme Inhibitors Phase 2
24 cadexomer iodine Phase 2
25 Micronutrients Phase 2
26 Trace Elements Phase 2
27 Nicotinic Acids Phase 2
28 Vitamin B Complex Phase 2
29 Vitamins Phase 2
30 Protein Kinase Inhibitors Phase 2
31 Endothelin Receptor Antagonists Phase 2
32 Folate Nutraceutical Phase 2
33 Vitamin B3 Nutraceutical Phase 2
34 Vitamin B9 Nutraceutical Phase 2
35
Pentoxifylline Approved, Investigational Phase 1 6493-05-6 4740
36 Phosphodiesterase Inhibitors Phase 1
37 Platelet Aggregation Inhibitors Phase 1
38 Protective Agents Phase 1
39 Radiation-Protective Agents Phase 1
40 Antioxidants Phase 1
41
Rifaximin Approved, Investigational 80621-81-4 6436173
42 rifamycin SV
43 Rifamycins
44 Gastrointestinal Agents
45 Nucleic Acid Synthesis Inhibitors

Interventional clinical trials:

(show all 11)

# Name Status NCT ID Phase Drugs
1 Echocardiographic Assessment of Intrapulmonary Vasodilatation: Agitated Saline Versus Polygeline Unknown status NCT00343122 Phase 4
2 Early Precise Diagnosis and Intervention of CPT Based on a Noninvasive 3D-vHPS Recruiting NCT02925975 Phase 4 Carvedilol
3 A Pilot Study of Norfloxacin for Hepatopulmonary Syndrome Completed NCT00362752 Phase 2 Norfloxacin;Placebo
4 Sorafenib for Hepatopulmonary Syndrome Recruiting 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 pentoxifylline and rifaximin;Pentoxifylline and placebo
9 Investigation of the Prevalence of Hepatopulmonary Syndrome in Cirrhosis Patients Caused by Hepatitis B in Western China Recruiting NCT03435406
10 Hepatopulmonary Syndrome and Postoperative Complications After Liver Transplantation : A Case-control Study Active, not recruiting NCT03092401
11 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:

38
Liver, Lung, Brain, Skin, Heart, Endothelial, Smooth Muscle

Publications for Hepatopulmonary Syndrome

Articles related to Hepatopulmonary Syndrome:

(show top 50) (show all 501)
# Title Authors Year
1
Placental Growth Factor: New Treatment Target Bringing Hope in Hepatopulmonary Syndrome. ( 29356037 )
2018
2
Impact of Bacterial Translocation on Hepatopulmonary Syndrome: A Prospective Observational Study. ( 29192374 )
2018
3
Development and Evaluation of an Online Resource for Hepatopulmonary Syndrome Patients and Caregivers: www.hpscare.com. ( 28302500 )
2017
4
Diammonium glycyrrhizinate alleviates hepatopulmonary syndrome via restoring superoxide dismutase 3 activity in rats. ( 28435094 )
2017
5
Ventilatory support and hospital stay after liver transplant in cirrhotic patients with hepatopulmonary syndrome. ( 29091154 )
2017
6
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
7
Severe Hepatopulmonary Syndrome in a Child with Caroli Syndrome. ( 28884036 )
2017
8
CXCR2 is involved in pulmonary intravascular macrophage accumulation and angiogenesis in a rat model of hepatopulmonary syndrome. ( 27879294 )
2017
9
Late complications of biliary atresia: hepatopulmonary syndrome and portopulmonary hypertension. ( 28983709 )
2017
10
Placental growth factor inhibition targets pulmonary angiogenesis and represents a novel therapy for hepatopulmonary syndrome in mice. ( 29023811 )
2017
11
Hepatopulmonary syndrome with large pulmonary arteriovenous malformations: CT findings with emphasis on its association with a mosaic pattern of the lung parenchyma. ( 28480722 )
2017
12
Unexplained cyanosis caused by hepatopulmonary syndrome in a girl with APECED syndrome. ( 28222032 )
2017
13
Do Late Bubbles Correspond to Early Hepatopulmonary Syndrome? ( 28612753 )
2017
14
Extracorporeal membrane oxygenation for post-transplant hypoxaemia following very severe hepatopulmonary syndrome. ( 29092970 )
2017
15
The effects of pioglitazone in cirrhotic rats with hepatopulmonary syndrome. ( 28969989 )
2017
16
Screening for Hepatopulmonary Syndrome in Cirrhotic Patients Using Technetium 99m-macroaggregated Albumin Perfusion Lung Scan (Tc-MAA): Diagnostic Approach and Clinical Correlations. ( 28961571 )
2017
17
Annexin A2-modulated proliferation of pulmonary arterial smooth muscle cells depends on caveolae and caveolin-1 in hepatopulmonary syndrome. ( 28729092 )
2017
18
Differential diagnosis of hepatopulmonary syndrome (HPS): Portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT). ( 28759737 )
2017
19
Hepatopulmonary syndrome is associated with the presence of hepatocellular carcinoma in patients with decompensated cirrhosis. ( 28243044 )
2017
20
Selective cyclooxygenase inhibition by SC-560 improves hepatopulmonary syndrome in cirrhotic rats. ( 28632747 )
2017
21
Hepatopulmonary syndrome: which blood gas analysis criteria and position should we use for diagnosis? ( 28972388 )
2017
22
Live donor liver transplantation for a child presented with severe hepatopulmonary syndrome and nodular liver lesions due to Abernethy malformation. ( 28078819 )
2017
23
Dyspnoea, cyanosis and digital clubbing in a 28-year-old patient as a result of hepatopulmonary syndrome. ( 29288484 )
2017
24
Dual organ beneficial effects of metformin in cirrhotic rats with hepatopulmonary syndrome. ( 28705726 )
2017
25
Isolated Intrapulmonary Vascular Dilatations and the Risk of Developing Hepatopulmonary Syndrome in Liver Transplant Candidates. ( 28611257 )
2017
26
Schistosomiasis and hepatopulmonary syndrome: the role of concomitant liver cirrhosis. ( 28591307 )
2017
27
Cryptogenic Cirrhosis and Hepatopulmonary Syndrome in a Boy with Hepatic Hemangioma in Botswana: A Case Report and Review of the Literature. ( 29410927 )
2017
28
Clinical efficacy of transjugular intrahepatic portosystemic shunt in the treatment of hepatopulmonary syndrome. ( 29245324 )
2017
29
Onset ages of hepatopulmonary syndrome and pulmonary hypertension in patients with biliary atresia. ( 28871319 )
2017
30
Hepatopulmonary Syndrome and Portopulmonary Hypertension: Implications for Liver Transplantation. ( 29128026 )
2017
31
Persistent Unexplained Dyspnea: A Case of Hepatopulmonary Syndrome. ( 28948051 )
2017
32
Pioglitazone decrease intrapulmonary shunt in biliary cirrhotic rats with hepatopulmonary syndrome. ( 28954708 )
2017
33
Successful venoarterial extracorporeal membrane oxygenation for prolonged hepatopulmonary syndrome following pediatric liver transplantation: A case report and review of the literature. ( 28833992 )
2017
34
Cyclooxygenase-2 promotes pulmonary intravascular macrophage accumulation by exacerbating BMP signaling in rat experimental hepatopulmonary syndrome. ( 28642034 )
2017
35
A case report of suspected hepatopulmonary syndrome secondary to ductal plate malformation with chronic active hepatitis in a dog. ( 26616155 )
2016
36
Hepatopulmonary Syndrome in Patients With Cystic Fibrosis and Liver Disease. ( 26867851 )
2016
37
Quercetin alleviates pulmonary angiogenesis in a rat model of hepatopulmonary syndrome. ( 27383124 )
2016
38
Inhibition of autophagy ameliorates pulmonary microvascular dilation and PMVECs excessive proliferation in rat experimental hepatopulmonary syndrome. ( 27480323 )
2016
39
Prevalence, Severity, and Prognostic Effect of Hepatopulmonary Syndrome in Liver Transplant Candidates. ( 27020907 )
2016
40
Hepatopulmonary Syndrome and Liver Transplantation: A Recent Review of the Literature. ( 27047772 )
2016
41
CHARACTER AND FREQUENCY OF THE VARIATIONS OF CO- AND POLYMORBID SYNTROPIC EXTRAHEPATIC LESIONS AND THEIR DEPENDENCE ON THE HEPATOPULMONARY SYNDROME SEVERITY DEGREE IN CIRRHOTIC PATIENTS. ( 28009313 )
2016
42
Autoimmune hepatitis in a child presenting with hepatopulmonary syndrome (HPS). ( 26992455 )
2016
43
CT Scan Does Not Differentiate Patients with Hepatopulmonary Syndrome from Other Patients with Liver Disease. ( 27384058 )
2016
44
Paediatric intrapulmonary right-to-left shunt caused by hepatopulmonary syndrome. ( 27165716 )
2016
45
Serum bile acids in patients with hepatopulmonary syndrome. ( 27951601 )
2016
46
Nitric oxide metabolites, nitrative stress, and paraoxonase activity in hepatopulmonary syndrome. ( 26051624 )
2016
47
Mechanical characteristics of the pulmonary artery in beagle dogs with hepatopulmonary syndrome and portopulmonary hypertension. ( 26870333 )
2016
48
A Case of Hepatopulmonary Syndrome. ( 27134920 )
2016
49
Hepatopulmonary Syndrome: A Brief Review. ( 27352437 )
2016
50
Hepatopulmonary syndrome: What we know and what we would like to know. ( 27433086 )
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 43)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.51 AKT1 EDN1 EDNRA EDNRB NOS2 NOS3
2
Show member pathways
13.26 AKT1 HMOX1 NOS2 TNF VEGFA VWF
3
Show member pathways
13.17 AKT1 ALB CAV1 NOS2 NOS3 VEGFA
4
Show member pathways
12.94 AKT1 CAV1 NOS2 NOS3 VEGFA
5
Show member pathways
12.87 AKT1 NOS3 TNF VEGFA VWF
6 12.69 AKT1 EDNRA EDNRB HMOX1 NOS2 VEGFA
7
Show member pathways
12.69 AKT1 CAV1 EDN1 EDNRB NOS3 TNF
8
Show member pathways
12.63 AKT1 EDNRA EDNRB NOS3
9
Show member pathways
12.57 AKT1 EDN1 EDNRA VEGFA
10
Show member pathways
12.5 AKT1 NOS2 NOS3 TNF
11
Show member pathways
12.49 AKT1 NOS3 TNF VEGFA
12
Show member pathways
12.4 AKT1 EDN1 EDNRB NOS2 NOS3 VEGFA
13
Show member pathways
12.31 AKT1 NOS2 NOS3 VEGFA
14 12.21 AKT1 CAV1 TNF VEGFA
15
Show member pathways
12.15 EDNRA EDNRB NOS2 NOS3
16 12.08 AKT1 NOS2 TNF VEGFA
17 12 AKT1 NOS2 NOS3
18 11.94 AKT1 NOS3 VWF
19 11.92 AKT1 NOS3 TNF
20
Show member pathways
11.87 AKT1 NOS3 TNF
21 11.86 AKT1 EDN1 TNF
22 11.82 AKT1 HMOX1 NOS2 TNF VEGFA
23 11.78 AKT1 CAV1 NR0B2
24 11.76 HMOX1 NOS3 VWF
25
Show member pathways
11.74 AKT1 CAV1 NOS3 VEGFA
26
Show member pathways
11.7 AKT1 CAV1 NOS3
27 11.65 AKT1 EDN1 NOS3 TNF VEGFA
28 11.61 AKT1 NOS2 NOS3
29 11.59 EDN1 HMOX1 TNF
30
Show member pathways
11.58 NOS2 TNF VEGFA
31 11.5 EDN1 NOS3 VEGFA
32 11.47 AKT1 NOS2 VEGFA
33 11.46 AKT1 EDN1 EDNRA EDNRB
34 11.43 AKT1 EDN1 HMOX1 NOS2 NOS3 VEGFA
35 11.37 AKT1 EDN1 HMOX1 NOS2 VEGFA
36 11.31 AKT1 CAV1 EDN1 HMOX1 NOS3 TNF
37 11.28 EDN1 NOS2 VEGFA
38 11.18 EDN1 EDNRA EDNRB
39 11.15 EDN1 EDNRA EDNRB NOS3
40 11.07 AKT1 NOS3 VEGFA
41 10.96 HMOX1 TNF
42 10.76 NOS2 NOS3
43 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 all 49)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.99 CAV1 NR0B2 TNF VEGFA
2 angiogenesis GO:0001525 9.93 CAV1 HMOX1 NOS3 VEGFA
3 in utero embryonic development GO:0001701 9.91 EDN1 EDNRA NOS3 VEGFA
4 positive regulation of protein phosphorylation GO:0001934 9.88 AKT1 EDNRB TNF VEGFA
5 positive regulation of cytosolic calcium ion concentration GO:0007204 9.87 EDN1 EDNRA EDNRB
6 positive regulation of angiogenesis GO:0045766 9.87 HMOX1 NOS3 VEGFA
7 cellular response to lipopolysaccharide GO:0071222 9.86 EDNRB NOS2 TNF
8 platelet degranulation GO:0002576 9.85 ALB VEGFA VWF
9 positive regulation of DNA binding transcription factor activity GO:0051091 9.85 AKT1 EDN1 TNF
10 positive regulation of MAP kinase activity GO:0043406 9.8 EDN1 TNF VEGFA
11 cellular response to hypoxia GO:0071456 9.78 AKT1 EDN1 HMOX1 VEGFA
12 positive regulation of nitric oxide biosynthetic process GO:0045429 9.77 AKT1 EDN1 TNF
13 negative regulation of gene expression GO:0010629 9.77 AKT1 NOS2 NR0B2 TNF VEGFA
14 positive regulation of blood vessel endothelial cell migration GO:0043536 9.74 AKT1 NOS3 VEGFA
15 lipopolysaccharide-mediated signaling pathway GO:0031663 9.73 AKT1 NOS3 TNF
16 branching involved in blood vessel morphogenesis GO:0001569 9.72 EDN1 EDNRA VEGFA
17 vasodilation GO:0042311 9.71 EDNRB NOS3
18 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.71 AKT1 CAV1 TNF VEGFA
19 neural crest cell development GO:0014032 9.7 EDN1 EDNRA
20 regulation of pH GO:0006885 9.7 EDN1 EDNRB
21 cellular response to peptide hormone stimulus GO:0071375 9.69 CAV1 EDN1
22 regulation of blood vessel size GO:0050880 9.69 EDN1 NOS3
23 nitric oxide mediated signal transduction GO:0007263 9.68 NOS2 NOS3
24 negative regulation of cAMP biosynthetic process GO:0030818 9.68 EDN1 EDNRA
25 negative regulation of macroautophagy GO:0016242 9.67 AKT1 HMOX1
26 positive regulation of renal sodium excretion GO:0035815 9.67 EDN1 EDNRB
27 glucose transport GO:0015758 9.67 AKT1 EDN1 EDNRA
28 positive regulation of urine volume GO:0035810 9.66 EDN1 EDNRB
29 enteric nervous system development GO:0048484 9.66 EDNRA EDNRB
30 positive regulation of guanylate cyclase activity GO:0031284 9.65 NOS2 NOS3
31 artery smooth muscle contraction GO:0014824 9.65 EDN1 EDNRA
32 response to fluid shear stress GO:0034405 9.65 AKT1 NOS3
33 positive regulation of chemokine biosynthetic process GO:0045080 9.64 HMOX1 TNF
34 arginine catabolic process GO:0006527 9.64 NOS2 NOS3
35 positive regulation of blood vessel diameter GO:0097755 9.63 HMOX1 NOS2 NOS3
36 regulation of systemic arterial blood pressure by endothelin GO:0003100 9.62 EDN1 NOS3
37 negative regulation of cellular protein metabolic process GO:0032269 9.62 EDN1 EDNRB
38 epithelial fluid transport GO:0042045 9.61 EDN1 EDNRB
39 regulation of nitric-oxide synthase activity GO:0050999 9.61 AKT1 CAV1 NOS3
40 vein smooth muscle contraction GO:0014826 9.6 EDN1 EDNRB
41 positive regulation of smooth muscle cell proliferation GO:0048661 9.56 AKT1 EDN1 HMOX1 TNF
42 regulation of blood pressure GO:0008217 9.55 EDN1 EDNRA EDNRB HMOX1 NOS3
43 endothelin receptor signaling pathway GO:0086100 9.54 EDNRA EDNRB
44 smooth muscle hyperplasia GO:0014806 9.52 HMOX1 NOS3
45 regulation of the force of heart contraction by chemical signal GO:0003057 9.51 CAV1 NOS3
46 nitric oxide biosynthetic process GO:0006809 9.5 AKT1 NOS2 NOS3
47 vasoconstriction GO:0042310 9.26 CAV1 EDN1 EDNRA EDNRB
48 response to hypoxia GO:0001666 9.1 CAV1 EDN1 EDNRA HMOX1 NOS2 VEGFA
49 negative regulation of transcription by RNA polymerase II GO:0000122 10.06 CAV1 EDN1 EDNRB NR0B2 TNF VEGFA

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

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.91 AKT1 ALB CAV1 TNF VEGFA VWF
2 heme binding GO:0020037 9.65 HMOX1 NOS2 NOS3
3 protein homodimerization activity GO:0042803 9.63 AKT1 HMOX1 NOS2 NR0B2 VEGFA VWF
4 FMN binding GO:0010181 9.46 NOS2 NOS3
5 arginine binding GO:0034618 9.4 NOS2 NOS3
6 NADPH-hemoprotein reductase activity GO:0003958 9.32 NOS2 NOS3
7 tetrahydrobiopterin binding GO:0034617 9.26 NOS2 NOS3
8 nitric-oxide synthase activity GO:0004517 8.96 NOS2 NOS3
9 endothelin receptor activity GO:0004962 8.62 EDNRA EDNRB
10 protein binding GO:0005515 10.18 AKT1 ALB CAV1 EDN1 EDNRA EDNRB

Sources for Hepatopulmonary Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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