Hepatopulmonary Syndrome disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

MCID: HPT009 MIFTS: 52	Hepatopulmonary Syndrome Categories: Endocrine diseases, Gastrointestinal diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs Expand all tables

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Aliases & Classifications for Hepatopulmonary Syndrome

MalaCards integrated aliases for Hepatopulmonary Syndrome:

Name: Hepatopulmonary Syndrome ¹² ⁵³ ⁵⁵ ⁴⁴ ¹⁵ ⁷³

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Endocrine diseases Gastrointestinal diseases

See all MalaCards categories (disease lists)

External Ids:

Disease Ontology ¹² DOID:900

ICD10 ³³ K76.81

ICD9CM ³⁵ 573.5

MeSH ⁴⁴ D020065

UMLS ⁷³ C0600452

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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...

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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:

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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

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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

Dobutamine

Approved

Phase 2

34368-04-2

36811

Norepinephrine

Approved

Phase 2

51-41-2

439260

Norfloxacin

Approved

Phase 2

70458-96-7

4539

Nicotinamide

Approved, Investigational

Phase 2

98-92-0

936

Iodine

Approved, Investigational

Phase 2

7553-56-2

807

Sorafenib

Approved, Investigational

Phase 2

284461-73-0

216239 406563

Bosentan

Approved, Investigational

Phase 2

147536-97-8

104865

Folic Acid

Approved, Nutraceutical, Vet_approved

Phase 2

59-30-3

6037

Niacin

Approved, Investigational, Nutraceutical

Phase 2

59-67-6

938

Liver Extracts

Phase 2,Phase 1

Anti-Bacterial Agents

Phase 2,Not Applicable

Bilirubin

Phase 2

635-65-4

5280352

Antibiotics, Antitubercular

Phase 2,Not Applicable

Vasoconstrictor Agents

Phase 2,Phase 1

Anti-Infective Agents

Phase 2,Not Applicable

Cytochrome P-450 Enzyme Inhibitors

Phase 2

Cytochrome P-450 CYP1A2 Inhibitors

Phase 2

Topoisomerase Inhibitors

Phase 2

Vitamin B9

Phase 2

Vitamins

Phase 2

Trace Elements

Phase 2

Vitamin B3

Phase 2

Protein Kinase Inhibitors

Phase 2

Micronutrients

Phase 2

cadexomer iodine

Phase 2

Nicotinic Acids

Phase 2

Vitamin B Complex

Phase 2

Folate

Phase 2

Antihypertensive Agents

Phase 2

Endothelin Receptor Antagonists

Phase 2

Pentoxifylline

Approved, Investigational

Phase 1,Not Applicable

6493-05-6

4740

Midodrine

Approved

Phase 1

133163-28-7, 42794-76-3

4195

Vasodilator Agents

Phase 1,Not Applicable

Antioxidants

Phase 1,Not Applicable

Radiation-Protective Agents

Phase 1,Not Applicable

Phosphodiesterase Inhibitors

Phase 1,Not Applicable

Free Radical Scavengers

Phase 1,Not Applicable

Protective Agents

Phase 1,Not Applicable

Platelet Aggregation Inhibitors

Phase 1,Not Applicable

Sympathomimetics

Phase 1

Adrenergic Agonists

Phase 1

Autonomic Agents

Phase 1

Adrenergic alpha-Agonists

Phase 1

Neurotransmitter Agents

Phase 1

Peripheral Nervous System Agents

Phase 1

Adrenergic Agents

Phase 1

Rifaximin

Approved, Investigational

Not Applicable

80621-81-4

6436173

rifamycin SV

Not Applicable

Gastrointestinal Agents

Not Applicable

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

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Genetic Tests for Hepatopulmonary Syndrome

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Anatomical Context for Hepatopulmonary Syndrome

MalaCards organs/tissues related to Hepatopulmonary Syndrome:

⁴¹

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

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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 )	Ceza MR...Coral GP	2019
2	Hepatopulmonary syndrome-attributed extreme hypoxemia and polycythemia revealing liver cirrhosis. ( 30269997 )	Nuzzo A...M?garbane B	2019
3	Placental Growth Factor: New Treatment Target Bringing Hope in Hepatopulmonary Syndrome. ( 29356037 )	Fernandez M.	2018
4	Review article: Update on current and emergent data on hepatopulmonary syndrome. ( 29599604 )	Soulaidopoulos S....Goulis I.	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 )	Yang C....Lu K.	2018
6	Hepatopulmonary Syndrome and Diffuse Interstitial Lung Disease: An Unusual Combination. ( 29588084 )	Rodriguez-Pons L....Portillo K.	2018
7	Hepatopulmonary syndrome. ( 29483331 )	Gupta S....Krowka M.J.	2018
8	Potential clinical targets in hepatopulmonary syndrome: lessons from experimental models. ( 29729196 )	Raevens S....Fallon M.B.	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 )	Tsauo J...Li X	2018
20	Hepatopulmonary Syndrome in Children: A 20-Year Review of Presenting Symptoms, Clinical Progression, and Transplant Outcome. ( 30066494 )	Warner S...Beath SV	2018
21	Pulse Oximetry is Insensitive for Detection of Hepatopulmonary Syndrome in Patients evaluated for Liver Transplantation. ( 30070715 )	Forde KA...Pulmonary Vascular Complications of Liver Disease 2 (PVCLD2) Study Group	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 )	Krishnan S...Lahm T	2018
23	Multifocal stroke with proliferation of small cerebral arteries in hepatopulmonary syndrome. ( 30105173 )	Younce JR...Lee JM	2018
24	Evidence for an Association Between Intrahepatic Vascular Changes and the Development of Hepatopulmonary Syndrome. ( 30292761 )	Lejealle C...Rautou PE	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 )	Liu JW...Chen DQ	2018
26	Cardiac involvement in patients with end-stage liver disease, beyond the hepatopulmonary syndrome. ( 30409693 )	Colunga Blanco S...de la Hera Galarza JM	2018
27	Outcome of liver transplantation for hepatopulmonary syndrome: a Eurotransplant experience. ( 30487203 )	Raevens S...Colle I	2018
28	Late complications of biliary atresia: hepatopulmonary syndrome and portopulmonary hypertension. ( 28983709 )	Karrer F.M....Estrada A.E.	2017
29	Selective cyclooxygenase inhibition by SC-560 improves hepatopulmonary syndrome in cirrhotic rats. ( 28632747 )	Chang C.C....Lee S.D.	2017
30	Pioglitazone decrease intrapulmonary shunt in biliary cirrhotic rats with hepatopulmonary syndrome. ( 28954708 )	Tsai H.C....Yang Y.Y.	2017
31	Ventilatory support and hospital stay after liver transplant in cirrhotic patients with hepatopulmonary syndrome. ( 29091154 )	Pereira J.L.F....Marroni C.A.	2017
32	Differential diagnosis of hepatopulmonary syndrome (HPS): Portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT). ( 28759737 )	Krynytska I....Sopel O.	2017
33	Dual organ beneficial effects of metformin in cirrhotic rats with hepatopulmonary syndrome. ( 28705726 )	Liu C.W....Yang Y.Y.	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 )	Gorospe SarasA_a L....PAcrez-Templado LadrA^n de Guevara J.	2017
35	Live donor liver transplantation for a child presented with severe hepatopulmonary syndrome and nodular liver lesions due to Abernethy malformation. ( 28078819 )	Yilmaz C....Kilic M.	2017
36	Annexin A2-modulated proliferation of pulmonary arterial smooth muscle cells depends on caveolae and caveolin-1 in hepatopulmonary syndrome. ( 28729092 )	Liao L....Gao J.	2017
37	Unexplained cyanosis caused by hepatopulmonary syndrome in a girl with APECED syndrome. ( 28222032 )	Celmeli F....Turkkahraman D.	2017
38	Dyspnoea, cyanosis and digital clubbing in a 28-year-old patient as a result of hepatopulmonary syndrome. ( 29288484 )	ZieliA8ski M....Ziora D.	2017
39	Isolated Intrapulmonary Vascular Dilatations and the Risk of Developing Hepatopulmonary Syndrome in Liver Transplant Candidates. ( 28611257 )	Mendizabal M....Silva M.O.	2017
40	Successful venoarterial extracorporeal membrane oxygenation for prolonged hepatopulmonary syndrome following pediatric liver transplantation: A case report and review of the literature. ( 28833992 )	Khan H.H....Khan K.M.	2017
41	Extracorporeal membrane oxygenation for post-transplant hypoxaemia following very severe hepatopulmonary syndrome. ( 29092970 )	Kumar L....Surendran S.	2017
42	Clinical efficacy of transjugular intrahepatic portosystemic shunt in the treatment of hepatopulmonary syndrome. ( 29245324 )	Zhao H....He F.	2017
43	Hepatopulmonary syndrome is associated with the presence of hepatocellular carcinoma in patients with decompensated cirrhosis. ( 28243044 )	Soulaidopoulos S....Cholongitas I.9.	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 )	Fragaki M....Kouroumalis E.	2017
45	Do Late Bubbles Correspond to Early Hepatopulmonary Syndrome? ( 28612753 )	DuBrock H.M....J Krowka M.	2017
46	CXCR2 is involved in pulmonary intravascular macrophage accumulation and angiogenesis in a rat model of hepatopulmonary syndrome. ( 27879294 )	Li X....Zhang H.	2017
47	Hepatopulmonary syndrome: which blood gas analysis criteria and position should we use for diagnosis? ( 28972388 )	Grilo I....Grilo A.	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

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Genes for Hepatopulmonary Syndrome

Genes related to Hepatopulmonary Syndrome (0 elite genes):

(show all 13)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence	PubmedIds
1	ALB	Albumin	Protein Coding	31.63	DISEASES inferred ¹⁵ Novoseek inferred ⁵⁵ GeneCards inferred via : Publications (show sections)	10453952 19953657 11782297 (more) 9453490 17353701 17558074 9549117 16323392
2	HMOX1	Heme Oxygenase 1	Protein Coding	23.91	DISEASES inferred ¹⁵ Novoseek inferred ⁵⁵ GeneCards inferred via : Publications (show sections)	15685544
3	NOS3	Nitric Oxide Synthase 3	Protein Coding	23.23	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
4	EDN1	Endothelin 1	Protein Coding	23.2	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
5	EDNRB	Endothelin Receptor Type B	Protein Coding	22.19	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
6	NOS2	Nitric Oxide Synthase 2	Protein Coding	21.8	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
7	NR0B2	Nuclear Receptor Subfamily 0 Group B Member 2	Protein Coding	21.41	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
8	TNF	Tumor Necrosis Factor	Protein Coding	20.87	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
9	VEGFA	Vascular Endothelial Growth Factor A	Protein Coding	20.81	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
10	AKT1	AKT Serine/Threonine Kinase 1	Protein Coding	20.67	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
11	BMPR2	Bone Morphogenetic Protein Receptor Type 2	Protein Coding	20.57	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
12	VWF	Von Willebrand Factor	Protein Coding	20.49	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
13	ACVRL1	Activin A Receptor Like Type 1	Protein Coding	20.32	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)

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Variations for Hepatopulmonary Syndrome

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Expression for Hepatopulmonary Syndrome

Search GEO for disease gene expression data for Hepatopulmonary Syndrome.

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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	PEDF Induced Signaling ⁶⁴ Show member pathways all-trans-Retinoic Acid Signaling in Brain ⁶⁴ Cytokine-cytokine receptor interaction ³⁷ Endothelin-1 Signaling Pathway ⁶⁴ Glucocorticoid Receptor Signaling ⁶⁴ IL-6 Pathway ⁶⁴ MIF Mediated Glucocorticoid Regulation ⁶⁴ MIF Regulation of Innate Immune Cells ⁶⁴ NFAT Signaling and Lymphocyte Interactions ⁶⁴ PGC1Alpha Pathway ⁶⁴ RAR-Gamma-RXR-Alpha Degradation ⁶⁴ STAT3 Pathway ⁶⁴	13.48	ACVRL1 AKT1 BMPR2 EDN1 EDNRB NOS2
2	Cytokine Signaling in Immune system ⁶⁶ Show member pathways Signaling by Interleukins ⁶⁶	13.21	AKT1 HMOX1 NOS2 TNF VEGFA VWF
3	Response to elevated platelet cytosolic Ca2+ ⁶⁶ Show member pathways Disinhibition of SNARE formation ⁶⁶ Hemostasis ⁶⁶ Platelet activation, signaling and aggregation ⁶⁶ Platelet degranulation ⁶⁶	13.17	AKT1 ALB NOS2 NOS3 VEGFA VWF
4	PI3K-Akt signaling pathway ³⁷ Show member pathways Human papillomavirus infection ³⁷	12.86	AKT1 NOS3 TNF VEGFA VWF
5	Development Angiotensin activation of ERK ²² Show member pathways Cell adhesion Integrin inside-out signaling ²² Development ACM2 and ACM4 activation of ERK ²² Development Angiotensin signaling via PYK2 ²² Development EDNRB signaling ²² Development EPO-induced MAPK pathway ²² Development G-Proteins mediated regulation MARK-ERK signaling ²² Signal transduction IP3 signaling ²²	12.8	AKT1 EDN1 EDNRB NOS3 TNF
6	Circadian entrainment ³⁷ Show member pathways Cholinergic synapse ³⁷ Dopaminergic synapse ³⁷ Estrogen signaling pathway ³⁷ Glutamatergic synapse ³⁷ Relaxin receptors ⁶⁶ Relaxin signaling pathway ³⁷ Synaptic Neurotransmission Pathways: Glutamatergic Excitation ⁶⁵	12.78	AKT1 EDN1 EDNRB NOS2 NOS3 VEGFA
7	Pathways in cancer ³⁷	12.68	AKT1 EDNRB HMOX1 NOS2 VEGFA
8	Immune response IL-23 signaling pathway ²² Show member pathways Angiopoietin receptor Tie2-mediated signaling ¹ Angiopoietin-TIE2 Signaling ⁶⁴ Development Angiopoietin - Tie2 signaling ²² Development PDGF signaling via STATs and NF-kB ²² IL23-mediated signaling events ¹ Immune response IL-10 signaling pathway ²² Rac1/Pak1/p38/MMP-2 pathway ¹	12.47	AKT1 NOS2 NOS3 TNF
9	Immune response Fc epsilon RI pathway ²² Show member pathways Fc epsilon RI signaling pathway ³⁷ Fc-epsilon receptor I signaling in mast cells ¹ Immune response BCR pathway ²² VEGF signaling pathway ³⁷	12.47	AKT1 NOS3 TNF VEGFA
10	Development VEGF signaling via VEGFR2 - generic cascades ²² Show member pathways Development VEGF signaling and activation ²² VEGF - VEGF R2 Signaling Pathways ⁶⁵	12.28	AKT1 NOS2 NOS3 VEGFA
11	MicroRNAs in cardiomyocyte hypertrophy ¹ Show member pathways Cardiac Hypertrophic Response ¹	12.05	AKT1 EDN1 TNF
12	Integrated Breast Cancer Pathway ¹	12.04	AKT1 BMPR2 VEGFA
13	IL-1 Family Signaling Pathways ⁶⁵ Show member pathways Immune response IL-1 signaling pathway ²² Mucin expression in CF via TLRs, EGFR signaling pathways ²²	12.04	EDN1 HMOX1 NOS2 TNF
14	Colorectal Cancer Metastasis ⁶⁴	12	AKT1 NOS2 TNF VEGFA
15	Apelin signaling pathway ³⁷	11.98	AKT1 NOS2 NOS3
16	Platelet activation ³⁷	11.93	AKT1 NOS3 VWF
17	Sphingolipid signaling pathway ³⁷	11.91	AKT1 NOS3 TNF
18	TNF signaling pathway ³⁷	11.87	AKT1 EDN1 TNF
19	Insulin resistance ³⁷ Show member pathways protein O-[N-acetyl]-glucosylation ¹	11.86	AKT1 NOS3 TNF
20	Interleukin-4 and 13 signaling ⁶⁶	11.78	AKT1 HMOX1 NOS2 TNF VEGFA
21	Signaling events mediated by VEGFR1 and VEGFR2 ¹ Show member pathways VEGFR1 specific signals ¹	11.76	AKT1 NOS3 VEGFA
22	Angiogenesis (CST) ⁴	11.73	HMOX1 NOS3 VWF
23	AGE-RAGE signaling pathway in diabetic complications ³⁷	11.65	AKT1 EDN1 NOS3 TNF VEGFA
24	AGE/RAGE pathway ¹	11.58	AKT1 NOS2 NOS3
25	Lung fibrosis ¹	11.55	EDN1 HMOX1 TNF
26	Photodynamic therapy-induced NF-kB survival signaling ¹ Show member pathways Immune response MIF-mediated glucocorticoid regulation ²²	11.54	NOS2 TNF VEGFA
27	HIF1Alpha Pathway ⁶⁴	11.46	EDN1 NOS3 VEGFA
28	HIF-1 signaling pathway ³⁷	11.43	AKT1 EDN1 HMOX1 NOS2 NOS3 VEGFA
29	Endothelins ¹	11.42	AKT1 EDN1 EDNRB
30	Hepatitis C and Hepatocellular Carcinoma ¹	11.39	AKT1 NOS2 VEGFA
31	HIF-1-alpha transcription factor network ¹	11.37	AKT1 EDN1 HMOX1 NOS2 VEGFA
32	Fluid shear stress and atherosclerosis ³⁷	11.31	AKT1 BMPR2 EDN1 HMOX1 NOS3 TNF
33	Photodynamic therapy-induced HIF-1 survival signaling ¹	11.2	EDN1 NOS2 VEGFA
34	Endothelin Pathways ¹	11.11	EDN1 EDNRB NOS3
35	Doxorubicin Pathway (Cardiomyocyte Cell), Pharmacodynamics ⁶¹	11.06	NOS2 NOS3
36	Overview of nanoparticle effects ¹	10.95	HMOX1 TNF
37	Angiogenesis (WikiPathways) ¹	10.92	AKT1 NOS3 VEGFA
38	NO-dependent CFTR activation (normal and CF) ²²	10.76	NOS2 NOS3
39	Effects of Nitric Oxide ¹	10.5	NOS2 NOS3

Sources

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

Sources for Hepatopulmonary Syndrome

¹ BioSystems

² BitterDB

³ CDC

⁴ Cell Signaling Technology

⁵ ClinicalTrials

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¹⁰ dbSNP

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⁴⁸ NCBI Bookshelf

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⁷¹ TGDB

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⁷³ UMLS

⁷⁴ UMLS via Orphanet

⁷⁵ UniProtKB/Swiss-Prot

⁷⁶ Wikipedia