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Hepatopulmonary syndrome

MedGen UID:
109071
Concept ID:
C0600452
Disease or Syndrome
Synonyms: Hepato Pulmonary Syndrome; Hepato-Pulmonary Syndrome; Hepato-Pulmonary Syndromes; Hepatopulmonary Syndrome; Hepatopulmonary Syndromes; Syndrome, Hepato-Pulmonary; Syndrome, Hepatopulmonary; Syndromes, Hepato-Pulmonary; Syndromes, Hepatopulmonary
SNOMED CT: Hepatopulmonary syndrome (371067004)
 
Monarch Initiative: MONDO:0004694

Definition

A disorder more commonly seen in patients with chronic liver disease and portal hypertension. Pulmonary manifestations include dyspnea and hypoxemia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHepatopulmonary syndrome

Professional guidelines

PubMed

Yoshiji H, Nagoshi S, Akahane T, Asaoka Y, Ueno Y, Ogawa K, Kawaguchi T, Kurosaki M, Sakaida I, Shimizu M, Taniai M, Terai S, Nishikawa H, Hiasa Y, Hidaka H, Miwa H, Chayama K, Enomoto N, Shimosegawa T, Takehara T, Koike K
J Gastroenterol 2021 Jul;56(7):593-619. Epub 2021 Jul 7 doi: 10.1007/s00535-021-01788-x. PMID: 34231046Free PMC Article
Bloom S, Kemp W, Lubel J
Intern Med J 2015 Jan;45(1):16-26. doi: 10.1111/imj.12590. PMID: 25230084
Khanna R, Sarin SK
J Hepatol 2014 Feb;60(2):421-41. Epub 2013 Aug 23 doi: 10.1016/j.jhep.2013.08.013. PMID: 23978714

Recent clinical studies

Etiology

De Gasperi A, Spagnolin G, Ornaghi M, Petrò L, Biancofiore G
Best Pract Res Clin Anaesthesiol 2020 Mar;34(1):51-68. Epub 2020 Feb 18 doi: 10.1016/j.bpa.2020.02.002. PMID: 32334787
Hilscher MB, Kamath PS
Clin Liver Dis 2019 May;23(2):209-220. Epub 2019 Mar 8 doi: 10.1016/j.cld.2018.12.004. PMID: 30947872
Cardoso FS, Karvellas CJ
J Intensive Care Med 2019 May;34(5):355-363. Epub 2018 Jun 10 doi: 10.1177/0885066618781526. PMID: 29886790
Khanna R, Sarin SK
J Hepatol 2014 Feb;60(2):421-41. Epub 2013 Aug 23 doi: 10.1016/j.jhep.2013.08.013. PMID: 23978714
Gaines DI, Fallon MB
Liver Int 2004 Oct;24(5):397-401. doi: 10.1111/j.1478-3231.2004.0944.x. PMID: 15482333

Diagnosis

Kumar P, Rao PN
N Engl J Med 2020 Mar 5;382(10):e14. doi: 10.1056/NEJMicm1901205. PMID: 32130817
Franchi-Abella S, Gonzales E, Ackermann O, Branchereau S, Pariente D, Guérin F; International Registry of Congenital Portosystemic Shunt members
Abdom Radiol (NY) 2018 Aug;43(8):2023-2036. doi: 10.1007/s00261-018-1619-8. PMID: 29730740
Lv Y, Fan D
Dig Dis Sci 2015 Jul;60(7):1914-23. Epub 2015 Mar 3 doi: 10.1007/s10620-015-3593-0. PMID: 25732713
Koch DG, Fallon MB
Clin Liver Dis 2014 May;18(2):407-20. Epub 2014 Feb 25 doi: 10.1016/j.cld.2014.01.003. PMID: 24679503
Rodríguez-Roisin R, Krowka MJ
N Engl J Med 2008 May 29;358(22):2378-87. doi: 10.1056/NEJMra0707185. PMID: 18509123

Therapy

Cardoso FS, Karvellas CJ
J Intensive Care Med 2019 May;34(5):355-363. Epub 2018 Jun 10 doi: 10.1177/0885066618781526. PMID: 29886790
Gana JC, Serrano CA, Ling SC
Ann Hepatol 2016 May-Jun;15(3):303-13. doi: 10.5604/16652681.1198799. PMID: 27049484
Machicao VI, Fallon MB
Semin Respir Crit Care Med 2012 Feb;33(1):11-6. Epub 2012 Mar 23 doi: 10.1055/s-0032-1301730. PMID: 22447256
Sussman NL, Kochar R, Fallon MB
Curr Opin Organ Transplant 2011 Jun;16(3):281-8. doi: 10.1097/MOT.0b013e32834664df. PMID: 21543981
Golbin JM, Krowka MJ
Clin Chest Med 2007 Mar;28(1):203-18, ix. doi: 10.1016/j.ccm.2006.11.004. PMID: 17338936

Prognosis

Yoshiji H, Nagoshi S, Akahane T, Asaoka Y, Ueno Y, Ogawa K, Kawaguchi T, Kurosaki M, Sakaida I, Shimizu M, Taniai M, Terai S, Nishikawa H, Hiasa Y, Hidaka H, Miwa H, Chayama K, Enomoto N, Shimosegawa T, Takehara T, Koike K
J Gastroenterol 2021 Jul;56(7):593-619. Epub 2021 Jul 7 doi: 10.1007/s00535-021-01788-x. PMID: 34231046Free PMC Article
Amin Z, Amin HZ, Tedyanto NM
Rom J Intern Med 2016 Apr-Jun;54(2):93-7. doi: 10.1515/rjim-2016-0015. PMID: 27352437
Koch DG, Fallon MB
Curr Opin Gastroenterol 2014 May;30(3):260-4. doi: 10.1097/MOG.0000000000000067. PMID: 24686436
Barua UK, Hossain AS, Roy GC, Rahman MA, Das M
Mymensingh Med J 2013 Jul;22(3):618-24. PMID: 23982562
Arguedas MR, Fallon MB
Clin Liver Dis 2005 Nov;9(4):733-46, viii. doi: 10.1016/j.cld.2005.07.006. PMID: 16207573

Clinical prediction guides

Di Giorgio A, Matarazzo L, Sonzogni A, Nicastro E, Pietrobattista A, Cananzi M, Gaio P, Sciveres M, Di Leo G, Iorio R, Marseglia A, Carioli G, Maggiore G, Guido M, D'Antiga L
Liver Int 2023 Jul;43(7):1523-1536. Epub 2023 May 8 doi: 10.1111/liv.15603. PMID: 37157951
Yoshiji H, Nagoshi S, Akahane T, Asaoka Y, Ueno Y, Ogawa K, Kawaguchi T, Kurosaki M, Sakaida I, Shimizu M, Taniai M, Terai S, Nishikawa H, Hiasa Y, Hidaka H, Miwa H, Chayama K, Enomoto N, Shimosegawa T, Takehara T, Koike K
J Gastroenterol 2021 Jul;56(7):593-619. Epub 2021 Jul 7 doi: 10.1007/s00535-021-01788-x. PMID: 34231046Free PMC Article
Lv Y, Fan D
Dig Dis Sci 2015 Jul;60(7):1914-23. Epub 2015 Mar 3 doi: 10.1007/s10620-015-3593-0. PMID: 25732713
Högman M
J Breath Res 2012 Dec;6(4):047103. Epub 2012 Jun 7 doi: 10.1088/1752-7155/6/4/047103. PMID: 22677778
Sussman NL, Kochar R, Fallon MB
Curr Opin Organ Transplant 2011 Jun;16(3):281-8. doi: 10.1097/MOT.0b013e32834664df. PMID: 21543981

Recent systematic reviews

Choe Y, Lee YJ, Lee YA, Ko JS, Shin CH
Front Endocrinol (Lausanne) 2024;15:1407686. Epub 2024 Oct 22 doi: 10.3389/fendo.2024.1407686. PMID: 39502571Free PMC Article
Wu WK, Grogan WM, Ziogas IA, Patel YJ, Bacchetta M, Alexopoulos SP
Transplant Rev (Orlando) 2022 Apr;36(2):100693. Epub 2022 Apr 6 doi: 10.1016/j.trre.2022.100693. PMID: 35413506
Aragon Pinto C, Iyer VN, Albitar HAH, Anderson A, Cajigas H, Simonetto DA, Krowka MJ, DuBrock HM, Gallo de Moraes A
Respir Med Res 2021 Nov;80:100852. Epub 2021 Jul 30 doi: 10.1016/j.resmer.2021.100852. PMID: 34418867
Sokollik C, Bandsma RH, Gana JC, van den Heuvel M, Ling SC
J Pediatr Gastroenterol Nutr 2013 Jun;56(6):675-81. doi: 10.1097/MPG.0b013e31828b3750. PMID: 23412540

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