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Abnormality of hepatobiliary system physiology

MedGen UID:
1368531
•Concept ID:
C4476621
•
Finding
HPO: HP:0025155

Definition

A functional anomaly of the hepatobiliary system [from HPO]

Professional guidelines

PubMed

Consensus clinical management guidelines for Alström syndrome.
Tahani N, Maffei P, Dollfus H, Paisey R, Valverde D, Milan G, Han JC, Favaretto F, Madathil SC, Dawson C, Armstrong MJ, Warfield AT, Düzenli S, Francomano CA, Gunay-Aygun M, Dassie F, Marion V, Valenti M, Leeson-Beevers K, Chivers A, Steeds R, Barrett T, Geberhiwot T
Orphanet J Rare Dis 2020 Sep 21;15(1):253. doi: 10.1186/s13023-020-01468-8. PMID: 32958032Free PMC Article
Management options for cholestatic liver disease in children.
Catzola A, Vajro P
Expert Rev Gastroenterol Hepatol 2017 Nov;11(11):1019-1030. Epub 2017 Jul 28 doi: 10.1080/17474124.2017.1359538. PMID: 28745070
The pathophysiology, evaluation and management of motility disorders of the biliary tract.
Summers RW, Johlin FC
Gastroenterol Clin North Am 1989 Jun;18(2):425-35. PMID: 2668177

Recent clinical studies

Etiology

The Spectrum of Disease Severity in Cirrhosis and Its Implications for Hemostasis.
Stotts MJ, Lisman T, Intagliata NM
Semin Thromb Hemost 2020 Sep;46(6):716-723. Epub 2020 Aug 20 doi: 10.1055/s-0040-1715449. PMID: 32820482
The hepatobiliary complications of malnutrition and nutritional support in adults.
McNeice A, Scott R, Rafferty GP, Cash WJ, Turner GB
Ir J Med Sci 2019 Feb;188(1):109-117. Epub 2018 May 31 doi: 10.1007/s11845-018-1836-8. PMID: 29850987
Management options for cholestatic liver disease in children.
Catzola A, Vajro P
Expert Rev Gastroenterol Hepatol 2017 Nov;11(11):1019-1030. Epub 2017 Jul 28 doi: 10.1080/17474124.2017.1359538. PMID: 28745070
Coagulation in hepatobiliary disease.
Kavanagh C, Shaw S, Webster CR
J Vet Emerg Crit Care (San Antonio) 2011 Dec;21(6):589-604. doi: 10.1111/j.1476-4431.2011.00691.x. PMID: 22316251
Cholangiocyte biology.
Tietz PS, LaRusso NF
Curr Opin Gastroenterol 2005 May;21(3):337-43. doi: 10.1097/01.mog.0000158111.21345.c2. PMID: 15818155

Diagnosis

Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes.
Putman MS, Norris AW, Hull RL, Rickels MR, Sussel L, Blackman SM, Chan CL, Ode KL, Daley T, Stecenko AA, Moran A, Helmick MJ, Cray S, Alvarez JA, Stallings VA, Tuggle KL, Clancy JP, Eggerman TL, Engelhardt JF, Kelly A
Diabetes Care 2023 Jun 1;46(6):1112-1123. doi: 10.2337/dc23-0380. PMID: 37125948Free PMC Article
Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes.
Putman MS, Norris AW, Hull RL, Rickels MR, Sussel L, Blackman SM, Chan CL, Ode KL, Daley T, Stecenko AA, Moran A, Helmick MJ, Cray S, Alvarez JA, Stallings VA, Tuggle KL, Clancy JP, Eggerman TL, Engelhardt JF, Kelly A
Diabetes 2023 Jun 1;72(6):677-689. doi: 10.2337/db22-0949. PMID: 37125945Free PMC Article
Management options for cholestatic liver disease in children.
Catzola A, Vajro P
Expert Rev Gastroenterol Hepatol 2017 Nov;11(11):1019-1030. Epub 2017 Jul 28 doi: 10.1080/17474124.2017.1359538. PMID: 28745070
Overview of neonatal lupus.
Johnson B
J Pediatr Health Care 2014 Jul-Aug;28(4):331-41. Epub 2013 Oct 5 doi: 10.1016/j.pedhc.2013.07.015. PMID: 24100008
Coagulation in hepatobiliary disease.
Kavanagh C, Shaw S, Webster CR
J Vet Emerg Crit Care (San Antonio) 2011 Dec;21(6):589-604. doi: 10.1111/j.1476-4431.2011.00691.x. PMID: 22316251

Therapy

Potential therapeutic role of melatonin in hepatobiliary diseases: current evidence and clinical observations.
Gonciarz M, Lombard I, Konecki L, Gietka P, Pajdo R, Brzozowski T
J Physiol Pharmacol 2023 Apr;74(2) Epub 2023 Jul 10 doi: 10.26402/jpp.2023.2.01. PMID: 37453089
The immunological roles in acute-on-chronic liver failure: An update.
Chen P, Wang YY, Chen C, Guan J, Zhu HH, Chen Z
Hepatobiliary Pancreat Dis Int 2019 Oct;18(5):403-411. Epub 2019 Jul 5 doi: 10.1016/j.hbpd.2019.07.003. PMID: 31303562
The hepatobiliary complications of malnutrition and nutritional support in adults.
McNeice A, Scott R, Rafferty GP, Cash WJ, Turner GB
Ir J Med Sci 2019 Feb;188(1):109-117. Epub 2018 May 31 doi: 10.1007/s11845-018-1836-8. PMID: 29850987
Management options for cholestatic liver disease in children.
Catzola A, Vajro P
Expert Rev Gastroenterol Hepatol 2017 Nov;11(11):1019-1030. Epub 2017 Jul 28 doi: 10.1080/17474124.2017.1359538. PMID: 28745070
Advances in primary sclerosing cholangitis.
Horsley-Silva JL, Carey EJ, Lindor KD
Lancet Gastroenterol Hepatol 2016 Sep;1(1):68-77. Epub 2016 Aug 10 doi: 10.1016/S2468-1253(16)30010-3. PMID: 28404115

Prognosis

Potential therapeutic role of melatonin in hepatobiliary diseases: current evidence and clinical observations.
Gonciarz M, Lombard I, Konecki L, Gietka P, Pajdo R, Brzozowski T
J Physiol Pharmacol 2023 Apr;74(2) Epub 2023 Jul 10 doi: 10.26402/jpp.2023.2.01. PMID: 37453089
The Spectrum of Disease Severity in Cirrhosis and Its Implications for Hemostasis.
Stotts MJ, Lisman T, Intagliata NM
Semin Thromb Hemost 2020 Sep;46(6):716-723. Epub 2020 Aug 20 doi: 10.1055/s-0040-1715449. PMID: 32820482
Advances in primary sclerosing cholangitis.
Horsley-Silva JL, Carey EJ, Lindor KD
Lancet Gastroenterol Hepatol 2016 Sep;1(1):68-77. Epub 2016 Aug 10 doi: 10.1016/S2468-1253(16)30010-3. PMID: 28404115
Coagulation in hepatobiliary disease.
Kavanagh C, Shaw S, Webster CR
J Vet Emerg Crit Care (San Antonio) 2011 Dec;21(6):589-604. doi: 10.1111/j.1476-4431.2011.00691.x. PMID: 22316251
Pathogenesis of gallstones.
Carey MC
Am J Surg 1993 Apr;165(4):410-9. doi: 10.1016/s0002-9610(05)80932-8. PMID: 8480873

Clinical prediction guides

Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes.
Putman MS, Norris AW, Hull RL, Rickels MR, Sussel L, Blackman SM, Chan CL, Ode KL, Daley T, Stecenko AA, Moran A, Helmick MJ, Cray S, Alvarez JA, Stallings VA, Tuggle KL, Clancy JP, Eggerman TL, Engelhardt JF, Kelly A
Diabetes Care 2023 Jun 1;46(6):1112-1123. doi: 10.2337/dc23-0380. PMID: 37125948Free PMC Article
Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes.
Putman MS, Norris AW, Hull RL, Rickels MR, Sussel L, Blackman SM, Chan CL, Ode KL, Daley T, Stecenko AA, Moran A, Helmick MJ, Cray S, Alvarez JA, Stallings VA, Tuggle KL, Clancy JP, Eggerman TL, Engelhardt JF, Kelly A
Diabetes 2023 Jun 1;72(6):677-689. doi: 10.2337/db22-0949. PMID: 37125945Free PMC Article
Clinical profiles and diagnostic challenges in 1158 children with rare hepatobiliary disorders.
Dong Y, Wang J, Zhu S, Zheng H, Wang C, Zhao P
Pediatr Res 2021 Jan;89(1):238-245. Epub 2020 Apr 12 doi: 10.1038/s41390-020-0888-4. PMID: 32289814
The Spectrum of Disease Severity in Cirrhosis and Its Implications for Hemostasis.
Stotts MJ, Lisman T, Intagliata NM
Semin Thromb Hemost 2020 Sep;46(6):716-723. Epub 2020 Aug 20 doi: 10.1055/s-0040-1715449. PMID: 32820482
The immunological roles in acute-on-chronic liver failure: An update.
Chen P, Wang YY, Chen C, Guan J, Zhu HH, Chen Z
Hepatobiliary Pancreat Dis Int 2019 Oct;18(5):403-411. Epub 2019 Jul 5 doi: 10.1016/j.hbpd.2019.07.003. PMID: 31303562

Recent systematic reviews

Consensus clinical management guidelines for Alström syndrome.
Tahani N, Maffei P, Dollfus H, Paisey R, Valverde D, Milan G, Han JC, Favaretto F, Madathil SC, Dawson C, Armstrong MJ, Warfield AT, Düzenli S, Francomano CA, Gunay-Aygun M, Dassie F, Marion V, Valenti M, Leeson-Beevers K, Chivers A, Steeds R, Barrett T, Geberhiwot T
Orphanet J Rare Dis 2020 Sep 21;15(1):253. doi: 10.1186/s13023-020-01468-8. PMID: 32958032Free PMC Article
Neurodevelopmental Outcomes in Children With Liver Diseases: a Systematic Review.
Rodijk LH, den Heijer AE, Hulscher JBF, Verkade HJ, de Kleine RHJ, Bruggink JLM
J Pediatr Gastroenterol Nutr 2018 Aug;67(2):157-168. doi: 10.1097/MPG.0000000000001981. PMID: 29601439

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