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Cholestasis
https://pubmed.ncbi.nlm.nih.gov/34016879/ Cholestasis
J Pediatr Gastroenterol Nutr. 2021 May 13.
doi: 10.1097/MPG.0000000000003153.Online ahead of print.
Mutation Analysis and Disease Features at Presentation in a Multi-Center Cohort of Children with Monogenic Cholestasis
Heather van Doren 1, Paula M Hertel, Laura N Bull, Richard J Thompson, Nathan P Goodrich, Wen Ye, John C Magee, Robert H Squires, Lee M Bass, James E Heubi, Grace E Kim, Sarangarajan Ranganathan, Kathleen B Schwarz, Molly A Bozic, Simon P Horslen, Matthew S Clifton, Yumirle P Turmelle, Frederick J Suchy, Riccardo A Superina, Kasper S Wang, Kathleen M Loomes, Binita M Kamath, Ronald J Sokol, Benjamin L Shneider, Childhood Liver Disease Research Network (ChiLDReN)
Abstract
https://www.ncbi.nlm.nih.gov/pubmed/32304554 Cholestasis
J Pediatr Gastroenterol Nutr. 2020 Apr 16. doi: 10.1097/MPG.0000000000002740. [Epub ahead of print]
Mutations in Myosin 5B (MYO5B) in Children with Early Onset Cholestasis.
Cockar I1, Foskett P2, Strautnieks S2, Clinch Y2, Fustok J2, Rahman O2, Sutton H1,3, Mtegha M4, Fessatou S5, Kontaki E5, Papaevangelou V5, Deheragoda M2, Thompson RJ1,3, Grammatikopoulos T1,3.
Abstract
OBJECTIVES:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3827866/
Amy G. Feldman, MD and Ronald J. Sokol, MD
Abstract
Presented by
Dr Avinash Khade
Moderated by
Dr S K Yachha
Panelists
Dr Malathi Sathiyasekaran
Dr Girish Gupte
Dr Ashley D'Cruz
https://www.ncbi.nlm.nih.gov/pubmed/31437071 Cholestasis
Fetal Pediatr Pathol. 2019 Aug 22:1-17. doi: 10.1080/15513815.2019.1652376. [Epub ahead of print]
Non Syndromic Paucity of Interlobular Bile Ducts in Children - A Clinicopathological Study.
Mohanty S1, Das K2, Anne Correa MM1.
Abstract
http://www.ncbi.nlm.nih.gov/pubmed/22595974
Oral Absorbable Fat-soluble Vitamin Formulation in Pediatric Patients With Cholestasis
Journal of Pediatric Gastroenterology and Nutrition, 11/13/2012
Shen YM et al. – High rates of Fat–soluble vitamin (FSV) deficiency were found in pediatric patients with chronic cholestasis under present follow–up. Oral, absorbable, fat–soluble vitamin formulation (OAFSV) supplementation is safe and potentially effective in pediatric patients with cholestasis.
Methods
http://www.ncbi.nlm.nih.gov/pubmed/27429423
Thébaut A, Nemeth A, Le Mouhaër J, Scheenstra R, Baumann U, Koot B, Gottrand F, Houwen R, Monard L, de Micheaux SL, Habes D, Jacquemin E. J Pediatr Gastroenterol Nutr. 2016 Jul 16.
Abstract
BACKGROUND/AIMS:
d-alpha-tocopheryl polyethylene glycol 1000 succinate (Tocofersolan, Vedrop), has been developed in Europe to provide an orally bioavailable source of vitamin E in children with cholestasis. The aim was to analyze the safety/efficacy of Vedrop in a large group of children with chronic cholestasis.
METHODS:
https://www.ncbi.nlm.nih.gov/pubmed/27429423
Thébaut A, Nemeth A, Le Mouhaër J, Scheenstra R, Baumann U, Koot B, Gottrand F, Houwen R, Monard L, de Micheaux SL, Habes D, Jacquemin E. J Pediatr Gastroenterol Nutr. 2016 Dec;63(6):610-615.
Abstract
OBJECTIVES:
D-Alpha-tocopheryl polyethylene glycol 1000 succinate (Tocofersolan, Vedrop), has been developed in Europe to provide an orally bioavailable source of vitamin E in children with cholestasis. The aim was to analyze the safety/efficacy of Vedrop in a large group of children with chronic cholestasis.
METHODS:
https://www.ncbi.nlm.nih.gov/pubmed/31863603 Cholestasis
Hepatology. 2019 Dec 21. doi: 10.1002/hep.31087. [Epub ahead of print]
OSTα deficiency: A disorder with cholestasis, liver fibrosis and congenital diarrhea.
Gao E1, Cheema H2, Waheed N2, Mushtaq I2, Erden N1, Nelson-Williams C3, Jain D1,4, Soroka CJ1, Boyer JL1, Khalil Y5, Clayton PT5, Mistry PK1,6, Lifton RP3,7,8, Vilarinho S1,4.
Abstract
SLC51A encodes the alpha subunit of the heteromeric organic solute transporter alpha-beta (OSTα-OSTβ), an important contributor to intestinal bile acid (BA) reabsorption in the enterohepatic circulation(1,2). Here, we identified the first case of OSTα deficiency in a child with unexplained elevated liver transaminases, cholestasis and congenital diarrhea.
https://pubmed.ncbi.nlm.nih.gov/34310436/ Cholestasis
J Pediatr Gastroenterol Nutr. 2021 Jul 23.
doi: 10.1097/MPG.0000000000003248.Online ahead of print.
Presentation and Outcomes of Infants with Idiopathic Cholestasis: A Multi-Center Prospective Study
Paula M Hertel 1, Kieran Hawthorne, Sehee Kim, Milton J Finegold, Benjamin L Shneider, James E Squires, Nitika A Gupta, Laura N Bull, Karen F Murray, Nanda Kerkar, Vicky L Ng, Jean P Molleston, Jorge A Bezerra, Kathleen M Loomes, Sarah A Taylor, Kathleen B Schwarz, Yumirle P Turmelle, Philip Rosenthal, John C Magee, Ronald J Sokol, Childhood Liver Disease Research Network (ChiLDReN)
Abstract
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