Literature DB >> 22614108

Pancreatic insufficiency is not a prevalent problem in Alagille syndrome.

Binita M Kamath1, David A Piccoli, John C Magee, Ronald J Sokol.   

Abstract

Alagille syndrome (ALGS) is an inherited multisystem disorder in which pancreatic insufficiency (PI) has been regarded a minor but important clinical manifestation. As part of a multicenter prospective study, 42 patients with ALGS underwent fecal elastase (FE) measurement to screen for exocrine PI. FE measurements were normal (>200  μg/g) in 40 (95%) and indeterminate (100-200  μg/g) in 2 (5%). As FE is the most reliable screen for PI, these data suggest that PI is not a prevalent problem in ALGS.

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Year:  2012        PMID： 22614108      PMCID： PMC3666161          DOI： 10.1097/MPG.0b013e31825eff61

Source DB:  PubMed          Journal:  J Pediatr Gastroenterol Nutr        ISSN： 0277-2116            Impact factor:   2.839


  10 in total

1.  Alagille syndrome is caused by mutations in human Jagged1, which encodes a ligand for Notch1.

Authors:  L Li; I D Krantz; Y Deng; A Genin; A B Banta; C C Collins; M Qi; B J Trask; W L Kuo; J Cochran; T Costa; M E Pierpont; E B Rand; D A Piccoli; L Hood; N B Spinner
Journal:  Nat Genet       Date:  1997-07       Impact factor: 38.330

2.  Towards the ideal quantitative pancreatic function test: analysis of test variables that influence validity.

Authors:  Susanne Schibli; Mary Corey; Kevin J Gaskin; Lynda Ellis; Peter R Durie
Journal:  Clin Gastroenterol Hepatol       Date:  2006-01       Impact factor: 11.382

3.  NOTCH2 mutations in Alagille syndrome.

Authors:  Binita Maya Kamath; Robert C Bauer; Kathleen M Loomes; Grace Chao; Jennifer Gerfen; Anne Hutchinson; Winita Hardikar; Gideon Hirschfield; Paloma Jara; Ian D Krantz; Pablo Lapunzina; Laura Leonard; Simon Ling; Vicky Lee Ng; Phuc Le Hoang; David A Piccoli; Nancy Bettina Spinner
Journal:  J Med Genet       Date:  2011-12-29       Impact factor: 6.318

4.  NOTCH2 mutations cause Alagille syndrome, a heterogeneous disorder of the notch signaling pathway.

Authors:  Ryan McDaniell; Daniel M Warthen; Pedro A Sanchez-Lara; Athma Pai; Ian D Krantz; David A Piccoli; Nancy B Spinner
Journal:  Am J Hum Genet       Date:  2006-05-10       Impact factor: 11.025

5.  Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur.

Authors:  D Alagille; M Odièvre; M Gautier; J P Dommergues
Journal:  J Pediatr       Date:  1975-01       Impact factor: 4.406

6.  How useful is fecal pancreatic elastase 1 as a marker of exocrine pancreatic disease?

Authors:  Satti Beharry; Lynda Ellis; Mary Corey; Margaret Marcon; Peter Durie
Journal:  J Pediatr       Date:  2002-07       Impact factor: 4.406

7.  Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases.

Authors:  D Alagille; A Estrada; M Hadchouel; M Gautier; M Odièvre; J P Dommergues
Journal:  J Pediatr       Date:  1987-02       Impact factor: 4.406

8.  Exocrine pancreatic insufficiency in syndromic paucity of interlobular bile ducts.

Authors:  S K Chong; J Lindridge; C Moniz; A P Mowat
Journal:  J Pediatr Gastroenterol Nutr       Date:  1989-11       Impact factor: 2.839

9.  Jagged1 is a competitive inhibitor of Notch signaling in the embryonic pancreas.

Authors:  Maria L Golson; John Le Lay; Nan Gao; Nuria Brämswig; Kathleen M Loomes; Rebecca Oakey; Catherine L May; Peter White; Klaus H Kaestner
Journal:  Mech Dev       Date:  2009-06-06       Impact factor: 1.882

10.  Rethinking growth failure in Alagille syndrome: the role of dietary intake and steatorrhea.

Authors:  Alisha J Rovner; Joan I Schall; Abbas F Jawad; David A Piccoli; Virginia A Stallings; Andrew E Mulberg; Babette S Zemel
Journal:  J Pediatr Gastroenterol Nutr       Date:  2002-10       Impact factor: 2.839
  10 in total
  4 in total

1.  Baseline Analysis of a Young α-1-Antitrypsin Deficiency Liver Disease Cohort Reveals Frequent Portal Hypertension.

Authors:  Jeffrey H Teckman; Philip Rosenthal; Robert Abel; Lee M Bass; Sonia Michail; Karen F Murray; David A Rudnick; Daniel W Thomas; Cathie Spino; Ronen Arnon; Paula M Hertel; James Heubi; Binita M Kamath; Wikrom Karnsakul; Kathleen M Loomes; John C Magee; Jean P Molleston; Rene Romero; Benjamin L Shneider; Averell H Sherker; Ronald J Sokol
Journal:  J Pediatr Gastroenterol Nutr       Date:  2015-07       Impact factor: 2.839

2.  Clinical Features and Genetic Analysis of Pediatric Patients with Alagille Syndrome Presenting Initially with Liver Function Abnormalities.

Authors:  Yan Liu; Hong Wang; Chen Dong; Jie-Xiong Feng; Zhi-Hua Huang
Journal:  Curr Med Sci       Date:  2018-04-30

3.  Longitudinal Outcomes in Young Patients with Alpha-1-Antitrypsin Deficiency with Native Liver Reveal that Neonatal Cholestasis is a Poor Predictor of Future Portal Hypertension.

Authors:  Jeffrey Teckman; Philip Rosenthal; Kieran Hawthorne; Cathie Spino; Lee M Bass; Karen F Murray; Nanda Kerkar; John C Magee; Saul Karpen; James E Heubi; Jean P Molleston; Robert H Squires; Binita M Kamath; Stephen L Guthery; Kathleen M Loomes; Averell H Sherker; Ronald J Sokol
Journal:  J Pediatr       Date:  2020-07-11       Impact factor: 6.314

4.  Exocrine pancreatic function in children with Alagille syndrome.

Authors:  Dorota Gliwicz; Irena Jankowska; Aldona Wierzbicka; Anna Miśkiewicz-Chotnicka; Aleksandra Lisowska; Jarosław Walkowiak
Journal:  Sci Rep       Date:  2016-10-17       Impact factor: 4.379
  4 in total

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