Literature DB >> 19907962

Progressive familial intrahepatic cholestasis: A case with improvement in liver tests and growth following partial external biliary diversion.

Abraham Koshy1, Hariharan Ramesh, Pushpa Mahadevan, Roy Joykutty Mukkada, Vadukkoot Jose Francis, Antony Paul Chettupuzha, Pradeep George Mathew, Johny Cyriac, Philip Augustine.   

Abstract

A 2(1/2)-year-old boy presented with pruritus and jaundice of 2 weeks duration. On investigation, serum total bilirubin was 23.4 mg/dL and gamma glutamyl transpeptidase was normal. Liver biopsy was consistent with progressive familial intrahepatic cholestasis (PFIC). A partial external biliary diversion (PEBD) was done. Pruritus disappeared, growth improved and serum total bilirubin became normal, 2 months after surgery. This is the first report from India, of PFIC treated with PEBD and suggests that PEBD should be considered in patients with PFIC even if bridging fibrosis is present.

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Year:  2009        PMID： 19907962     DOI： 10.1007/s12664-009-0038-8

Source DB:  PubMed          Journal:  Indian J Gastroenterol        ISSN： 0254-8860


  8 in total

1.  The wide spectrum of multidrug resistance 3 deficiency: from neonatal cholestasis to cirrhosis of adulthood.

Authors:  E Jacquemin; J M De Vree; D Cresteil; E M Sokal; E Sturm; M Dumont; G L Scheffer; M Paul; M Burdelski; P J Bosma; O Bernard; M Hadchouel; R P Elferink
Journal:  Gastroenterology       Date:  2001-05       Impact factor: 22.682

2.  Clinical and biochemical findings in progressive familial intrahepatic cholestasis.

Authors:  P F Whitington; D K Freese; E M Alonso; S J Schwarzenberg; H L Sharp
Journal:  J Pediatr Gastroenterol Nutr       Date:  1994-02       Impact factor: 2.839

3.  Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis.

Authors:  J M de Vree; E Jacquemin; E Sturm; D Cresteil; P J Bosma; J Aten; J F Deleuze; M Desrochers; M Burdelski; O Bernard; R P Oude Elferink; M Hadchouel
Journal:  Proc Natl Acad Sci U S A       Date:  1998-01-06       Impact factor: 11.205

4.  The role of bile salt export pump mutations in progressive familial intrahepatic cholestasis type II.

Authors:  Lin Wang; Carol J Soroka; James L Boyer
Journal:  J Clin Invest       Date:  2002-10       Impact factor: 14.808

5.  Partial internal biliary diversion through a cholecystojejunocolonic anastomosis--a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report.

Authors:  Joaquim Bustorff-Silva; Lourenço Sbraggia Neto; Hugo Olímpio; Roberta Vacari de Alcantara; Erica Matsushima; Adriana Maria Alves De Tommaso; Maria Angela Bellomo Brandão; Gabriel Hessel
Journal:  J Pediatr Surg       Date:  2007-08       Impact factor: 2.545

6.  Histologic pathology of the liver in progressive familial intrahepatic cholestasis.

Authors:  E M Alonso; D C Snover; A Montag; D K Freese; P F Whitington
Journal:  J Pediatr Gastroenterol Nutr       Date:  1994-02       Impact factor: 2.839

7.  Liver transplantation in children with progressive familial intrahepatic cholestasis.

Authors:  Cornelia Englert; Enke Grabhorn; Andrea Richter; Xavier Rogiers; Martin Burdelski; Rainer Ganschow
Journal:  Transplantation       Date:  2007-11-27       Impact factor: 4.939

8.  Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families.

Authors:  Sandra S Strautnieks; Jane A Byrne; Ludmila Pawlikowska; Dita Cebecauerová; Anne Rayner; Laura Dutton; Yvonne Meier; Anthony Antoniou; Bruno Stieger; Henrik Arnell; Figen Ozçay; Hussa F Al-Hussaini; Atif F Bassas; Henkjan J Verkade; Björn Fischler; Antal Németh; Radana Kotalová; Benjamin L Shneider; Joanna Cielecka-Kuszyk; Patricia McClean; Peter F Whitington; Etienne Sokal; Milan Jirsa; Sami H Wali; Irena Jankowska; Joanna Pawłowska; Giorgina Mieli-Vergani; A S Knisely; Laura N Bull; Richard J Thompson
Journal:  Gastroenterology       Date:  2008-01-18       Impact factor: 22.682
  8 in total
  4 in total

Review 1.  Progressive familial intrahepatic cholestasis.

Authors:  Anshu Srivastava
Journal:  J Clin Exp Hepatol       Date:  2013-11-23

2.  Therapeutic interventions in progressive familial intrahepatic cholestasis: experience from a tertiary care centre in north India.

Authors:  Satvinder Kaur; Deepa Sharma; Nishant Wadhwa; Subash Gupta; Sujit Kumar Chowdhary; Anupam Sibal
Journal:  Indian J Pediatr       Date:  2011-07-19       Impact factor: 1.967

3.  A Child with Debilitating Pruritus.

Authors:  Nikhil Sonthalia; Samit S Jain; Vinay B Pawar; Vinay G Zanwar; Ravindra G Surude; Pravin M Rathi; Kshitij K Munde; Sandeep Bavdekar
Journal:  Clin Pract       Date:  2016-11-24

4.  Spectrum of genomic variations in Indian patients with progressive familial intrahepatic cholestasis.

Authors:  Anjali Sharma; Ujjal Poddar; Shikha Agnihotry; Shubha R Phadke; Surender K Yachha; Rakesh Aggarwal
Journal:  BMC Gastroenterol       Date:  2018-07-04       Impact factor: 3.067
  4 in total

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