Paucity of Intrahepatic Bile Ducts in Neonates: the First Case Series from Iran

Mozhgan  Zahmatkeshan; Bita Geramizadeh; Mahmood Haghighat; Hajar Enteshari

IJ Pediatrics

The Scientific Journal of Growth & Development Research Center

Outlines

Abstract
Fulltext
References
Copyright

Paucity of Intrahepatic Bile Ducts in Neonates: the First Case Series from Iran

Author(s):

Mozhgan Zahmatkeshan^1,*,

Bita Geramizadeh²,

Mahmood Haghighat³,

Hajar Enteshari⁴

IJ Pediatrics:Vol. 23, issue 1; 65-70

Published online:Jan 01, 2013

Article type:Research Article

Received:May 10, 2012

Accepted:Sep 03, 2012

How to Cite:Mozhgan ZahmatkeshanBita GeramizadehMahmood HaghighatHajar EnteshariPaucity of Intrahepatic Bile Ducts in Neonates: the First Case Series from Iran.Iran J Pediatr.23(1):65-70.

Abstract

Objective: Paucity of interlobular bile ducts (PIBD), defined as absence or marked decrease in the number of interlobular bile ducts, is one of the causes of neonatal cholestasis. Treatment includes treating the intractable pruritus caused by persistent cholestasis. PIBD can be part of a familial syndrome of cholestasis named Alagille syndrome (AGS). We report clinical status of a case series of Iranian patients with PIBD.
Methods: In this retrospective study, patients with cholestasis admitted to the pediatric gastroenterology ward in a referral hospital in Shiraz from January 2006 to January 2010 and underwent liver biopsy were evaluated. Clinical and paraclinical status of children with the pathologic diagnosis of PIBD was assessed.
Findings: Disease was presented in all jaundiced patients with aged in average 3 days at presentation. Seven patients had the criteria of AGS. Despite medical treatment, cholestasis was not controlled in 6 (28.6%) patients. Liver transplantation led to the survival of 5 patients while the other patient who did not undergo liver transplantation died at 2 months of age. One patient underwent peritoneal dialysis due to renal insufficiency and died at 9 months of age. After 1-5 years of follow-up, the mortality rate was 9.5%.
Conclusion: In patients with intractable cholestasis, only patients that underwent liver transplantation survived. Thus, the most important criterion for liver transplantation in neonatal PIBD is intractable cholestasis. This is the first report that shows AGS can result in neonatal-onset renal insufficiency.

Keywords

Paucity of Interlobular Bile Ducts

Alagille Syndrome

Neonates

Cholestasis

Fulltext

Click here to download PDF.

References

1.
References are available on the PDF.

comments