Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
Vol. 23, No. 1, 2013, pp. 65-70
Bioline Code: pe13011
Full paper language: English
Document type: Research Article
Document available free of charge
Iranian Journal of Pediatrics, Vol. 23, No. 1, 2013, pp. 65-70
© Copyright 2013 - Iran Journal of Pediatrics
Paucity of Intrahepatic Bile Ducts in Neonates: the First Case Series from Iran|
Zahmatkeshan, Mozhgan; Geramizadeh, Bita; Haghighat, Mahmood & Enteshari, Hajar
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.
Paucity of Interlobular Bile Ducts; Alagille Syndrome; Neonates; Cholestasis
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