title: Paucity of intrahepatic bile ducts in neonates: the first case series from iran.
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.
  
  
  RESULTS
  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%.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Zahmatkeshan
authors.initials: Z
authors.lastname: Mozhgan
authors.forename: Geramizadeh
authors.initials: G
authors.lastname: Bita
authors.forename: Haghighat
authors.initials: H
authors.lastname: Mahmood
authors.forename: Enteshari
authors.initials: E
authors.lastname: Hajar
keywords: Alagille Syndrome; Cholestasis; Neonates; Paucity of Interlobular Bile Ducts
references.citation: Am J Med Genet. 2002 Oct 1;112(2):163-70
references.pmid: 12244550
references.citation: Semin Gastrointest Dis. 2003 Oct;14(4):156-64
references.pmid: 14719766
references.citation: J Pediatr. 1975 Jan;86(1):63-71
references.pmid: 803282
references.citation: Can J Gastroenterol. 2007 Nov;21(11):743-51
references.pmid: 18026579
references.citation: Gastroenterol Clin North Am. 2003 Sep;32(3):857-75, vi
references.pmid: 14562578
references.citation: J Pediatr. 1995 Aug;127(2):220-4
references.pmid: 7636645
references.citation: Eur J Pediatr. 2011 Mar;170(3):279-84
references.pmid: 21249394
references.citation: J Pediatr Gastroenterol Nutr. 2011 Jan;52(1):84-9
references.pmid: 21119543
references.citation: J Pediatr Gastroenterol Nutr. 2010 Jun;50(6):580-6
references.pmid: 20479679
references.citation: Curr Gastroenterol Rep. 1999 Jun;1(3):223-30
references.pmid: 10980954
references.citation: J Hepatobiliary Pancreat Surg. 2001;8(4):303-15
references.pmid: 11521175
references.citation: J Pediatr Gastroenterol Nutr. 2002;35 Suppl 1:S17-23
references.pmid: 12151816
references.citation: Nat Genet. 1997 Jul;16(3):235-42
references.pmid: 9207787
references.citation: Clin Invest Med. 1996 Oct;19(5):325-30
references.pmid: 8889270
references.citation: Curr Opin Pediatr. 1999 Dec;11(6):558-64
references.pmid: 10590916
references.citation: Clin Liver Dis. 2000 Nov;4(4):765-78
references.pmid: 11232356
references.citation: J Med Genet. 1997 Feb;34(2):152-7
references.pmid: 9039994
references.citation: Hepatology. 1999 Mar;29(3):822-9
references.pmid: 10051485
references.citation: Arch Dis Child. 1996 Mar;74(3):232-5
references.pmid: 8787429
references.citation: Genomics. 1997 Aug 1;43(3):376-9
references.pmid: 9268641
references.citation: Am J Med Genet A. 2004 Jan 15;124A(2):220-1; reply 222-3
references.pmid: 14699626
references.citation: Adv Clin Path. 1998 Apr;2(2):87-99
references.pmid: 10358336
references.citation: Curr Opin Genet Dev. 2002 Jun;12(3):336-42
references.pmid: 12076678
references.citation: Pediatr Rev. 2004 Nov;25(11):388-96
references.pmid: 15520084
references.citation: Gastroenterology. 2003 Oct;125(4):1227-34
references.pmid: 14517804
references.citation: Neth J Med. 2010 Jan;68(1):38-9
references.pmid: 20103821
references.citation: J Pediatr Gastroenterol Nutr. 2010 Jan;50(1):11-5
references.pmid: 19949348
references.citation: Curr Gastroenterol Rep. 2011 Apr;13(2):193-201
references.pmid: 21225381