Bile duct paucity in childhood—spectrum, profile, and outcome
Babu Lal Meena
Received: 14 February 2018 / Revised: 16 May 2018 / Accepted: 23 May 2018
Springer-Verlag GmbH Germany, part of Springer Nature 2018
We studied the etiological spectrum, clinicolaboratory and histological profile, and outcome of infants and children under 18 years
of age presenting between December 2010 and May 2016 with histological evidence of paucity of intralobular bile ducts (PILBD,
bile ducts to portal tract ratio < 0.6) Post-transplant PILBD was excluded. Of 632 pediatric liver biopsies screened, 70 had
PILBD—44 were infants. PILBD was classified histologically into destructive (n = 50) and non-destructive PILBD (n =20).
Presentations were jaundice (98%), organomegaly (94%), pale stools (50%), and pruritus (43%). Infants had more cholestasis but
less fibrosis on histology. Overall, 29 required liver transplantation (LT) for portal hypertension (n = 26), decompensation (n =
25), growth failure (n = 20), intractable pruritus (n = 5), and recurrent cholangitis (n = 2). Destructive PILBD has an odds for poor
outcome (decompensation or need for LT within 1 year) of 1.53 (95% CI = 1.15–2.04). On binary logistic regression analysis,
poor outcome was related to advanced fibrosis on liver biopsy [Exp (B) = 5.46, 95% CI = 1.56–19.04].
Conclusion: PILBD was present in 11% of pediatric liver biopsies and has a varied etiological spectrum. Destructive PILBD
has poor outcome. Need for LT is guided by the presence of advanced fibrosis.
What is Known:
• Natural history of syndromic ductal paucity (Alagille syndrome) is complex.
• Duct loss is commonly seen with late presentation of biliary atresia.
What is New:
• The study classifies the etiological spectrum of ductal paucity histologically into destructive and non-destructive.
• Destructive duct loss carries poor prognosis regardless of the etiology of liver disease with subsequent need for liver transplantation.
ARC syndrome Arthrogryposis renal dysfunction
AST Aspartate transaminase
CI Confidence interval
CPSS Congenital portosystemic shunt
GGT Gamma-glutamyl transpeptidase
INR International normalized ratio
IQR Interquartile range
PFIC Progressive familial
PILBD Paucity of intralobular bile ducts
LB Liver biopsy
LT Liver transplantation
Cholestasis refers to the consequences of obstruction of flow
of bile or its constituents at any level from hepatocyte to am-
pulla of vater. Cholestasis in infants and children results from
Communicated by Peter de Winter
* Rajeev Khanna
Babu Lal Meena
Department of Paediatric Hepatology, Institute of Liver and Biliary
Sciences, D-1, Vasant Kunj, New Delhi 110070, India
Department of Pathology, Institute of Liver and Biliary Sciences,
D-1, Vasant Kunj, New Delhi 110070, India
European Journal of Pediatrics