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Developmental Assessment of Infants With Biliary Atresia: Differences Between Boys and Girls

Caudle, Susan E.*; Katzenstein, Jennifer M.; Karpen, Saul; McLin, Valérie§

Journal of Pediatric Gastroenterology and Nutrition: October 2012 - Volume 55 - Issue 4 - p 384–389
doi: 10.1097/MPG.0b013e318259ed20
Hepatology and Nutrition

Objective: The aim of the present study was to investigate whether male and female infants with biliary atresia (BA) differ cognitively and to confirm previously documented developmental lags in infants with BA before liver transplantation.

Methods: With the Mullen Scales of Early Learning, we examined 21 female and 12 male infants (ages 3–20 months) with BA, comparing scores across indices by sex and correlating Mullen Scales of Early Learning scores with standard clinical and biochemical parameters.

Results: Overall, both boys and girls were found to be vulnerable to developmental lags in the areas of expressive language (EL) and gross motor skills. In comparison with their male peers, girls were found to be weaker in the area of visual reception skills (P = 0.05) with a trend found for EL (P = 0.08). Girls were also found to have higher C-bilirubin levels and to be of shorter length. Growth parameters were found to be correlated with EL scores. International normalized ratio was found to be correlated with gross motor performance and with a trend also noted for fine motor skills. Age at Kasai predicted receptive language skills.

Conclusions: As has been shown, infants with BA appear to be vulnerable to developmental lags before transplantation. In particular, female infants appear to be vulnerable to cognitive and skill delays in comparison with their male peers. C-bilirubin levels may play a role in this increased vulnerability for females.

*Section of Psychology, Psychology Service, Texas Children's Hospital, Baylor College of Medicine, Houston, TX

Department of Neurology, Section of Neuropsychology, Indiana University School of Medicine, Indiana University Health, Indianapolis, IN

Emory School of Medicine, Atlanta, GA

§Unité de Gastroentérologie Pédiatrique, Hôpital des Enfants, Geneva, Switzerland.

Address correspondence and reprint requests to Susan E. Caudle, PhD, Psychology Service, Texas Children's Hospital, 6701 Fannin St, CC 1630.30, Houston, TX 77030 (e-mail:

Received 14 December, 2011

Accepted 11 April, 2012

The authors report no conflicts of interest.

Copyright 2012 by ESPGHAN and NASPGHAN