https://www.ncbi.nlm.nih.gov/pubmed/30244988 Biliary atresia

J Pediatr. 2018 Sep 21. pii: S0022-3476(18)30895-3. doi: 10.1016/j.jpeds.2018.07.002. [Epub ahead of print]
Impact of Steroid Therapy on Early Growth in Infants with Biliary Atresia: The Multicenter Steroids in Biliary Atresia Randomized Trial.
Alonso EM1, Ye W2, Hawthorne K3, Venkat V4, Loomes KM5, Mack CL6, Hertel PM7, Karpen SJ8, Kerkar N9, Molleston JP10, Murray KF11, Romero R12, Rosenthal P13, Schwarz KB14, Shneider BL15, Suchy FJ16, Turmelle YP17, Wang KS18, Sherker AH19, Sokol RJ6, Bezerra JA20, Magee JC21; ChiLDReNNetwork.

Abstract

OBJECTIVE:
To investigate the impact of corticosteroid therapy on the growth of participants in the Steroids in Biliary Atresia Randomized Trial (START) conducted through the Childhood Liver Disease Research Network. The primary analysis in START indicated that steroids did not have a beneficial effect on drainage in a cohort of infants with biliary atresia. We hypothesized that steroids would have a detrimental effect on growth in these infants.

STUDY DESIGN:
A total of 140 infants were enrolled in START, with 70 randomized to each treatment arm: steroid and placebo. Length, weight, and head circumference were obtained at baseline and follow-up visits to 24 months of age.

RESULTS:
Patients treated with steroids had significantly lower length and head circumference z scores during the first 3 months post-hepatoportoenterostomy (HPE), and significantly lower weight until 12 months. Growth trajectories in the steroid and placebo arms differed significantly for length (P < .0001), weight (P = .009), and head circumference (P < .0001) with the largest impact noted for those with successful HPE. Growth trajectory for head circumference was significantly lower in patients treated with steroids irrespective of HPE status, but recovered during the second 6 months of life.

CONCLUSIONS:
Steroid therapy following HPE in patients with biliary atresia is associated with impaired length, weight, and head circumference growth trajectories for at least 6 months post-HPE, especially impacting infants with successful bile drainage.