Original ArticleBiliary Atresia: Clinical Profiles, Risk Factors, and Outcomes of 755 Patients Listed for Liver Transplantation
Section snippets
Patient Population
The study group comprised all 755 children < age 18 years with BA listed for their first LT and enrolled in the SPLIT registry from May 1995 to June 2003. As described previously, all of the 39 SPLIT centers had Institutional Review Board approval or a waiver for data collection and analysis.14, 15, 16 Individual informed consent was obtained from parents and/or guardians. Coded information was submitted to the SPLIT data-coordination center at the time of listing for LT. Follow-up data were
Characteristics of Patients With BA Listed for LT
Clinical and demographic details of the 755 patients with BA at the time of listing for LT are summarized in Table I (available online at www.mosby.com/jpeds). More than 70% of the patients were < 1 year of age, and 60% were female. Most (82%) were not hospitalized at the time of listing. Most had PELD scores between 10 and 20 (mean, 11.7; median, 12.1). More than 40% of patients had growth failure, although only 16% received nasogastric supplements. The mean height z-score at listing was −1.3
Discussion
These data, the largest cumulative dataset available to date describing outcomes from the time of listing for LT in patients with BA needing such therapy, reflect the current state of the art in the United States and Canada. Special considerations regarding LT for BA apply, including the predominance of infant recipients, difficulties in predicting outcomes, and timing of donor acquisition. To date, clinical profiles, likelihood of living or dying while on the LT waiting list, and donor
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SPLIT (Studies of Pediatric Liver Transplantation) is a consortium of 39 pediatric liver transplant centers in the United States and Canada (http://www.splitregistry.com). SPLIT is supported by a grant (1 U01 DK061693-01A1) from the National Institute of Diabetes and Digestive and Kidney Diseases, an unrestricted educational grant from Fujisawa Healthcare, and additional support from Wyeth-Ayerst Labs, Roche Labs, Medimmune Inc., and Novartis.