학술논문

The need for early Kasai portoenterostomy: a Western Pediatric Surgery Research Consortium study
Document Type
article
Source
Pediatric Surgery International. 38(2)
Subject
Liver Disease
Pediatric
Clinical Research
Prevention
Perinatal Period - Conditions Originating in Perinatal Period
Rare Diseases
Digestive Diseases
6.4 Surgery
Evaluation of treatments and therapeutic interventions
Oral and gastrointestinal
Good Health and Well Being
Biliary Atresia
Humans
Infant
Liver Transplantation
Portoenterostomy
Hepatic
Retrospective Studies
Treatment Outcome
Transplant-free survival
Liver transplant
Biliary atresia
Kasai portoenterostomy
Paediatrics and Reproductive Medicine
Pediatrics
Language
Abstract
PurposeThe purpose of this study was to investigate factors impacting transplant-free survival among infants with biliary atresia.MethodsA multi-institutional, retrospective cohort study was performed at nine tertiary-level children's hospitals in the United States. Infants who underwent Kasai portoenterostomy (KP) from January 2009 to May 2017 were identified. Clinical characteristics included age at time of KP, steroid use, surgical approach, liver pathology, and surgeon experience. Likelihood of transplant-free survival (TFS) was evaluated using logistic regression, adjusting for patient and surgeon-level factors. Secondary outcomes at 1 year included readmission, cholangitis, reoperation, mortality, and biliary clearance.ResultsOverall, 223 infants underwent KP, and 91 (40.8%) survived with their native liver. Mean age at surgery was 63.9 days (± 24.7 days). At 1 year, 78.5% experienced readmission, 56.9% developed cholangitis, 3.8% had a surgical revision, and 5 died. Biliary clearance at 3 months was achieved in 76.6%. Controlling for patient and surgeon-level factors, each additional day of age toward operation was associated with a 2% decrease in likelihood of TFS (OR 0.98, 95% CI 0.97-0.99).ConclusionEarlier surgical intervention by Kasai portoenterostomy at tertiary-level centers significantly increases likelihood for TFS. Policy-level interventions to facilitate early screening and surgical referral for infants with biliary atresia are warranted to improve outcomes.