Early diagnosis of biliary atresia

Abstract

Biliary atresia is the most common indication for liver transplantation in the pediatric population and the most frequent liver-related cause of death in early childhood. However, in some cases, long-term survival with the native liver can be achieved by surgical reestablishment of bile flow. The procedure of choice involves removal of the fibrotic, atretic bile duct segment and construction of a drainage route via hepatoportoenterostomy (the Kasai procedure). The outcome following hepatoportoenterostomy is closely related to the patient's age at the time of the procedure. It is universally acknowledged that the Kasai procedure performed before 60 days of age can improve the likelihood of survival with the native liver and avoid liver transplant. The problem that we continually witness in our clinical practice is late referral of patients with biliary atresia, emphasizing the importance of screening for this potentially devastating disease. The lack of bile flow into the intestine leads to acholic stools—a reliable clue that obstruction is likely. Pale, nonpigmented stools appear within the first month after birth in patients with biliary atresia. Thus, the concept of using a stool color card for screening and parent education regarding the disease was initially introduced to the local population in Japan (Tochigi Prefecture) by Matsui et al in 1993 (Screening 1993;2:201-9). Their preliminary results documented the success in ensuring a prompt diagnosis and early Kasai procedure. The concept of stool color card based mass screening has subsequently been utilized in several other countries, also resulting in earlier referral of patients with biliary atresia. In this issue of The Journal, Gu et al now report the cumulative impact of the stool color card program over the 19 years that it has been used for mass screening of biliary atresia in Tochigi Prefecture. Beginning in 1994, the stool color card was distributed to all pregnant women prior to or during the postnatal one-month health check-up and the mothers returned the completed card to the attending physician. All suspected cases of biliary atresia were referred for further examination, where the diagnosis was confirmed and patients were followed from the date of Kasai procedure until liver transplant or death up to 2013. A total of 313 230 live born infants were screened; 34 patients were confirmed to have biliary atresia. The sensitivity and specificity of stool color card screening at the one-month check-up was 76.5% and 99.9%, respectively. Mean age at the time of performance of the Kasai procedure was 59.7 days. This was associated with high rates of long-term native liver survival (88%, 77%, and 49% at 5, 10, and 15 years, respectively). These data are convincing evidence that this simple, patient-empowered screening for biliary atresia should be universally adopted to improve the outcome of affected children. Article page 897▶ Stool Color Card Screening for Early Detection of Biliary Atresia and Long-Term Native Liver Survival: A 19-Year Cohort Study in JapanThe Journal of PediatricsVol. 166Issue 4PreviewTo evaluate the sensitivity and specificity of a stool color card used for a mass screening of biliary atresia conducted over 19 years. In addition, the age at Kasai procedure and the long-term probabilities of native liver survival were investigated. Full-Text PDF