Akira
Matsui, M.D., Ph.D.
Dept
of Paediatrics, University of Tsukuba, Ibaraki, 305-8575, Japan
In
2000, the Japanese Biliary Atresia (BA) Society published a report on a nationwide
registry of 1,084 BA patients (686 females) from 1989 to 1998. The aim of the present study was to
summarise the results of the registry and to try to find measures to improve
the long-term prognosis of this intractable disease. Epidemiology: the first-born (507), the second-born (379), low
birth weight infants (120), gestational age<36 weeks (35), chromosomal
abnormalities (2/589), abnormal meconium colour (179/643), initially
bile-pigmented stools (631/914), phototherapy (421/981), completely bottle-fed
(261/963), pathologic bleeding (94), associated anomalies (215). Initial surgery: portoenterostomy
(986), age<60 days (452: 42.4%), Types I (121), II (23), III (911),
disappearance of jaundice, cholangitis, recurrence of jaundice were not related
with age at operation. Outcome:
survive without jaundice (642), survive with jaundice (232), dead (94), survive
with transplant (208), dead after transplant (21). Conclusion: #1 perinatal
epidemiology should be more thoroughly elucidated, #2 early referral is not
satisfactory, #3 long-term prognosis is still poor in BA children and their QOL
should be related to age at initial surgery.