Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese biliary atresia registry
Section snippets
Materials and methods
The JBAR, consisting of an initial questionnaire and follow-up questionnaires, were sent to all major institutions (132 institutions) belonging to the Japanese Society of Pediatric Surgeons. The initial questionnaire was designed to obtain patients’ information including gestational history, perinatal history, diagnosis, treatment, and early results. Once a patient is registered, the institution is obliged each year to complete and return the follow-up questionnaire including current results of
Results of the initial questionnaire
The number of patients registered each year is shown in Table 1. Between 100 and 150 patients were registered every year. There was no clear tendency of increase or decrease in these 11 years. Because the total number of live births in Japan between 1989 and 1999 was 13,313,000, the incidence was 1 in 9,640 live births. The sex ratio showed a female to male predominance of 1:0.59 (863 girls and 507 boys). The family history of surgical hepatobiliary diseases was found in 3 patients, 2 siblings
Discussion
A nationwide surveillance like the JBAR was carried out in US-Canada (1976 to 89),1 France (1986 to 96),2 and UK-Ireland (1993 to 95).3 The JBAR is still ongoing and has become the largest one.
The incidence of biliary atresia in Japan was estimated to be 10.4 in 100,000 live births from the current study. According to the report by Chardot’s et al,2 this value is the third highest after the 32 in 100,000 live births in French Polynesia and 10.6 in 100,000 live births in Hawaii. Considering that
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