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A population-based study on the incidence and possible pre- and perinatal etiologic risk factors of biliary atresia
Björn Fischler1, Bengt Haglund, Anders Hjern
1Department of Pediatrics, Huddinge University Hospital, Karolinska Institutet, Stockholm, Sweden.
Insights
Biliary atresia affects 1 in 14,000 Swedish newborns. Key risk factors include advanced maternal age, high parity, prematurity, and low birth weight, suggesting maternal vulnerability and potential viral transmission.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Epidemiology
Background:
- Biliary atresia (BA) is a rare but serious neonatal liver disease.
- Understanding its incidence and risk factors is crucial for early diagnosis and intervention.
Purpose of the Study:
- To determine the incidence, identify etiologic risk factors, and analyze the outcome of biliary atresia in Sweden.
- To investigate the association between major heart malformations and biliary atresia.
Main Methods:
- A nationwide register study of 1,204,791 children born in Sweden (1987-1997).
- Biliary atresia cases were identified using national health databases.
- Follow-up was conducted until 2 years of age.
Main Results:
- The incidence of biliary atresia was 1 in 14,000 live births.
- Major heart malformations were present in 15% of BA cases.
- Independent risk factors identified: advanced maternal age (OR=3.0), high parity (OR=2.2), prematurity (OR=2.9), and low birth weight for gestational age (OR=4.7).
- No significant differences in outcome were observed based on heart malformations or identified risk factors.
Conclusions:
- The incidence of biliary atresia in Sweden is comparable to other European countries.
- Identified risk factors suggest potential maternal vulnerability and the role of maternal-fetal/neonatal viral infections.
Objective:
To study the incidence, etiologic risk factors, and outcome of biliary atresia.
Study Design:
Register study using Swedish national health databases. The study population consisted of 1,204,791 children, corresponding to 99% of the entire cohort of Swedish children born between 1987 and 1997, with an end point of follow-up at 2 years of age. Cases with biliary atresia with and without major heart malformations were identified with indicators from various national health databases.
Results:
Eighty-five cases with biliary atresia were identified, the incidence being 1 in 14,000. A major heart malformation was found in 13 (15%) cases. In a multivariate analysis, 4 independent risk factors were identified: high maternal age (odds ratio [OR] = 3.0), parity of at least 4 (OR = 2.2), prematurity (OR = 2.9), and low birth weight for gestational age (OR = 4.7). No significant differences were found in the distribution of birth months. The outcome did not differ between the two groups with and without major heart malformations, nor with respect to any of the risk factors.
Conclusions:
The Swedish incidence of biliary atresia is similar to that found in other European countries. The identified risk factors may suggest the existence of a maternal vulnerability and the importance of viral infections transmitted from mother to fetus/neonate.
