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. 2022 Jul:246:89-94.e2.
doi: 10.1016/j.jpeds.2022.03.038. Epub 2022 Mar 30.

The Epidemiology of Biliary Atresia: Exploring the Role of Developmental Factors on Birth Prevalence

Laurel Cavallo  1 , Erin M Kovar  2 , Amal Aqul  3 , Lucille McLoughlin  4 , Naveen K Mittal  5 , Norberto Rodriguez-Baez  3 , Benjamin L Shneider  1 , Robert J Zwiener  6 , Tiffany M Chambers  2 , Peter H Langlois  7 , Mark A Canfield  8 , A J Agopian  9 , Philip J Lupo  2 , Sanjiv Harpavat  10
Affiliations

The Epidemiology of Biliary Atresia: Exploring the Role of Developmental Factors on Birth Prevalence

Laurel Cavallo et al. J Pediatr. 2022 Jul.

Abstract

Objective: To identify key epidemiologic factors relevant to fetal development that are associated with biliary atresia.

Study design: This population-based registry study examined infants born in Texas between 1999 and 2014. Epidemiologic data relevant to fetal development were compared between cases of biliary atresia identified in the Texas Birth Defects Registry (n = 305) vs all live births (n = 4 689 920), and Poisson regression was used to calculate prevalence ratios (PRs) and 95% CIs.

Results: The prevalence of biliary atresia over the study period was 0.65 per 10 000 live births. Biliary atresia was positively associated with female sex (adjusted PR, 1.68; 95% CI, 1.33-2.12), delivery before 32-37 weeks of gestation (adjusted PR, 1.64; 95% CI, 1.18-2.29), delivery before 32 weeks of gestation (adjusted PR, 3.85; 95% CI, 2.38-6.22), and non-Hispanic Black vs non-Hispanic White maternal race/ethnicity (adjusted PR, 1.54, 95% CI, 1.06-2.24), while biliary atresia was inversely associated with season of conception in the fall relative to spring (adjusted PR, 0.62; 95% CI, 0.45-0.86). In addition, biliary atresia was associated with maternal diabetes (adjusted PR, 2.34; 95% CI, 1.57-3.48), with a stronger association with pregestational diabetes compared with gestational diabetes. In subgroup analyses, these associations were present in isolated biliary atresia cases that do not have any additional birth defects.

Conclusions: Biliary atresia is associated with multiple factors related to fetal development, including pregestational maternal diabetes, female sex, and preterm birth. These associations also were observed in isolated cases of biliary atresia without other malformations or laterality defects. Our results are consistent with early life events influencing the pathogenesis of biliary atresia, and support further studies investigating in utero events to better understand etiology and time of onset.

Keywords: maternal diabetes; prematurity.

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Conflict of interest statement

Conflicts of interest: SH serves on the DSMB for a therapeutic trial for biliary atresia (DSMB coordinated by Syneos Health). The other authors declare no conflicts of interest.

Figures

Figure 1.
Figure 1.
Cases included in this study. Of the births captured by the TDBR between 1999–2014, there were 305 cases of BA not associated with chromosomal abnormalities or Mendelian disorders.
Figure 2.
Figure 2.
BA birth prevalence in Texas by birth year. There were no significant differences in BA prevalence across years during the study period (p=0.98).
See this image and copyright information in PMC

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