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Biliary atresia in twins: a systematic review and meta-analysis

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Abstract

Purpose

Biliary atresia (BA) in twins is extremely rare reported in the literature, but twin studies are useful methods of examining the associated factors of a complex disease. The objective of this study was to analyze the characteristics and patterns of biliary atresia in twins from reviewing available articles.

Methods

PubMed and EMBASE databases were reviewed for related articles using the keywords ‘‘biliary atresia’’, ‘‘twins’’, ‘‘monozygotic (MZ)’’, and ‘‘dizygotic (DZ)’’, including relevant papers in the reference lists.

Results

This analysis was extracted from 12 articles, with a total of 35 twin pairs included. BA was found in 36 out of 70 twin subjects (51.4%), of which had an even gender split. 97.1% twins were discordant, among 55.9% of which were monozygotic twin sets, indicating that BA may be related to genetic phenotype or penetrance. Isolated BA was the largest group with 27 (75%) affected twins. Only one pair of dizygotic twins (2.9%) demonstrate concordance for BA, and have one affected family member.

Conclusion

BA was found in nearly half of twin subjects with an even gender split. Isolated BA was the largest group, in which the number of monozygotic twins was similar with dizygotic twins, so the onset of the disease may not associate with the zygosity of twins. Most of twin sets had discordant disease presentation, especially monozygotic twins therein, emphasizing the role of epigenetic factor in the pathogenesis of BA. Future studies should take genetic testing among any twin sets in BA, especially the disease-associated mutations, thus be useful to investigate the etiology of disease.

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Funding

This study was funded by the National Natural Science Foundation of China (Grant Number 81570471) and Tianjin Health Bureau special Grant (Grant Number 14KG129).

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Authors and Affiliations

  1. Graduate College, Tianjin Medical University, Tianjin, 300070, China

    Xiaodan Xu

  2. Department of General Surgery, Tianjin Children’s Hospital, LongYan Road 238, Beichen District, Tianjin, 300134, People’s Republic of China

    Jianghua Zhan

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  1. Xiaodan Xu
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  2. Jianghua Zhan
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Correspondence to Jianghua Zhan.

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Xu, X., Zhan, J. Biliary atresia in twins: a systematic review and meta-analysis. Pediatr Surg Int 36, 953–958 (2020). https://doi.org/10.1007/s00383-020-04690-4

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