Biliary atresia in twins: a systematic review and meta-analysis
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In brief
In brief
Systematic review of 35 twin pairs reveals biliary atresia affects half of twin subjects with 97% discordance, predominantly in monozygotic twins, suggesting epigenetic rather than purely genetic etiology. Isolated BA was most common (75%), with disease presentation independent of zygosity, highlighting the need for genetic testing to elucidate pathogenesis.
Written by the GCMD Library team from the article.
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.