Biliary atresia in twins’population: a retrospective multicenter study in mainland China
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In brief
In brief
Retrospective multicenter study of 19 twin pairs discordant for biliary atresia in China demonstrates genetic and environmental factors in disease etiology. Kasai procedure performed at median 78 days achieved 61% native liver survival at 2 years, with monozygotic twins showing higher viral infection rates than dizygotic twins.
Written by the GCMD Library team from the article.
Abstract
Aim
We evaluated the demographic of biliary atresia (BA) children from twins family and aimed to investigated what it can add to the twins’ literature and our understanding of the disease.
Methods
This study contains 11 medical centers in mainland China and the medical record of twins with BA was retrospectively analyzed from January 2012 to December 2018. Follow-up was carried out by out-patient review and questionnaire.
Results
The study included 19 twin pairs in whom there was discordance for BA. Sixteen (84.2%) affected twin underwent Kasai Procedure (KP); median age at KP was 78 (49–168) days. There were ten affected twins that became jaundice-free at 3 months post-KP, and eight occurred with different degrees of cholangitis post-KP. Six affected twins received Liver Transplantation (LT) successfully. The 2 year native liver survival rate and the 2 year overall survival rate of affected twins were 61.1 and 94.4%, respectively. There were three affected monozygotic (MZ) twins and one healthy co-twin with BA-associated congenital malformations, all of which were cardiac malformations. The number of virus infection of affected MZ twins was significantly more (p = 0.04) than affected dizygotic (DZ) twin.
Conclusions
Discordance for BA in 19 pairs of twins supported that BA may be related to genetic phenotype or penetrance. The difference in genetic background between MZ and DZ affects the susceptibility of the host to virus infection. High acceptance of KP (84.2%) in our study implied a high motivation for treatment for twins with BA. Delays of KP (78 days) in affected twin may be related to the postnatal gradual onset and the late diagnosis.