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Biliary atresia in twins’population: a retrospective multicenter study in mainland China

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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.

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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).

Author information

Authors and Affiliations

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

    Qingyun Gou & Jinfu Jia

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

    Qingyun Gou, Jinfu Jia & Jianghua Zhan

  3. Department of Center for Reproductive Medicine, Center for Prenatal Diagnosis, First Hospital of Jilin University, Changchun, 130021, China

    Yang Chen

  4. Department of Neonatal Surgery, Anhui Province Children’s Hospital, Hefei, 230000, China

    Chen Yu

  5. Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China

    Jiexiong Feng

  6. Department of General Surgery, Tianjin First Central Hospital, Tianjin, 300192, China

    Wei Gao

  7. Department of General Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, 100050, China

    Zhijun Zhu

  8. Department of Pediatric Surgery, Children’s Hospital of Nanjing Medical University, Nanjing, 210008, China

    Weibing Tang

  9. Department of Neonatal Surgery, Children’s Hospital, School of Medicine, Zhejiang University, Zhejiang, 310003, China

    Jinfa Tou

  10. Department General of Surgery, Wuhan Children’s Hospital, Wuhan, 430030, China

    Hongqiang Bian

  11. Department of Surgery, Shenzhen Children’s Hospital, Shenzhen, 518026, China

    Bin Wang

  12. Department of Surgery, Second Hospital of Hebei Medical University, Shijiazhuang, 050000, China

    Yingchao Li

  13. Department of Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou, 511440, China

    Le Li

  14. Department of Neonatal Surgery, Children’s Hospital of Shanxi, Taiyuan, 030013, China

    Hongxia Ren

  15. Department of Thoracic and Oncological Surgery, Qilu Children’s Hospital of Shandong University, Jinan, 250022, China

    Yurui Wu

Authors
  1. Qingyun Gou
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  2. Yang Chen
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  3. Chen Yu
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  4. Jinfu Jia
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  5. Jiexiong Feng
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  6. Wei Gao
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  7. Zhijun Zhu
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  8. Weibing Tang
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  9. Jinfa Tou
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  10. Hongqiang Bian
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  11. Bin Wang
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  12. Yingchao Li
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  13. Le Li
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  14. Hongxia Ren
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  15. Yurui Wu
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  16. Jianghua Zhan
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Contributions

QG and YC contributed significantly and equally to the planning of the study and the study design, performed statistical analysis and drafted the manuscript. Data were collected by CY; JJ; JF; WG; ZZ; WT; JT; HB; BW; YL; LL; HR and YW. JZ contributed significantly for manuscript editing and expertise.

Corresponding author

Correspondence to Jianghua Zhan.

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Conflict of interest

No authors have a conflict of interest or financial ties to disclose.

Ethics approval

This study was approved by the Review Board of Ethics Committee of Tianjin Children’s Hospital (Number: 201401) and complied with the Declaration of Helsinki.

Informed consent

All blood samples from subjects were obtained with written informed consent from their own and their parents and/or legal guardians.

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Gou, Q., Chen, Y., Yu, C. et al. Biliary atresia in twins’population: a retrospective multicenter study in mainland China. Pediatr Surg Int 36, 711–718 (2020). https://doi.org/10.1007/s00383-020-04662-8

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  • DOI: https://doi.org/10.1007/s00383-020-04662-8

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