https://orcid.org/0000-0003-2354-0168
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Abstract
Background
Biliary atresia (BA) is a severe neonatal progressive cholangiopathy of unknown etiology. A timely Kasai portoenterostomy (KPE) improves survival of the native liver in patients with BA, although liver transplantation remains the ultimate treatment for most (60%–80%) patients. However, postoperative adverse effects of liver transplantation may be significant. In addition, patients require lifelong immunosuppressive therapy after liver transplantation.
Case Summary
Here, we report a case of a newborn female baby (birthday: 10–03-2018) with congenital BA (confirmed at 76 days of life) who survived KPE (first surgery at 85 days of life) and underwent successful living-related liver transplantation (LRLT) (second surgery at 194 days of life). Additionally, we reviewed the existing literature on BA. After KPE (at 85 days of life), the liver function of the baby did not improve, and the indicators of liver and kidney function showed a trend of aggravation, indicating that the liver function had been seriously damaged before KPE (at 85 days of life), demonstrating the urgent need for liver transplantation surgery. The female baby survived after part of her father’s liver was successfully transplanted into her body (at 194 days of life). The patient recovered successfully. No other diseases were found at the 4-year follow-up, and all indices of liver and kidney functions tended to be normal.
Conclusion
This case highlights the following. Postoperative alkaline phosphatase was consistently above the normal range, although the reason for this was unclear; neither tacrolimus nor cyclosporine A has formulations designed specifically for infants, which does not meet the needs of clinical individualized medication, suggesting that these anti-rejection drugs are future development directions. Only one case of congenital BA has been found thus far in Hefei, and this case has extremely important reference significance for the prevention, treatment, and diagnosis of BA in Hefei, Anhui province.
Graphical Abstract
Abbreviations
ADA, Adenosine Deaminase; ALP, Alkaline Phosphatase; ALT, Alanine Aminotransferase; AST, Aspartate Aminotransferase; BA, Biliary Atresia; CK, Creatine Kinase; CNI, Calcineurin inhibitor; CREA, Creatinine; CsA, Cyclosporine A; DBIL, Direct Bilirubin; GGT, Gamma-Glutamyl Transpeptidase; HCT, Hematocrit; HGB, Hemoglobin; IBIL, Indirect Bilirubin; ISD, Immunosuppressive drug; KPE, Kasai Portoenterostomy; LDH, Lactate Dehydrogenase; LRLT, living-related liver transplantation; RBC, Red Blood Cell; Tac, Tacrolimus; TBA, Total Bile Acid; TBIL, Total Bilirubin; TDM, Therapeutic drug monitoring.
Data Sharing Statement
The original contributions presented in the study are all included in the article/Supplementary Material, and further inquiries can be directed to the first author.
Ethical Approval and Consent to Participate
The patient’s mother had given her consent for the article to be published. This article was performed in accordance with the principles of the Declaration of Helsinki, and the Hospital Ethics Committees of Hefei Hospital Affiliated to Anhui Medical University (The Second People’s Hospital of Hefei) approved the publication of case details.
Consent to Publish
The patient’s mother had given her consent for the article to be published.
Acknowledgments
We thank Hefei Hospital Affiliated to Anhui Medical University (The Second People’s Hospital of Hefei) for providing the original data. We thank LetPub (www.letpub.com) for its linguistic assistance during the preparation of this manuscript. We are immensely grateful to our generous patient’s mother for allowing us to publish their case.
Author Contributions
All authors made a significant contribution to the work reported, whether that is in the conception, study design, execution, acquisition of data, analysis and interpretation, or in all these areas; took part in drafting, revising or critically reviewing the article; gave final approval of the version to be published; have agreed on the journal to which the article has been submitted; and agree to be accountable for all aspects of the work.
Disclosure
The authors declare that this research was conducted in the absence of any commercial or financial relationships that could be interpreted as a potential conflict of interest.