Living related donor liver transplantation for infant with Crigler-Najjar syndrome type Ⅰ: a report of one case and literature review

Gu Guangxiang; Zong Zhipeng; Zhou Tao; Xia Qiang

doi:10.3969/j.issn.1674-7445.2020.04.008

Volume 11 Issue 4

Jul. 2020

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Gu Guangxiang, Zong Zhipeng, Zhou Tao, et al. Living related donor liver transplantation for infant with Crigler-Najjar syndrome type Ⅰ: a report of one case and literature review[J]. ORGAN TRANSPLANTATION, 2020, 11(4): 471-476. doi: 10.3969/j.issn.1674-7445.2020.04.008

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Living related donor liver transplantation for infant with Crigler-Najjar syndrome type Ⅰ: a report of one case and literature review

doi: 10.3969/j.issn.1674-7445.2020.04.008

Department of Liver Surgery, Renji Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200127, China

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Corresponding author: Xia Qiang, Email: xiaqiang@shsmu.edu.cn
Received Date: 2020-03-26
Available Online: 2021-01-19
Publish Date: 2020-07-15

Abstract

Abstract

Objective To summarize the therapeutic effects of living related donor liver transplantation for Crigler-Najjar syndrome type Ⅰ (CNS type Ⅰ). Methods A 3-month-old male infant had appeared a progressive xanthochromia of the skin and sclera 4 d after birth without obvious cause. Other causative factors were eliminated after relevant tests were completed, and identified as CNS type Ⅰ by genetic testing. Living related donor liver transplantation was performed with his mother as the donor. An immunosuppression regimen was routinely applied postoperatively and tacrolimus doses were adjusted according to biochemical indicators and cytochrome P450 (CYP) 3A5 genotype of the recipient. Results The liver enzymes of the recipient returned to normal at 7 d postoperatively, and bilirubin decreased daily and fell to the normal range at 22 d postoperatively. Followed up to the submission date, the recipient's xanthochromia of skin and scleral faded with normal bilirubin and stable liver enzymes. The condition of the recipient was generally good with high quality of life. Conclusions Living donor liver transplantation can treat unconjugated hyperbilirubinemia and other diseases caused by CNS type Ⅰ, which greatly improve the quality of life of patients.
- Liver transplantation,
- Crigler-Najjar syndrome,
- Alanine aminotransferase,
- Aspartate aminotransferase,
- Total bilirubin,
- Bilirubin encephalopathy,
- Cytochrome P450 (CYP)3A5

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References(41)

References

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Living related donor liver transplantation for infant with Crigler-Najjar syndrome type Ⅰ: a report of one case and literature review

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Living related donor liver transplantation for infant with Crigler-Najjar syndrome type Ⅰ: a report of one case and literature review

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