Parental Living Donor Liver Transplantation as a Solution in Medical Treatment-Resistant Cystathionine-β-Synthase Deficiency: A Single-Center Case Series.
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引用次数: 0
Abstract
Introduction: Cystathionine-β-synthase deficiency (CBSd) is an inherited metabolic liver disease causing morbidities in eyes, skeleton, brain, and vasculature. Despite its potential lethality due to thromboembolism and liver failure, sole diagnosis of CBSd seemed not to fulfill the enlistment criteria for deceased donor liver transplantation in previous reports.
Methods: We retrospectively reviewed three cases of living donor liver transplantation (LDLT) for pediatric CBSd patients responding poorly to conservative treatment in Beijing Friendship Hospital, and a literature review was performed.
Results: Patients 1 and 3 received donated partial liver from heterozygous carrier parents, and Patient 2 received donated partial liver from a CBS-competent parent. Patient 2 developed portal thrombus 1 day after LDLT, which was resolved with surgical thrombectomy and reconstruction. Patients 1 and 3 had their resected liver donated to other patients with advanced liver cancer, and the domino grafts functioned well until the death due to tumor recurrence.
Conclusion: Parental LDLT, whether from carriers or not, is an appropriate alternative for CBSd patients resistant to medical treatment. Risk of peri-operative thromboembolism might be higher in CBSd than in other LDLT cases, and explanted livers with CBSd could serve as domino grafts.
期刊介绍:
The aim of Pediatric Transplantation is to publish original articles of the highest quality on clinical experience and basic research in transplantation of tissues and solid organs in infants, children and adolescents. The journal seeks to disseminate the latest information widely to all individuals involved in kidney, liver, heart, lung, intestine and stem cell (bone-marrow) transplantation. In addition, the journal publishes focused reviews on topics relevant to pediatric transplantation as well as timely editorial comment on controversial issues.