Background: Liver allograft fibrosis (LAF) is prevalent among children with long-term survival after liver transplantation (LT). We aimed to identify clinical risk factors, with a focus on the impact of immunosuppression (IS) level in the early posttransplant period on LAF. Methods: A retrospective study was conducted on pediatric LT recipients with at least one year of follow-up. Cox regression models were used to analyze risk factors associated with LAF, and landmark analysis was used to evaluate the impact of IS level on LAF. Longitudinal analysis was also conducted in patients with paired biopsies. Results: A total of 139 patients involving 174 liver biopsies were included. With 2.3 to 5.9 years of follow-up, LAF was detected in 91.4% of patients (7.9% were significant), up to 88.2% of whom showed normal liver function. Episodes of acute rejection, biliary complications, CMV infection and prolonged cold ischemia time were independent risk factors. Besides, the risk of LAF in patients with relatively low IS levels at postoperative 1-3, 3-6, 6-12, and 12-36 months was higher than the counterparts. Especially, in patients with relatively high IS levels (mean tacrolimus trough concentration ≥5.1 ng/mL) during postoperative 12-36 months, the risk of LAF was 67% lower in the short future (P=0.006). In paired analysis, patients with increased IS levels were more likely to achieve fibrosis reduction (HR=7.53, P=0.025). Conclusions: Mild to moderate LAF is common among pediatric LT recipients and can appear early and silently. Maintaining adequate levels of immunosuppression during 1 to 3 years after LT seems crucial to ensure protection against LAF.
Background Maple syrup urine disease (MSUD) is a rare genetic deficiency of the branched-chain α-keto acid dehydrogenase (BCKAD) complex that breaks down amino acids, resulting in multi-organ failure. This report is of 5 pediatric cases of domino liver transplantation (DLT) from live donors with MSUD from a single transplant center in Beijing. Case Reports All MSUD donors were confirmed to have disease-causing mutations in BCKDHA (branched-chain keto acid dehydrogenase E1, α polypeptide) or BCKDHB (branched-chain keto acid dehydrogenase E1, β polypeptide) genes by peripheral blood whole-exon sequencing. Serum leucine and valine concentrations were significantly higher than normal values. Recipients ranged in age from 0.75 to 9 years old. Three patients underwent auxiliary liver transplantation, and the other children all underwent liver or partial liver transplantation. This case report was followed up for 25 to 79 months. The prognosis, growth, and development of patients were followed up. By the end of the last follow-up, all children had survived. All patients had normal serum leucine and valine concentrations after surgery. In case 1, portal vein stenosis post-operatively. In case 2, stenosis of hepatic artery and bile duct occurred. In case 5, hepatic artery and portal vein stenosis occurred, resulting in graft loss. Conclusions The findings from our center support the findings from other pediatric liver transplant centers that liver transplantation using MSUD donors can have successful outcomes without the development of MSUD in the recipient.
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