Viral Infections and Prolonged Fever after Liver Transplantation in Young Children with Inborn Errors of Metabolism

• Published in: Journal of the Formosan Medical Association Vol. 104; no. 9; pp. 623 - 629
• Main Authors: 黃祥博(Hsiang-Po Huang), 簡穎秀(Yin-Hsiu Chien), 黃立民(Li-Min Huang), 倪衍玄(Yen-Hsuan Ni), 張美惠(Mei-Hwei Chang), 何明志(Ming-Chih Ho), 李伯皇(Po-Huang Lee), 胡務亮(Wuh-Liang Hwu)
• Format: Journal Article
• Language: English
• Published: Singapore 臺灣醫學會 01.09.2005
• Subjects: Carbamoyl-Phosphate Synthase I Deficiency Disease - surgery; Child, Preschool; Cytomegalovirus Infections - etiology; Epstein-Barr Virus Infections - etiology; Fever - etiology; Humans; Infant; Liver Transplantation - adverse effects; Metabolism, Inborn Errors - surgery; Methylmalonic Acid - urine; Ornithine Carbamoyltransferase Deficiency Disease - surgery; Postoperative Complications - etiology; Cytomegalovirus infections; Inborn errors of metabolism; Liver thransplantation; Epstein-Barr virus infections
• ISSN: 0929-6646
• DOI: 10.29828/JFMA.200509.0002

Liver transplantation could be a useful treatment for selected inborn errors of metabolism. This study evaluated the outcome and viral infections after liver transplantation in young children and infants with these diseases. The outcome of liver transplantation and clinical characteristics of the following 4 patients were assessed: 1 infant with ornithine transcarbamylase deficiency (OTCD) who received liver transplant aged 3 years and 4 months; 1 infant with carbamyl phosphate synthetase I deficiency (CPSID) who received liver transplant at 14 months of age; and 2 infants with methylmalonic acidemia (MMA) who received liver transplant at 8 months and 11 months of age, respectively. All donors, except the 8-month-old infant with MMA, showed serologic evidence of previous cytomegalovirus (CMV) infection before transplantation. All 4 of these donors showed serologic evidence of previous infection of Epstein-Barr virus (EBV). None of the recipients had previous CMV infection. Both the infant with OTCD and the 8-month-old infant with MMA had previous EBV infection, while the other 2 patients did not. Preoperative hemodialysis was performed in both infants with MMA. Postoperative follow-up included metabolic stability, disability degree, and viral infections. None of the patients developed severe metabolic decompensation after transplantation and all increased protein intake postoperatively. Symptomatic viral infections, however, were present in all patients postoperatively, including CMV infection in the infant with OTCD and the 11-month-old infant with MMA, reactivation of EBV infection in the infant with OTCD and the 8-month-old infant with MMA, and primary EBV infection in the infant with CPSID. Liver transplantation was an effective treatment for all 4 of these patients with inborn errors of metabolism. The risk of symptomatic viral infections for these patients was high. This was likely associated with conditions including immunosuppression, young age, endemic nature of CMV and EBV infections, and lack of CMV prophylaxis.