title: Viral infections and prolonged fever after liver transplantation in young children with inborn errors of metabolism.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
authors.forename: Hsiang-Po
authors.initials: HP
authors.lastname: Huang
authors.forename: Yin-Hsiu
authors.initials: YH
authors.lastname: Chien
authors.forename: Li-Min
authors.initials: LM
authors.lastname: Huang
authors.forename: Yen-Hsuan
authors.initials: YH
authors.lastname: Ni
authors.forename: Mei-Hwei
authors.initials: MH
authors.lastname: Chang
authors.forename: Ming-Chih
authors.initials: MC
authors.lastname: Ho
authors.forename: Po-Huang
authors.initials: PH
authors.lastname: Lee
authors.forename: Wuh-Liang
authors.initials: WL
authors.lastname: Hwu
mesh_terms: D020165:Carbamoyl-Phosphate Synthase I Deficiency Disease / Q000601:surgery; D002675:Child, Preschool; D003586:Cytomegalovirus Infections / Q000209:etiology*; D020031:Epstein-Barr Virus Infections / Q000209:etiology*; D005334:Fever / Q000209:etiology*; D006801:Humans; D007223:Infant; D016031:Liver Transplantation / Q000009:adverse effects*; D008661:Metabolism, Inborn Errors / Q000601:surgery*; D008764:Methylmalonic Acid / Q000652:urine; D020163:Ornithine Carbamoyltransferase Deficiency Disease / Q000601:surgery; D011183:Postoperative Complications / Q000209:etiology*
chemical_list: D008764:Methylmalonic Acid