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A girl aged 6 years 9 months with severe heart disease secondary to homozygous familial hypercholesterolaemia underwent orthotopic cardiac transplantation and her liver was replaced with the liver of the same donor. In the first 10 weeks after transplantation serum cholesterol fell to 270 mg/dl from preoperative concentrations of more than 1000 mg/dl.

We studied 51 consecutive pediatric patients for the frequency and morbidity of viral infections after liver transplantation. The incidence of primary (67fT/o) and reactivation (480/0) Epstein-Barr virus (EBV) infections and reactivation (88fT/o) cytomegalovirus (CMV) infection was comparable to that seen in adult transplant recipients. However, fewer pediatric than adult transplant recipients experienced primary CMV infection (P < .01). Five (38%) of 13 CMV infections were symptomatic and included hepatitis, pneumonitis, enteritis, and mononucleosis. Two of 14 patients with primary EBV infection subsequently developed, at two months and two years after initial infection, an EBV-associated lymphoproliferative syndrome, and one of 10 patients with reactivated EBV infection developed a possible EBV-associated febrile encephalopathy. Other viruses causing infection in these children included herpes simplex virus, varicella-zoster virus, adenovirus, parainfluenza virus, respiratory syncytial virus, and rotavirus.Both Epstein-Barr virus (EBV) and cytomegalovirus (CMV) are notable for their ubiquity, latency, and tendency to reactivate in the immunosuppressed host. Reports on the frequency and morbidity of EBV and CMV infections in adult transplant recipients from this center are available [1][2][3]. Comparable data in children have not been reported because, until recently, they had not undergone transplantations in large numbers. The behavior of EBV and CMV in pediatric transplant recipients is of interest not only because children may respond differently, but unlike adults, a large proportion of children have not been infected by these viruses before transplantation. Hence, a smaller number of children are at risk of developing reactivation infections; however, a greater number may be susceptible to primary infections and their consequent morbidity.

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Between March 3, 1981, and June 1, 1984, 216 children were evaluated for orthotopic liver transplantation. Of the 216 patients, 117 (55%) had received at least one liver transplant by June 1, 1985. Fifty-five (25%) died before transplantation. The 117 patients who received transplants were grouped according to severity of disease and degree of general decompensation at the time of transplantation. The severity of a patient's medical condition with the possible exception of deep hepatic coma, did not predict outcome following orthotopic liver transplantation. Severity variables were assessed at the time of the evaluation. Twenty-three of the 70 variables were found to have prognostic significance with regard to death from progressive liver disease before transplantation. These 23 variables were incorporated into a multivariate model to provide a means of determining the relative risk of death among pediatric patients with end-stage liver disease. This information may allow more informed selection of candidates awaiting liver transplantation.The advent of cyclosporine and advances in surgical technique changed the status of orthotopic liver transplantation from an experimental procedure to accepted care for lethal liver disease. 1 As 1-year survival probabilities approached 66% of OLTx recipients in late 1981 and early 1982, 2 the question of who should receive the next available organ took on critical importance. The improvement in success of OLTx increased the number of children referred for the procedure. More referrals not only sparked an increase in transplantation but drew attention to the shortage of available organs. 3 Twenty-five percent of referred children died of progressive liver disease before transplantation because an appropriate organ could not be located. Donated organs became a precious resource, and their utilization demanded a logical approach to recipient selection.The process of selecting a recipient when a donor liver becomes available is determined primarily by matching the donor and recipient according to size and according to ABO blood group, conforming to blood banking rules of blood transfusions. 4 In situations of great urgency, even ABO incompatibility may be disregarded as a condition for donor recipient matching. 5 No attempt to match minor red cell antigens or Rh factor is made. Extensive tissue typing is not performed because of time constraints, 4 and it appears from retrospective analysis that it may be unnecessary. 4, 5 A number of appropriately matched potential recipients may exist for each donated organ. We attempted to determine whether providing a transplant for the sickest available potential recipient has an adverse effect on transplantation outcome, and second, to develop criteria to distinguish the sickest patient among those with heterogeneous but lethal liver diseases.

A child with homozygous protein C deficiency was treated at age 20 months by orthotopic hepatic transplantation. Postoperatively there was complete reconstitution of protein C activity and resolution of the thrombotic condition.

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