A survey of psychosocial adaptation in long-term survivors of pediatric liver transplants.

We assessed 41 children and adolescents who had received liver transplants at least 4 years ago, for social, behavioral, and emotional adaptation; physical function; and family stress. We compared their level of adaptive functioning to published data from chronically ill and medically well children. On many measures, transplant recipients had equivalent levels of function to the comparison groups. However, 6- to 11-year-old patients showed mild social and scholastic deficits. Patients' parents report less negative impact of the illness on the family than do parents of other chronically ill children. A listing of medication side effects and the degree to which they are problematic was obtained.

Activity of C1 esterase inhibitor in patients with vascular leak syndrome after bone marrow transplantation.

Vascular-leak syndrome (VLS) is a common complication in the first 3 weeks after bone marrow transplantation (BMT). The patients present with weight gain, generalized edema, ascites, pericardial or pleural effusions, tachycardia, arterial hypotonia, and/or pre-renal failure. The aim of our study was to investigate the role of the complement system in VLS. The protein concentrations of C3 and C4 were studied by immunodiffusion, and total hemolytic complement activity was studied by assessment of CH50. C1 esterase inhibitor (C1 Inh), the major inhibitor of the classical pathway of complement, was assessed by a functional test. Activation of complement was assessed by C4d (a C4 activation product). Twelve patients were followed prospectively from start of conditioning therapy to day +21 after bone marrow transplantation. Eight of 12 patients did not develop VLS. These patients had an increase of C3 between day +9 and day +13 (range: 1.3- to 1.5-fold, median: 1.4-fold), C4 (range: 1.3- to 1.9-fold, median: 1.4-fold), CH50 (range: 1.3- to 1.6-fold, median: 1.4-fold), and C1 Inh (range: 1.2- to 1.5-fold, median: 1.3-fold). Four of 12 patients developed VLS. C1 Inh activity was decreased to 0.60- to 0.80-fold. This decrease began 2-6 days prior to clinical diagnosis of VLS (n = 3), or at onset of VLS (n = 1). Patients with VLS showed elevated C4d concentrations (up to 2.4 mg/dl, upper normal threshold value: 0.9 mg/dl). Patients with VLS reveal an activated state of the complement system which is accompanied by a reduced activity of C1 Inh. Insufficient control of complement activation may contribute to VLS in patients after BMT.

Identifying factors in post-operative successful adaptation to pediatric liver transplantation.

In order to identify variables contributing to successful adaptation to liver transplantation, 38 families, at least six months post-transplant, were divided according to transplant team members' rating into two groups: those with successful adaptation to transplant and those families at risk for difficulties. Families who had successful follow-up periods tended to be characterized by intact marriages, private insurance coverage, less subjective financial stress, and higher intellectual and developmental functioning in the child. Single-parent families who had no pre-evaluation involvement with liver transplant organizations and had lengthy post-operative hospital stays more frequently had complications post-transplant. Case studies are presented illustrating the application of this information.