A highly selected subject group comprising pediatric recipients of liver (n = 36) and small intestine alone (n = 1) or multivisceral graft (n = 2) were converted to sirolimus maintenance therapy for tacrolimus-related side effects (n = 32) or by primary intent (n = 7). Indications were nephrotoxicity (n = 14), primary intent (n = 7), post-transplant lymphoproliferative disorder (n = 6), seizures (n = 4), recurrent acute rejection (n = 2), and cardiomyopathy (n = 1). Thirty subjects (78%) experienced successful conversion, with one subject requiring atorvastatin for hypercholesterolemia and hypertriglyceridemia. Nine subjects (22%) were converted back to tacrolimus for serious adverse events including acute rejection (n = 2), elevated liver function tests (n = 1), severe leucopenia (n = 1), non-compliance (n = 2), recurrent malignancy/death (n = 1), steatohepatitis (n = 1), and thrombocytopenic thrombotic purpura (n = 1). Among subjects with nephrotoxicity, significant benefit was seen only in those subjects with shorter time to rescue after transplantation (n = 8 of 14 subjects). Additional benefits included a significant decrease in mean serum creatinine from pretransplant values for the entire population, and elimination of antihypertensive treatment in all five subjects receiving it prior to conversion. Hemoglobin, serum cholesterol and triglycerides, white cell counts and platelets remained within normal limits for the duration of follow-up (36 month). Conversion from tacrolimus to sirolimus is successful in selected pediatric liver and intestine recipients. Chronic nephrotoxicity may be ameliorated by early conversion. Improvement in renal function and hypertension management, and absence of sirolimus-related adverse events argue for prospective evaluation of regimens in which mTOR inhibitors are used without calcineurin inhibitors in children.
Although liver transplantation has become an effective treatment for end-stage liver disease and liver/intestine transplantation is becoming an increasingly viable procedure for end-stage shortgut syndrome in children, little is known about the impact of these procedures on the child's family. Examination of the impact of these transplantations on the family is needed to identify psychosocial factors that may adversely affect the child's physical and emotional health and to plan for preventive interventions. The psychosocial impact of pediatric liver and/or intestine transplantation on parents was assessed in a cross-sectional sample of 41 mothers and 20 fathers evaluated pretransplantation and 2 months posttransplantation. Parental mental health, parenting stress, and quality of life were assessed, as well as demographic, child, and family characteristics as they related to parental outcomes. Parent adjustment did not differ with regard to time of assessment. A majority of parents (n ؍ 31) reported elevated psychological symptoms on the Brief Symptom Inventory (BSI), with fathers showing greater distress than mothers (P F .05). Parents' total scores on the Parenting Stress Index and the Parent and Child Domain subscales were in the normal range. Quality of life was assessed by the Physical Health, General Health Perception, and Vitality subscales of the SF-36. Parents reported better physical functioning (P ؍ .02) but lower vitality ratings than a normative population (P F .01). Family conflict was associated with higher psychological distress on the BSI (P ؍ .02), whereas demographic factors, including the child's age, sex, and number of people in the household, proved most useful in predicting parenting stress and quality of life. These findings of significant psychological distress in parents of children undergoing liver and/or small-intestine transplantation have implications for the child's adaptation and underscore the need for psychosocial assessment of both parents in the perioperative period. Early identification of families at psychosocial risk and the development of interventions that may prevent or reduce psychological distress are indicated to ensure the best possible outcomes for these children and their families.
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