We describe four patients with Barth syndrome who have undergone successful orthotopic heart transplantation. Patients are one, seven, 12.5 and 14.7 yr post-transplantation. One episode of severe infection occurred. Renal dysfunction and coronary allograft vasculopathy do not appear accelerated over non-Barth patients. Despite withholding purine synthesis inhibitors, these patients have not demonstrated an increased rate of rejection.

No abstract

Journal:Pediatric TransplantationMany young transplant recipients experience psychological distress and adjustment difficulties, yet there is little research investigating lung transplantation from the recipients' perspective. This Examples include supporting identity integration post-transplant; facilitating social inclusion;considering alternative means of support; and involving adolescents in healthcare decisions.

The process of heart transplantation poses numerous challenges and adaptive tasks for paediatric patients and their families. Few studies have examined how the experience of transplant interacts with developmental transitions such as adolescence, a period of significant change, and adjustment in itself. We explored adolescent heart transplant recipients' and their parents' experience of transplant from the point at which their heart condition was diagnosed to several months after transplantation. We adopted a developmental focus, to consider how participants negotiated the tasks of adolescence in the context of their transplant experiences. A qualitative approach was used to interview five adolescent-parent dyads, who reported few post-transplant complications, and the data were analysed according to the principles of IPA. Our findings revealed transplant to be a transformative experience, with two themes marking a contrast between pre- and post-transplant states: "Restriction and Dependence" and "Autonomy and Freedom." The themes are considered in relation to adolescent development. We propose that clinicians working with paediatric heart transplant recipients and their families need to consider the particular developmental challenges faced by adolescent patients and view the attainment of developmental milestones alongside physical and psychological markers of successful adjustment.

The British Transplant Games are held annually for children and adults who have received transplanted organs. The aim of our pilot study was to determine whether participation in such an event had an impact on psychological well-being. We asked 26 children participating in the Games to complete a specifically designed questionnaire before and after the event. The 13 questions covered areas such as state of mood, perceived physical health, fatigue, anxiety, and confidence. There were mean improvements over time on 10 of the 13 questions, and the change in overall score was significant (p = .036). For the majority of young people, participation in the Transplant Games was associated with a positive impact on psychological functioning in the short term. Further evaluation is now required to determine whether such benefits are maintained in the longer term.