Emotional quality-of-life and patient-reported limitation in sports participation in children with uncorrected congenital and acquired heart disease in healthcare-restricted settings in low- and middle-income countries.

BACKGROUND: Little is known about emotional quality-of-life in paediatric heart disease in low- and middle-income countries where the prevalence of uncorrected lesions is high. Research on emotional quality-of-life and its predictors in these settings is key to planning interventions. METHODS: Ten-year retrospective cross-sectional study of children aged 6-17 years with uncorrected congenital or acquired heart disease in 12 low- and middle-income countries was conducted. Emotional functioning score of the PedsQL TM 4.0 generic core scale and data on patient-reported limitation in sports participation were collected via in-person interview and analysed using regression analyses. RESULTS: Ninety-four children reported mean emotional functioning scores of 71.94 (SD 25.32) [95% CI 66.75-77.13] with lower scores independently associated with having a parent with a chronic illness or who had died (p = 0.005), having less than three siblings (p = 0.007), and reporting a subjective limitation in carrying an item equivalent to a 4 lb load (p = 0.021). Patient-reported limitation in sports participation at least "sometimes" was present in 69% and was independently associated with experiencing symptoms at least once a month (p < 0.001). CONCLUSION: Some of the factors which were associated with better emotional quality-of-life were similar to those identified in previous studies in patients with corrected defects. Patient-reported limitation in sports participation is common. In addition to corrective surgery and exercise, numerous other interventions which are practicable during surgical missions might improve emotional quality-of-life.

Early renal benefit of rapamycin combined with reduced calcineurin inhibitor dose in pediatric heart transplantation patients.

BACKGROUND: Calcineurin inhibitors such as cyclosporine are effective in preventing rejection in recipients of solid organ transplants. Unfortunately, the prolonged use of calcineurin inhibitors may result in progressive renal injury. METHODS: We studied the renal function of 15 pediatric heart transplant recipients who were taking calcineurin inhibitors. Their renal function was studied before and after rapamycin was introduced to their immunosuppression regimen. With the introduction of rapamycin, the patients were given a lower dose of calcineurin inhibitors, and the calcineurin inhibitor was discontinued in 5 patients. RESULTS: Renal function improved significantly in the patients by 30 days after these changes in the calcineurin inhibitor dose were instituted. Mean levels of blood urea nitrogen and mean serum creatinine decreased, and mean creatinine clearance increased. Pre-rapamycin, the patients' mean level of blood urea nitrogen was 27.1 +/- 12.4 mg/dl and post-rapamycin they decreased to 18.6 +/- 11.1 mg/dl (p = 0.014). Similarly, creatinine decreased from 1.0 +/- 0.5 mg/dl to 0.8 +/- 0.3 mg/dl (p = 0.019). Their creatinine clearance increased from 88 +/- 28 ml/min/1.73 mol/liter2 to 105 +/- 27 ml/min/1.73 mol/liter2 (p = 0.008). The patients' lipid levels did not change after they were prescribed rapamycin. Biopsy-negative rejection developed in 2 patients. CONCLUSIONS: The introduction of rapamycin to the immunosuppressive regimen of patients taking calcineurin inhibitors, with a concomitant reduction of the calcineurin inhibitor dose, may improve renal function within 30 days, without a significant increase in rejection.