The CERTAIN Registry: a novel, web-based registry and research platform for pediatric renal transplantation in Europe.

BACKGROUND: The results of pediatric renal transplantation have improved markedly in the last decade. However, a number of relevant clinical problems remain, such as organ damage caused by chronic rejection, long-term toxicity of immunosuppressive therapy, difficulty in developing tolerance-inducing protocols, secondary cardiovascular comorbidity, post-transplantation lymphoproliferative disease, suboptimal longitudinal growth, quality of life, adherence to immunosuppressive medication, and structured transition programs to adult care. These unmet clinical needs require intense collaborative and interdisciplinary clinical research. We recently founded the Cooperative European Paediatric Renal TransplAnt INitiative (CERTAIN; www.certain-registry.eu) as a research network and platform built on a novel, web-based registry. RESULTS: The registry's dataset provides essential information on generic kidney transplantation-related topics and also captures pediatric-specific topics, such as growth, physical and psychosocial development, and adherence. Due to its flexibility the system can be used as follows: (1) as a registry capturing a minimal or an extended dataset; (2) as a center and/or country-specific transplantation database; or (3) as a patient-specific electronic transplantation chart. The data can be exported directly from the CERTAIN web application into statistical software packages for scientific analyses. The rights regarding data ownership, evaluation, and publications are regulated in the registry's rules of procedure. Data quality is ensured by automatic software validation and a manual data review process. To avoid redundant data entry, CERTAIN has established interfaces for data change with Eurotransplant, the Collaborative Transplant Study (CTS), and the registry of the European Society of Pediatric Nephrology (ESPN) and European Renal Association - European Dialysis and Transplant Association (ERA-EDTA) (ESPN/ERA-EDTA registry). CERTAIN fulfils all regulatory and ethical requirements of the European Union and Germany, in particular, regarding patients' data privacy and security. CONCLUSION: Using modern information technology, the recently established multinational CERTAIN Registry fills a gap in Europe for collaborative 5 research and quality assurance in the field of pediatric renal transplantation.

Renal transplantation for fibromuscular dysplasia.

This is the first report that presents renal transplantation after bilateral nephrectomy as the final treatment for severe renovascular hypertension due to fibromuscular dysplasia (FMD). We describe the history of a 1-year-old girl who suffered from renovascular hypertension due to FMD. Imaging revealed multiple bilateral stenoses of the renal artery extending into the distal branches. The hypertension proved unresponsive to pharmacologic treatment and the intrarenal peripherally located stenoses rendered a conventional approach such as transluminal or surgical angioplasty not feasible. At the age of 5 years, a unilateral nephrectomy of the most affected kidney was performed, but she remained hypertensive and developed progressive cardiomyopathy and retinopathy. At the age of 6 years the remaining kidney was removed, followed by a living related renal transplantation with a kidney donated by her mother. Posttransplantation, she developed mild hypertension due to a postanastomotic stenosis, which was easily controlled with antihypertensives. Now 8 years after transplantation, she has experienced no further blood pressure related problems. Although there is a risk of recurrence of FMD after performing a living related transplantation, our report suggests that this procedure is relatively safe, provided appropriate preoperative evaluation and follow up is performed.

Resting energy expenditure and nutritional status in children with juvenile rheumatoid arthritis.

OBJECTIVE: Undernutrition is frequently encountered in children with juvenile rheumatoid arthritis (JRA). We assessed resting energy expenditure (REE) in relation to nutritional status and body composition in patients with JRA. METHODS: We selected 33 children (age 6 to 18 yrs) with JRA (13 oligoarticular, 10 polyarticular, 10 systemic JRA) and 17 controls matched for age and sex. Nutritional status was assessed for height, weight, and fat-free mass (FFM), and REE was measured with indirect calorimetry. RESULTS: Nutritional status in the patients with systemic JRA was diminished compared to the controls for height (140 vs. 159 cm; p<0.01) and FFM (28 vs. 38 kg; p = 0.03). Oligo and polyarticular patients with JRA had normal height and FFM. No significant differences existed in crude REE among the groups. However, after correcting REE for body weight and FFM, the patients with systemic JRA, compared to controls, had 18% higher REE per kg body weight (159 vs. 134 kJ/kg/day; p<0.01) and 21% higher REE per kg FFM (196 vs. 162 kJ/kg/day; p<0.01). Oligo and polyarticular JRA patients had 8% increased values for REE per kg body weight or FFM, but these differences were not statistically significant. CONCLUSION: Patients with systemic JRA show stunting, low FFM, and a significantly increased REE when nutritional status is taken into account. These data suggest that assessment of individual energy requirements should include correction for fat-free mass in the treatment of malnutrition in patients with systemic JRA.