European chronic kidney disease registries for children not on kidney replacement therapy: tools for improving health systems and patient-centred outcomes.

Chronic kidney disease (CKD) in children, from birth to late adolescence, is a unique and highly challenging condition that requires epidemiological research and large-scale, prospective cohort studies. Since its first launch in 2007, the European Society for Paediatric Nephrology/European Renal Association (ESPN/ERA) Registry has collected data on patients on kidney replacement therapy (KRT). However, slowing the progression of CKD is of particular importance and thus the possibility to extend the current registry dataset to include patients in CKD stages 4-5 should be a priority. A survey was sent to the national representatives within the ESPN/ERA Registry to collect information on whether they are running CKD registries. All the representatives from the 38 European countries involved in the ESPN/ERA Registry participated in the survey. Eight existing CKD registries have been identified. General characteristics of the national registry and detailed data on anthropometry, laboratory tests and medications at baseline and at follow-up were collected. Results provided by this survey are highly promising regarding the establishment of an ESPN CKD registry linked to the ESPN/ERA KRT registry and subsequently linking it to the ERA Registry with the same patient identifier, which would allow us to monitor disease progression in childhood and beyond. It is our belief that through such linkages, gaps in patient follow-up will be eliminated and patient-centred outcomes may be improved.

Long-term outcome of focal segmental glomerulosclerosis after pediatric renal transplantation.

Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation can limit graft survival. Despite new immunosuppressive agents, the incidence of recurrence remains relatively high. To identify risk factors for recurrence and efficacy of treatment, we reviewed the outcome of 23 grafts in 16 children with FSGS who had undergone transplantation between 1985 and 2007 at La Paz Children's Hospital. Recurrence was 56.3% after the first transplantation. We did not find significant differences in age at diagnosis, age at transplantation, age at end-stage renal disease (ESRD), progression to ESRD, bilateral nephrectomy of native kidneys prior to transplantation, use of induction therapy or of different immunosuppressive regimens between patients with and without recurrence. Plasmapheresis (PP) was carried out in seven of nine patients who had suffered recurrence, achieving remission in six of them. One patient received high doses of cyclosporin (CsA) and plasmapheresis, attaining remission. Graft survival was lower (P = 0.043) in patients with FSGS than in those with other ESRD etiologies (first year 75% vs 91%; fifth year 44% vs 78%). Recurrence of FSGS limited graft survival (first year 66% vs 85%; third year 20% vs 68%) (P = 0.07). In our experience, PP can be effective in treating FSGS recurrence, although its effect on long-term graft survival seems more limited.

Anti-GBM and anti-MPO antibodies coexist in a case of pulmonary renal syndrome.

The case of a 12-year-old boy with pulmonary renal syndrome is described. Antimyeloperoxidase (anti-MPO) and antiglomerular basement membrane (anti-GBM) antibodies were positive. The clinical course and immunosuppressive therapy are discussed. Pulmonary renal syndrome is a rare event in childhood and coexistence of the two types of antibodies is exceptional.