A2.3 Management of children with congenital nephrotic syndrome: challenging treatment paradigms
| Autor: | VS Conti, C Aufricht, Rukshana Shroff, E Ylinen, A. Edefonti, Enrico Vidal, G. Ariceta, RY Cicek, Stephanie Dufek, Aysun Karabay Bayazit, A. Jankauskiene, M do Sameiro Faria, Ismail Dursun, Hazel Webb, J Bacchetta, K. Vondrak, A Zampetoglou, N Printza, M. Ekim, S. Bakkaloglu, G. Klaus, Tuula Hölttä, C Stefanidis, H Alpay, CP Schmitt, E Verrina, Andrea Pasini, A Trautmann, F Paglialonga |
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| Rok vydání: | 2017 |
| Předmět: |
Nephrology
medicine.medical_specialty Pediatrics business.industry medicine.medical_treatment 030232 urology & nephrology 030204 cardiovascular system & hematology medicine.disease Nephrectomy Transplantation 03 medical and health sciences 0302 clinical medicine Interquartile range Internal medicine medicine Renal replacement therapy business Congenital nephrotic syndrome Dialysis Bilateral Nephrectomy |
| Zdroj: | Parallel session abstracts. |
| Popis: | Background Management of children with congenital nephrotic syndrome (CNS) is challenging. Early bilateral nephrectomies followed by dialysis and transplantation is currently practised in most centres, but conservative treatment may also be effective. Methods We conducted a 6 year survey across members of the European Society for Paediatric Nephrology Dialysis Working Group to compare management strategies and their outcomes in children with CNS. Results 81 children (51% male) across 17 tertiary nephrology units in Europe were included (NPHS1 n=57; NPHS n=2, WT1 n=10, others n=12; details of mutations not examined). Antiproteinuric treatment was given in 48 (59%) with an increase in S-albumin in 68% by median 6 (interquartile range 3–8) g/L (p Children with NPHS1 mutations and >12 months follow-up were divided into two groups and their outcomes were compared: bilateral nephrectomy (n=26) versus conservative management (no nephrectomy; n=17). Nephrectomised children presented earlier (3 vs 29 days; p=0.01), with comparable S-albumin (p=0.21) and S-creatinine (p=0.19). There was no difference in the number of septic or thrombotic episodes and growth was comparable. At final follow-up (median age 34 months) 9 (53%) children in the conservative group remained without renal replacement therapy, 4 (24%) received a renal transplant and 2 died. Amongst nephrectomised children 21 (81%; p Conclusion An individualised, stepwise approach, with prolonged conservative management, followed by unilateral nephrectomy may be a reasonable alternative to early bilateral nephrectomies in children with CNS secondary to NPHS1 mutations. |
| Databáze: | OpenAIRE |
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