Background and objectives Treatment of congenital nephrotic syndrome (CNS) and steroid-resistant nephrotic syndrome (SRNS) is demanding, and renal prognosis is poor. Numerous causative gene mutations have been identified in SRNS that affect the renal podocyte. In the era of high-throughput sequencing techniques, patients with nongenetic SRNS frequently escape the scientific interest. We here present the long-term data of the German CNS/SRNS Follow-Up Study, focusing on the response to cyclosporin A (CsA) in patients with nongenetic versus genetic disease.Design, setting, participants, & measurements Cross-sectional and longitudinal clinical data were collected from 231 patients with CNS/SRNS treated at eight university pediatric nephrology units with a median observation time of 113 months (interquartile range, 50-178). Genotyping was performed systematically in all patients.Results The overall mutation detection rate was high at 57% (97% in CNS and 41% in SRNS); 85% of all mutations were identified by the analysis of three single genes only (NPHS1, NPHS2, and WT1), accounting for 92% of all mutations in patients with CNS and 79% of all mutations in patients with SRNS. Remission of the disease in nongenetic SRNS was observed in 78% of patients after a median treatment period of 2.5 months; 82% of nongenetic patients responded within 6 months of therapy, and 98% of patients with nongenetic SRNS and CsA-induced complete remission (normalbuminemia and no proteinuria) maintained a normal renal function. Genetic SRNS, on the contrary, is associated with a high rate of ESRD in 66% of patients. Only 3% of patients with genetic SRNS experienced a complete remission and 16% of patients with genetic SRNS experienced a partial remission after CsA therapy. ConclusionsThe efficacy of CsA is high in nonhereditary SRNS, with an excellent prognosis of renal function in the large majority of patients. CsA should be given for a minimum period of 6 months in these patients with nongenetic SRNS. In genetic SRNS, response to CsA was low and restricted to exceptional patients.

Vicorder aPWV values are similar to those obtained by SphygmoCor applanation tonometry. The best agreement between devices was obtained with the path length that most accurately describes the aortic tree. Excellent intra- and interobserver repeatability and ease of measurements make Vicorder appropriate for large multicentre studies in children and adolescents.

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Posters 492was >99 th percentile in the four limbs. The retina was normal. She performed the following exams, all normal: blood cell count, blood urea, creatinin, ions, lipid profile, thyroid function, rennin, aldosterone, urine sediment and urinary catecolamins, metanefrins, homovanilic and vanilmandelic acid. The cardiac and abdominal ultrasounds were also normal. The renal Doppler ultrasound was "not suggestive of renovascular disease". With these results, she performed a renal cintigraphy with DMSA that showed a "hipofixation of the contrast in the superior half of the left upper renal pole". After it, she performed a renal arteriography, which confirmed the left renal artery stenosis and an angioplasty was made, with success.Conclusions: This diagnosis was delayed by the normality of some exams, specially the renal Doppler ultrasound and the plasmatic renin activity. However, it is important to underline that if the suspicion index is high, the persistence on the evaluation should involve the second line exams, like in this case. GROWTH MONITORING IN CHRONIC KIDNEY DISEASE