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Background: Recommendations for management of Finnish-type congenital nephrotic syndrome (CNS) followed by many teams include daily albumin infusions, early bilateral nephrectomy, dialysis and transplantation. We aimed to assess the treatment and outcome of patients with CNS in France., Methods: We conducted a nationwide retrospective study on 55 consecutive children born between 2000 and 2014 treated for non-infectious CNS., Results: The estimated cumulative incidence of CNS was 0.5/100 000 live births. The underlying defect was biallelic mutations in NPHS1 (36/55, 65%), NPHS2 (5/55, 7%), PLCE1 (1/55, 2%), heterozygous mutation in WT1 (4/55, 7%) and not identified in nine children (16%). Fifty-three patients (96%) received daily albumin infusions from diagnosis (median age 14 days), which were spaced and withdrawn in 10 patients. Twenty children (35%) were managed as outpatients. Thirty-nine patients reached end-stage kidney disease (ESKD) at a median age of 11 months. The overall renal survival was 64% and 45% at 1 and 2 years of age, respectively. Thirteen children died during the study period including four at diagnosis, two of nosocomial catheter-related septic shock, six on dialysis and one after transplantation. The remaining 13 patients were alive with normal renal function at last follow-up [median 32 months (range 9-52)]. Renal and patient survivals were longer in patients with NPHS1 mutations than in other patients. The invasive infection rate was 2.41/patient/year., Conclusions: Our study shows: (i) a survival free from ESKD in two-thirds of patients at 1 year and in one-half at 2 years and (ii) a significant reduction or even a discontinuation of albumin infusions allowing ambulatory care in a subset of patients. These results highlight the need for new therapeutic guidelines for CNS patients., (© The Author(s) 2018. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.)

It is crucial not to miss the first consultation with an adolescent. This article reviews some of the essential step necessary to help make the first consultation a success: the time to devote to the patient, the preparation of the consultation, mandatory issues to address, clinical/physical examination, conclusion of the consultation, the prescriptions and the follow-up.

Unlabelled: The aim of this study was to determine the metrological parameters of a french version of the Pleasure Scale for Children (PSC): 214 (121 males and 93 females) with a mean age of 8.69 years (sd: 1.95) ranging from 6 years to 12 years were included in the study. The children were inpatients presenting various somatic disorders., Statistical Analysis: First a principal component analysis was done on the 39 items of the correlation matrix. Several guidelines were used to limit the number of factors (Kaiser criteria, Cattell scree test, Horn parallel analysis). Secondly the construct validity was studied using the alpha Cronbach coefficient and by calculing the Pearson correlation coefficient between each item and the total score. Thirdly the concurrent validity was determinated using two items of the Children Depression Rating Scale--Revised (CDRS-R) measuring pleasure (social withdrawal and enjoyment capacity). Fourthly the discriminant validity of the PSC was studied by comparing non depressive children (score lower than 30 to the CDRS-R) and depressive children (score higher than 30 to the CDRS-R)., Results: The principal component analysis showed a one factor solution with 33 items among the 39 having a higher than 0.3 saturation. The Cronbach alpha coefficient was 0.84. All the items correlated with the total score. The mean value was 0.37. The correlations between the total score of the scale and the CDRS-R enjoyment capacity and social withdrawal items were respectively -0.37 (p < 0.01) and -0.38 (p < 0.01). PSC score were significantly lower in depressive children (m = 86.96; sd = 8.33) than in non depressive children (m = 94.67; sd = 10) (t = 5.32; df = 212; p < 0.001).

High-titer (1:30,000) antiglucagon antibodies were detected in a 14-month-old patient with nesidioblastosis who had been treated by intramuscular long-acting glucagon since age 5 months. In this patient no correct equilibrium of blood glucose level could be obtained without maintaining glucagon treatment, even after subtotal pancreatectomy. Antiglucagon antibodies titer remained stable at age 18 months.