Syndrome hémolytique et urémique de l'enfant (étude d'une série de 27 cas en Auvergne (1990-2002))

CLERMONT FD-BCIU-Santé (631132104) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Evaluation de croissance des enfants porteurs de rachitisme hypophosphatémique de l'interrégion Rhône Alpes Auvergne

Le rachitisme hypophosphatémique est une pathologie héréditaire rare pour laquelle de nombreux progrès physiopathologiques et génétiques ont été faits ces dernières années. Le traitement permet une amélioration du rachitisme mais son efficacité sur la croissance est controversée. L'objectif de cette étude rétrospective était de rechercher des facteurs pronostics clinico-biologiques de bonne croissance sur une population de 39 enfants de l'interrégion Rhône-Alpes Auvergne. Les facteurs de bonne croissance retrouvés sont le sexe féminin, les cas sporadiques, un diagnostic avant -2 DS et une taille à 4 ans supérieure à -2 DS, ce qui pourrait confirmer cliniquement le rôle évoqué par Jehan sur le génotype du récepteur de la vitamine D. Par contre, la phosphorémie moyenne ou le nombre de phosphorémie supérieure à 1 et le traitement ne semblent pas discriminant. Il ne paraît donc pas nécessaire de vouloir normaliser la biologie en augmentant les posologies de traitement au risque d'avoir des effets indésirables (néphrocalcinose et hyperparathyroïdie) sans amélioration de croissance. La prévalence de la néphrocalcinose dans notre cohorte est de 31%. Elle est corrélée au nombre d'hypercalciuries et à la posologie de la supplémentation en phosphates. Les patients ayant une néphrocalcinose ont une croissance plus défavorable.CLERMONT FD-BCIU-Santé (631132104) / SudocSudocFranceF

Plasma exchange in Rasmussen's encephalitis

The authors observed a 4-year-old girl who has Rasmussen's encephalitis. She started with frequent localized and generalized seizures. Standard antiepileptic treatment was almost ineffective. The frequency of the generalized seizures decreased, but the myoclonic jerks of the left part of the body persisted. An EEG showed partial status epilepticus. The results of the CT scan were normal. Antibodies to viruses were absent from the blood and cerebrospinal fluid. An MR scan showed a T2-weighted hypersignal zone in the right frontal region. Intravenous bolus injections of corticosteroids and drips of immunoglobulins were inefficient, and we started plasma exchanges which have continued for 9 months. The clinical state stabilized, and the images on the MR scan improved, but the results of the EEG did not improve. The authors discuss the effect of the plasma exchange, the use of which is questionable in this disease

Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study.

Long-term outcome of idiopathic steroid-resistant nephrotic syndrome was retrospectively studied in 78 children in eight centers for the past 20 years. Median age at onset was 4.4 years (1.1-15.0 years) and the gender ratio was 1.4. Median follow-up period was 7.7 years (1.0-19.7 years). The disease in 45 patients (58%) was initially not steroid-responsive and in 33 (42%) it was later non-responsive. The main therapeutic strategies included administration of ciclosporine (CsA) alone (n = 29; 37%) and CsA + mycophenolate mofetil (n = 18; 23%). Actuarial patient survival rate after 15 years was 97%. Renal survival rate after 5 years, 10 years and 15 years was 75%, 58% and 53%, respectively. An age at onset of nephrotic syndrome (NS) > 10 years was the only independent predictor of end-stage renal disease (ESRD) in a multivariate analysis using a Cox regression model (P < 0.001). Twenty patients (26%) received transplants; ten showed recurrence of the NS: seven within 2 days, one within 2 weeks, and two within 3-5 months. Seven patients lost their grafts, four from recurrence. Owing to better management, kidney survival in idiopathic steroid-resistant nephrotic syndrome (SRNS) has improved during the past 20 years. Further prospective controlled trials will delineate the potential benefit of new immunosuppressive treatment

Low incidence of SARS-CoV-2, risk factors of mortality and the course of illness in the French national cohort of dialysis patients

International audienceThe aim of this study was to estimate the incidence of COVID-19 disease in the French national population of dialysis patients, their course of illness and to identify the risk factors associated with mortality. Our study included all patients on dialysis recorded in the French REIN Registry in April 2020. Clinical characteristics at last follow-up and the evolution of COVID-19 illness severity over time were recorded for diagnosed cases (either suspicious clinical symptoms, characteristic signs on the chest scan or a positive reverse transcription polymerase chain reaction) for SARS-CoV-2. A total of 1,621 infected patients were reported on the REIN registry from March 16th, 2020 to May 4th, 2020. Of these, 344 died. The prevalence of COVID-19 patients varied from less than 1% to 10% between regions. The probability of being a case was higher in males, patients with diabetes, those in need of assistance for transfer or treated at a self-care unit. Dialysis at home was associated with a lower probability of being infected as was being a smoker, a former smoker, having an active malignancy, or peripheral vascular disease. Mortality in diagnosed cases (21%) was associated with the same causes as in the general population. Higher age, hypoalbuminemia and the presence of an ischemic heart disease were statistically independently associated with a higher risk of death. Being treated at a selfcare unit was associated with a lower risk. Thus, our study showed a relatively low frequency of COVID-19 among dialysis patients contrary to what might have been assumed