[Childhood steroid-dependent idiopathic nephrotic syndrome: Predictive factors for the need of immunosuppressive treatment]. / Syndrome néphrotique idiopathique corticodépendant de l'enfant : facteurs prédictifs de recours à un traitement immunosuppresseur.

BACKGROUND AND AIMS: More than half of the children with idiopathic nephrotic syndrome become steroid-dependent (or frequent relapsers) and will later require the use of complementary treatment aiming to reduce steroids' side effects and to limit the number of proteinuria relapses. It appears important to identify these children as early as possible in order to adapt their treatment. The aim of this study was to analyze the population of children, under 18 years of age, diagnosed between 1/01/2000 and 31/05/2015 with an idiopathic nephrotic syndrome and followed at the Montpellier University Hospital to search for criteria predictive of steroid-sparing agent use. METHODS: In this retrospective study of children with idiopathic nephrotic syndrome, the exclusion criteria were primary steroid resistance and children with no proteinuria relapse after diagnosis. RESULTS: Eighty-four children (54 boys) were included in this study. The mean follow-up duration was 5.5 years (0.75-16). The mean age at diagnosis was 4.6 years. Sixty-five children (77%) received at least one steroid-sparing agent during their follow-up, within a mean 10 months after diagnosis. In these patients, the first relapse of the disease occurred earlier when compared with the children who were maintained on steroid alone (4 months vs 7 months; P<0.001). The use of methylprednisolone pulses to obtain a remission, the cumulative dose of steroid treatment, and the number of proteinuria relapses were also significantly correlated with the use of complementary immunosuppressive therapy. CONCLUSION: We found no predictive criteria of the use of steroid-sparing agents at diagnosis in our population of children. Nevertheless, with the steroid regimen used, the time within which the first proteinuria relapse occurred appears to be a significant criterion for the secondary use of a steroid-sparing agent. These data should be taken into account when choosing the treatment regimen.

[Hemolytic uremic syndrome with severe neurological involvement: how should it be managed?]. / Syndrome hémolytique et urémique avec atteinte neurologique sévère: quelle prise en charge?

INTRODUCTION: The management of diarrhea-associated hemolytic and uremic syndrome (D(+) HUS) with severe acute neurological involvement continues to be debated. We report on 2 cases and discuss the treatment. CASE REPORT 1: A 2.5-year-old girl presented with generalized seizures during gastroenteritis. Blood tests revealed features of HUS and a pyramidal syndrome was found on physical examination. Brain MRI, 24h after admission, showed lesions in the periventricular and subcortical area. She was started on peritoneal dialysis (PD) and daily plasma exchanges (PE) for 10 days. Her neurological condition improved quickly as well as the findings of the second brain MRI performed after PE. One year later she had no apparent neurological or renal sequelae. CASE REPORT 2: A 2.5-year-old boy presented with generalized seizures during gastroenteritis for 3 days, leading to a diagnosis of HUS. He also had a severe pyramidal syndrome with spastic tetraparesis and aphasia. Brain MRI, 48 h after admission, revealed severe bilateral and symmetric lesions involving the thalami, internal and external capsules, lenticular nuclei, and brainstem. He was started on PD and daily PE for 10 days. Brain MRI performed after PE was unchanged. Clinically, his neurological condition improved slowly with regression of spastic tetraparesis and progressive recovery of motor skills. Nine months later, his renal function is normal but he is still having intensive physiotherapy. DISCUSSION: Both children have received similar management including 10 PEs started within 48 h after the diagnosis of D(+) HUS with severe neurological involvement, but their neurological outcome appeared to be significantly different. There is no clear proof in the literature concerning the effects of PE in such patients, even when performed very early. Eculizumab, an antibody that inhibits complement factor 5a and the formation of the membrane attack complex, has recently been used in such cases and seems to provide a more specific therapeutic action. Control studies are needed to specify its use in this disease.

[Posterior urethral valves: prenatal diagnosis, neonatal data and outcome]. / Les valves de l'urètre postérieur. Etat des lieux en Languedoc-Roussillon de 1998 à 2007 : données anténatales, néonatales et devenir à moyen terme.

AIM OF THE STUDY: To evaluate the impact of prenatal diagnosis on the epidemiology and outcome of children with posterior urethral valves (PUV), considering that today termination of pregnancy may be proposed in the most severe cases. PATIENTS AND METHODS: Forty-three cases of patients with PUV were diagnosed between 1998 and 2007 in the Languedoc-Roussillon region. In this study, we detailed the prenatal data and postnatal outcome of those patients with a mean follow-up period of 7.6 years. RESULTS: Medical interruption of pregnancy was performed in 15 severe cases. One death in utero was also observed. Twenty-seven patients were managed postnatally and one child died during the neonatal period because of lung hypoplasia. For the 26 remaining patients at the end of the follow-up period, 5 had mild renal insufficiency with a creatinine clearance calculated with the Schwartz formula of less than 80 ml/min/1.73 m2. One reached end-stage renal failure at the age of 7 years. Proteinuria and hypertension were rare. DISCUSSION AND CONCLUSION: Today, because of the increase in medical termination of pregnancy in the most severe cases, most of the children born alive with PUV experienced a better renal prognosis when compared with previous data. Prenatal diagnosis and therapeutic progress significantly modified the epidemiology and outcome of this disease. Nevertheless, the best predictor of renal outcome remains renal function at 1 year of age.

Antibiotic prophylaxis for the prevention of recurrent urinary tract infection in children with low grade vesicoureteral reflux: results from a prospective randomized study.

PURPOSE: Antibiotic prophylaxis is given to children at risk for urinary tract infection. However, evidence concerning its effectiveness in grade I to III vesicoureteral reflux is lacking. The objective of this study was to determine whether antibiotic prophylaxis reduces the incidence of urinary tract infection in young children with low grade vesicoureteral reflux. MATERIALS AND METHODS: Children 1 month to 3 years old with grade I to III vesicoureteral reflux were assigned randomly to receive daily cotrimoxazole or no treatment, and followed for 18 months. A urinary tract infection constituted an exit criterion. Infection-free survival rates were calculated using the Kaplan-Meier method and compared using the log rank test. RESULTS: A total of 225 children were enrolled in the study. Distribution of gender, age at inclusion and reflux grade were similar between the 2 groups. There was no significant difference in the occurrence of urinary tract infection between the 2 groups (17% vs 26%, p = 0.2). However, a significant association was found between treatment and patient gender (p = 0.017). Prophylaxis significantly reduced urinary tract infection in boys (p = 0.013), most notably in boys with grade III vesicoureteral reflux (p = 0.042). CONCLUSIONS: These data suggest that antibiotic prophylaxis does not reduce the overall incidence of urinary tract infection in children with low grade vesicoureteral reflux. However, such a strategy may prevent further urinary tract infection in boys with grade III reflux.

[Association of severe bronchial disease (bronchial casts, bronchiectasis) and partial abnormal pulmonary venous drainage in 2 children with Turner's syndrome]. / Association d'une bronchopathie sévère (moules bronchiques, dilatation des bronches) et d'un retour veineux pulmonaire anormal partiel à un syndrome de Turner chez deux enfants.

UNLABELLED: The prevalence of cardiovascular malformation is high in Turner's syndrome. We report two cases of partial anomalous pulmonary venous drainage associated with a severe bronchial disease. CASE REPORTS: Case 1: a 5-year-old girl with monosomy X presented with acute respiratory failure. The chest X-ray showed an atelectasis of the left lower lobe. The patient improved following bronchoscopic removal of a bronchial cast. During hospitalization, an anomalous pulmonary venous drainage of the right lung was discovered. In spite of complete surgical repair of the cardiac malformation, several episodes of respiratory failure occurred several years after the operation. Case 2: a 9-year-old girl with monosomy X and coarctation of the aorta underwent pre-operative cardiac catheterization. She had a history of bronchiectasis, leading to a lobectomy. Her respiratory condition improved following surgery. A partial anomalous pulmonary venous return was discovered during the cardiac angiography. The coarctation was repaired surgically. The correction of the left-to-right shunt was not necessary. CONCLUSION: The partial anomalous pulmonary venous return is frequently reported in Turner's syndrome. The possible respiratory disease associated with this cardiac malformation is usually described as a consequence of the increase in pulmonary blood flow. Our two cases did not match this hypothesis.

[Hematuria revealing primary hyperparathyroidism in a child]. / Hématurie révélatrice d'une hyperparathyroïdie primaire chez l'enfant.

BACKGROUND: Hematuria rarely reveals primary hyperparathyroidism in the child. CASE REPORT: A 10-year-old boy presented with gross hematuria and urolithiasis. Biological findings showed hypercalcemia and hypercalciuria. Initially, parathormone plasma levels remained within normal range, but raised after a few months and at that time, ultrasound scan examination showed a parathyroid adenoma. Nevertheless, two surgical explorations were necessary to cure hypercalcemia and hyperparathyroidism. Histological aspects of parathyroid adenoma were not found on pathological examination. CONCLUSION: Symptomatic hypercalciuria may be the first symptom revealing primary hyperparathyroidism in children. Surgical treatment remains difficult in such a case.