Search

Aims: To estimate the incidence and describe the clinical presentation and outcome (steroid responsiveness, clinical course, complications) of idiopathic nephrotic syndrome in children in a population-based retrospective study., Methods: Using local registries and the hospital discharge diagnosis system from two centers, all new cases of idiopathic nephrotic syndrome were identified in Gironde (France) between January 1992 and May 2008. To estimate incidence, population-based denominators were obtained from the National Institute for Statistics and Economic Studies (INSEE). Clinical data were collected from medical charts., Results: Ninety-nine cases of idiopathic nephrotic syndrome were reported (66 boys, 18 non-Caucasians) with an incidence of 2.3/100,000 (CI, 1.8-3.0) children less than 15 years. Ninety patients (91%) had steroid-sensitive nephrotic syndrome (SSNS) and nine (9%) were steroid-resistant (SRNS). The median time to remission in SSNS was 11 days. Relapses occurred in 75 (83%) children with SSNS with a median of four relapses (range, 1-32). The cumulative relapse-free incidence was 60% at 10 years after diagnosis in SSNS and 13% of patients aged 18 years old or over still had active disease. In SSNS, the only significant factor associated with steroid dependency or use of non steroid drugs was the time to initial response to steroids greater than 14 days. Nineteen children (19%) experienced severe complications of nephrotic syndrome including 11 bacterial infections and two thromboembolic complications. Two children with SRNS, of whom one was initially steroid-responsive, developed end-stage renal failure., Conclusion: The incidence and outcome of idiopathic nephrotic syndrome in Gironde are comparable to the rates found in other studies. The disease may have a long course and the time for response to steroids at disease onset is the main predictor of steroid dependency and of use of non steroid agents., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Objective: To evaluate the use of neonatal central venous catheters (CVC) in 38 french neonatal units and occurrence of pericardial effusion (PCE) over the past 5 years., Materials and Methods: We surveyed 38 units with a questionnaire and studied the cases of PCE in five units., Results: Response rate was 89% (34/38). Accepted CVC tip positions were: junction of right atrium (RA) and vena cava (VC) 76%, VC 58%, RA 11%. Fifty percent of the centers had been exposed to PCE. 16 cases of PCE were studied. Median gestational age was 31 weeks (range: 26.1 to 40 weeks). Median time from insertion: 3.2 days (range: 0.4-13.5). In all cases CVC tip was intracardiac at insertion with inadequate withdrawing in 13 cases. Sudden cardiac collapse was reported in eight cases, and unexplained cardiorespiratory instability in six cases. Echography showed PCE in 14 cases. One diagnosis was post-mortem. CVC was withdrawn in 12 patients and 13 underwent pericardiocentesis. Four patients died and two had neurological sequelae., Conclusion: PCE was associated with intracardiac CVC tip. The CVC tip should be controlled with radiography or echography outside the cardiac silhouette. PCE diagnosis must be considered in face of unexplained cardiovascular decompensation of neonate with CVC.

On the occasion of 2 cases, torsion of an ovarian cyst and volvulus of a cystic intestinal duplication, the authors emphasize the usual benignity of fetal intra-abdominal solitary cystic pictures, among which the most frequent are the ovarian cysts. Antenatal diagnosis allows for detection of complications, especially torsions and for early neonatal management always leading to surgical treatment.

After analysis of the results of 142 CT brains scans performed from 1976 to 1982 on children with acute lymphoblastic leukemia, we can propose indications for this exam in relation with the different clinical stages and evolution of the disease. An exam is carried out as soon as possible at the start of the disease without injection of contrast media for use as a reference point. Excepting complications or neurological localisations, a scan will be performed at the end of the third year of treatment, without injection of contrast media, in screening for possible iatrogenic lesions. Neurological complications of unknown origin and leucoblastic meningitis seems to require a scan with injection of contrast media firstly as a mean of diagnosis, and secondly, in case of a cerebro meningeal localisation, in order to judge the efficiency of treatment and to screen for early complications. Computerized tomography however has its limits, particularly in case of purely meningeal leucoblastic infiltration.

Three cases of subcutaneous fat necrosis of the newborn (SFNN) are presented. They occurred after a therapeutic coma induced by intravenous barbiturates to control convulsions unresponsive to conventional treatment. Two patients had widespread lesions. Histopathology showed prominent vascular involvement. Since 1970, 26 cases of SFNN have been published. Among the aetiological factors already described, hypothermia and ischaemia play a major role. It is suggested that brown adipose tissue might be of importance in the pathophysiology of subdural fat necrosis of the newborn.

Three cases of meningitis with E. coli and H. influenzae are reported. They illustrate the recrudescence and relapse in bacterial meningitis. After the failure of initial antibiotic therapy, the treatment by Cefotaxime allowed a good outcome in all the cases. On account of the bacteriological, pharmacological and clinical data the authors advise using Cefotaxime in first purpose in meningitis with common gram negative bacilli.