[Canavan disease or N-acetyl aspartic aciduria: a case report]. / La maladie de Canavan ou acidurie N-acétyl aspartique: à propos de 1 cas.

Canavan disease or N-acetyl aspartic aciduria, is an autosomal recessive leukodystrophy characterized by spongy degeneration of brain. The disease is an inborn error of metabolism caused by aspartoacylase deficiency resulting from accumulation of N-acetyl aspartic acid in the brain. The authors report a case in a 10-month-old boy who presented with developmental delay and megalencephaly noticeable after 4 months of age. Magnetic resonance imaging of the brain showed diffuse white matter degeneration. The diagnosis of Canavan disease was confirmed by nuclear magnetic resonance spectroscopy and gas chromatography-mass spectrometry.

[Pneumococcal meningitis mortality in children. Prognostic factors in a series of 73 cases]. / Mortalité par méningites à pneumocoque chez l'enfant. Facteurs pronostiques à propos d'une série de 73 observations.

OBJECTIVE: Despite advances in antibiotic therapy strategies and pediatric intensive care, prognosis of Streptococcus pneumoniae meningitis remains very poor. To determine the factors associated with hospital mortality of children with pneumococcal meningitis. METHODS: We conducted a retrospective study of 73 cases of childhood pneumococcal meningitis admitted in 4 teaching hospitals in the center of Tunisia during a 8-year period (1995-2002). RESULTS: Hospital mortality was 13.7% (10 of 71 patients), and neurologic sequela were observed in 34.5% of survivors. Based on univariable analysis, five variables were associated with the outcome: Pediatric Risk of Mortality score (p < 0.001), coma (p=0.0009), use of mechanical ventilation (p=0.0001), convulsions (p = 0.0449), and shock (p=0.0085). In multivariable analysis, only 2 factors were independently associated with in-hospital mortality: Pediatric Risk of Mortality score and the use of mechanical ventilation. 11.8% of pneumococcal isolates were intermediate and resistant to penicillin. Non-susceptible pneumococcus strains to penicillin and the use of steroids were not associated significantly with the mortality rate. CONCLUSIONS: Pneumococcal meningitis remains a devastating childhood disease. Two variables were independently associated with the in-hospital death in our series (high Pediatric Risk of Mortality score, and the use of mechanical ventilation). According to these data we may recommend the inclusion of vaccination against streptococcus pneumonia in the children's immunization program in Tunisia.

[Epidemiological and bacteriological characteristics of uropathogen bacteria isolated in a pediatric environment]. / Caractéristiques épidémiologiques et bactériologiques des bactéries uropathogènes isolées dans un milieu pédiatrique.

Urinary tract infection (UTI) in children is a grave pathology, which requires a fast and effective care. Bacteriological and epidemiological data play a determining role in patient's care. We report a retrospective study, which spreads out from January 1st till August 31st, 2009, having concerned hospitalized children for urinary infection in pediatrics service of Farhat Hached teaching hospital in Sousse. Our series contained 51 children with a sex ratio of 0.76, an average age of 32 months. The majority of cases was pyelonephritis (94.1%). A pathology is associated with the urinary infection in 41.2%. Three cases of vesico-ureteral reflux were noted. The diagnosis of urinary infection was confirmed by cytobacteriological exam of urine (CBEU). Enterobacteriacea were isolated in 96.1%. Escherichia coli remains the most often isolated (80.4%), followed by Klebsiella pneumoniae (9.8%) then by Proteus mirabilis (5.9%). E. coli was resistant to amoxicillin in 78% of cases, to the association amoxicillin-clavulanic acid in 64,8%, to cephalosporins of 3(rd) generation (C3G) in 5% and to cotrimoxazole in 51%. No K. pneumoniae or P. mirabilis strain was resistant to C3G. UTI in children is always an indication for CBEU realization at first intention. The results of this exam are very important considering the diversity of the responsible bacteria and the growing frequency of acquired antibiotic resistance.