RESUMO
AIM: Analyze the clinical and evolutive particularities of complete primary distal renal tubular acidosis in children, METHODS: We studied retrospectively 11 cases enrolled in the pediatrics department of Sousse during 10 years period (1993-2002). RESULTS: It is about 9 boys and 2 girls (sex-ratio = 45) aged 3 month to 5 years (mean age: 18 months). Diagnosis was suspected on clinical and biological data of presumption and confirmed by acidification test. Radiological investigation objectified a nephrocalcinosis in eight patients and urinary lithiasis in two other cases. Auditive exploration showed sensorineural deafness in three patients. The illness appears sporadic in two cases and autosomal recessive in nine other cases. After alkali treatment (sodium bicarbonate), evolution was globally favorable.