[Complete primary distal renal tubular acidosis in children: 11 cases]. / L'acidose tubulaire distale complete et primitive de l'enfant à propos de 11 observations.

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.

[Graves's disease in children and adolescent: study of seven cases]. / La maladie de Basedow chez l'enfant et l'adolescent: etude de sept cas.

BACKGROUND: In spite of its rarity in the paediatric age, Graves' disease constitutes the principal aetiology of hyperthyroidism in child. AIM: Our goal is to analyze the clinical and evolutive particularities of Graves's disease in children. METHODS: We studied retrospectively seven cases of Graves' disease in children enrolled in the pediatrics department of Sousse during ten years period (1993-2002). RESULTS: There were six girls and one boy (sex - ratio = 0.16) aged 4.5 to 16 years (mean age: nine years and one month). The diagnosis has been established clinically on the presence of classic symptoms of the illness associated to the biological and radiological findings. As part of research of possible associations with this illness, we observed solely in a case, in addition of Graves's disease, the coexistence of Down syndrome and coeliac disease, rarely described. Among the HLA antigens predisposing the Graves's disease, we only found HLA B8 antigen in a patient. The evolution under antithyroid drug treatment (ATD) has been marked by fast disappearance of functional signs in all patients. However, biological and clinical euthyroidism was more difficult to achieve. The treatment has been stopped in only one patient after 40 months period. CONCLUSION: Graves' disease is usually easy to recognize but difficult to treat. Radical treatments (thyroidectomy or radioactive iodine therapy) are indicated in second intention after having tempted ATD beforehand.

[Henoch Schonlein purpura in children: clinical and evolutive study of 122 cases]. / LE purpura rhumatoide de l'enfant : étude clinique et évolutive de 122 cas.

AIM: Analyze the clinical and evolutive particularities of Henoch Schonlein purpura in children METHODS: We studied retrospectively 122 cases enrolled in the pediatrics department of Sousse during 10 years period (1992-2001). RESULTS: It is about 66 boys and 56 girls (sex - ratio= 1.18) aged 3 to 13 years (mean age: 7 years and half). The diagnosis has been established clinically on the presence of cutaneous syndrome with symmetrical declivitous region purpura in all patients with articular syndrome (91cases) and/or digestive syndrome (65cases). Complications were variable: digestive hemorrhage (19 cases), occlusive syndrome (2cases), renal involvement at variable severity (56 cases), scrotal and testicular complications (11 cases), cardiac complications (tamponade in a case). Henoch Schonlein purpura was associated with a primary antiphospholipid syndrome in a case, renal tuberculosis in a case and cholestatic hepatitis A in another case. All patient receeved symptomatic treatment (rest in bed + / - antalgic treatment). Digestive rest was prescribed for 20 patients presenting severe abdominal pains with corticosteroid during 2 at 4 weeks (1-2mg/kg/d) in eight cases. Corticosteroid-cyclophosphamid association was prescribed for 2 patients with severe renal involvement; one of them benefitted of extra-renal purification. One or several relapses of Henoch Schonlein purpura were noted in 13 patients. All sick evolved favorably same those presenting renal or cardiac involvement (middle receding of 5 years).

Oxidant, antioxidant status and metabolic data in patients with beta-thalassemia.

BACKGROUND: In beta-thalassemia major impaired biosynthesis of beta globin leads to accumulation of unpaired alpha globin chain. An iron overload, usually observed, generates oxygen-free radicals and peroxidative tissue injury. AIM: To investigate hematological parameters, oxidative stress and the antioxidant capacity in beta-thalassemia patients compared to control subjects in order to determine their impact in several organs functions. METHODS: This study was conducted on 56 beta-thalassemia major patients compared to 51 healthy subjects. We determined metabolic parameters (glycaemia, lipid parameters, electrolytes, iron indices, hepatic, renal and heart functions tests), plasmatic thiobarbituric acid reactive substances (TBARS), plasmatic peroxyl radical trapping potential (TRAP), plasmatic superoxide dismutase (SOD), erythrocyte gluthathione peroxidase (GPX), plasmatic vitamin E, vitamin A and trace elements. RESULTS: Except triglycerides, lipid fractions were significantly decreased in beta-thalassemia compared to controls. Serum ferritin, iron, TBARS concentrations, SOD and GPX activities were significantly increased. But TRAP, vitamin E and zinc concentrations were significantly decreased. CONCLUSION: Our findings confirm the peroxidative status generated by iron overload in beta-thalassemia major patients and highlight the rapid formation of marked amounts of TBARS and the increase of SOD and GPX activity. Our study suggested that in beta-thalassemia the first organ impaired is the liver.