[Primary type 1 hyperoxaluria - about two particular cases]

Primary hyperoxaluria type I is an autosomal recessive disease due to congenital defect in alanine glyoxylate aminotransferase [ACT]. It is a rare disorder in witch only combined liver-kidney transplantation is curative. Our two cases of primary type I hyperoxaluria were particular by the early renal failure, thrombocytopenia and intestinal invagination. On the basis of our two cases we discuss diagnostic and therapeutic methods

[Bronchiectasis in children - about 29 cases]

Bronchiectasis is defined by a permanent dilatation of the bronchi. The aim of this study was to precise the epidemiological, clinical particularities, evolution and management of bronchiectasis. It is a retrospective study of 29 children admitted in the pediatric departement of the university hospital in Monastir, for bronchiectasis withing a period of 11 years [1992-2002]. The mean age of children was 6 years and 51,7% of them were female. The clinical signs were dominated by recurring disease of the lungs, chronic cough and expectoration. The X ray radiography of the chest was abnormal in all cases [nests sign in 55,2% of cases]. The bronchiectasis was bilateral in 55,18% of cases and unilateral in 44,82% of cases. Causes were found in 65,5% of cases [congenital bronchiectasis in 20,7% of cases]. The management was firstly medical in all cases and surgical in 20,7% of cases. The outcome was favorable in 69% of cases and unfavorable in 31% of cases. Mortality rate, post surgical recurrence and aggravation of the disease were observed respectively in 10,3%, 3,4% and 17,3% of cases. The management of broncheactasisis still based on prevention. We insist on vaccination, bronchoscopy after any inhalation of foreign body and adequate treatment of respiratory infections in children