[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

[Idiopathic thrombocytopenic purpura. Study of 61 cases]

Idiopathic thrombocytopenic purpura [ITP] represented the most frequent cause of children's thrombocytopenia. Until our days universal management of ITP does not exist. We performed retrospective and then prospective study of 61 children admitted for ITP during 15 years. The aim of the study was to precise the epidemiological, clinical and therapeutic profiles and propose management algorithm. The incidence of ITP was 0.34 per cent. The mean age was 6 years with male predominance. Initial thrombocytopenia was inferior than 10.000/mm3 in 44,2 per cent of cases. Spontaneous recovering was noted in 17 cases. Oral prednisone therapy with 2mg/kg/day during 21 to 35 days and 4mg/kg/day during 4 days was performed respectively in 21 and 19 cases. Intravenous immunoglobulin and methylprednisolone pulse therapy was performed respectively in 17 and 5 cases. Splenectomy was successfully performed in 3 cases. Chronic ITP was noted in 13 cases. Acute ITP without hemorrhagic syndrome should be treated in first stage with oral prednisone at 4mg/kg/day during 4 days. In case of failure, a second cure may be prescribed. If not we return to intravenous immunoglobulin

[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

[Febrile seizures in child. A retrospective study. About 511 cases]

The aim of this study was to analyse epidemiological, clinical profile of febrile seizures, to identify indications of laboratory studies and EEG examination, and to assess risk of recurrence, and epilepsy. A retrospective study of 511 children aged from three months to five years who presented with a first febrile seizure hospitalized in the pedatrie department of Monastir Hospital from the 1[st] January 1990 to 31[th] of December 1999 was conducted. Febrile seizure represents 4, 9% of all hospitalized children between 1990 and 1999. 78% of children aged from three months to two years, sex ratio was 1, 27 with 286 boys and 225 girls. In 99, 4% of cases, febrile seizure was generalized, and tonic clonic in 82, 2%. In our study seizures [86,3%] were considered simple, although those focal onset, prolonged duration, or that occur more than once within the same febrile illenes were considered complex in 13, 7%. Recurrence was observed in 9, 9% and only 1, 2% developed epilepsy

[Compartive study of the effectiveness of ordinary phototherapy [OP] versus intensive phototherapy [IP] in the treatment of hyperbilirubinemia due to abo incompatibility]

Jaundice is the common clinical sign in neonatal medicine. Its causes were dominated by ABO incompatibility. The aim of the study was to compare the effectiveness of ordinary phototherapy [OP] versus intensive phototherapy [IP] in the treatment of hyperbilirubinemia due to ABO incompatibility. A total of 104 newborns infants with haemolytic hyperbilirubinemia were evaluated. Group I [G I], 52 infants treated with OP or OP and exchange transfusion and group II [G II], 52 infants treated with IP or IP and OP. Our study demonstrated that the overall percentage decline rate of unconjugated serum bilirubin was 0.31 %/h in G I and 0.7 %/h in G II. Median duration of phototherapy was shorter in G II [16h vs 47.3h]. The use of continue OP was reduced in G II [7 vs 31 cases]. Exchange transfusion was no longer used in G I. Also we found significant decrease of the mean duration of hospitalisation in G II [4.54 vs 5.15 days] This study showed that in G II of jaundiced babies treated with IP, kernicterus was totally eliminated also the need of exchange transfusion. The use of IP reduced mother-baby separation