[Caustic esophagitis in children: about 86 cases]

Caustic ingestion is a relatively frequent accident in infants. The most redoubtable complication is the oesophageal stenosis which requires a multidisciplinary management. The aim of this work is to study epidemiological, clinic, endoscopic, therapeutic aspects and out comes of caustic esophagitis. We report a retrospective study about 86 cases of caustic esophagitis registered in the paediatric service of Ibn El Jazzar's hospital of Kairouan during the period between 1[st] January 1996 and 31[st] December 2006. The hospital prevalence was 0.3per cent. The median age was 4 years. The most incriminated caustic products were bleach and soda. Clinic examination was more often poor. Among the 86 patients, 41 had a serious caustic esophagitis, all of them received great doses of methyl-prednisolone. 12 patients developed a stenosis. Pneumatic dilatation was done for 9 patients with good results and two patients underwent surgery. However, prevention represents the only way to minimize the dramatic consequences of caustic ingestion on the patient him self, his surroundings and the society

[Wilson's disease in children - about 21 cases]

Wilson's disease is a familial affection, described the first time in 1912, which has an autosomic recessive transmission. It is characterized by an association of hepatic cirrhosis and neurologic manifestations caused by tissular accumulation of copper. To show clinical, genetic, diagnostic, therapeutic particularities and outcomes of this disease, we carried out a retrospective study concerning three pediatric departments in the Center of Tunisia [Kairouan, Farhat Hached and Sahloul hospitals]. We have collected 21 cases during a period of 17 years [from January 1983 to december 2000]. The finding's age of the disease ranges from 5 to 13 years [mean age 8 years and 9 months]. The sex ratio is 1,1. Consanguinity is found in 90% of cases. Finding circumstances are dominated by edematous and ascitic syndrome [28%] and by hepatomegaly [57.14%] a jaundice [19,5%], a pallor [9.52] and neurologic disorders [19% of cases]. A hepatic cytolysis was found in 38% and a decrease of prothrombin rate in 61% of cases. The ceruloplasmin concentration is lower than 200 mg/1 in 80% and a decrease in the blood rate of copper in 75% of cases. The urinary rate of copper was measured only in 9 patients; it was increased in 8 cases. The therapy consisted in D penicillamin in the first line. Outcome was marked by 3 cases of death. 18 patients are followed up and they are treated by D penicilamin

[ leucinosis-Study of seven cases]

Maple syrup urine disease [MSUD] is an antosomal recessive metabolic disorder caused by decreased activity of this branched chain alpha-ketacid dehydrogenase. Work object is to clear the clinical, therapeutical and evolutive characteristics of this disease, through a retrospective study of seven patients identified over a 12 years period in Kairouan Pediatric department. The mean age of diseasis revelation was 7 days [2 to 12 days]. The consanguinity was found in 5 cases, neurological diseas dominate the clinical manifestations. The DNPH test was positive in five cases. The diagnosis was confirmed by the AAC in blood and OAC in urines. All the 7 new born died in a mean age of 21 days. The light incidence, problems of management and the poor prognosis underline the improtance of antenatal diagnosis