Clinical characteristics and outcome of cystic fibrosis: report of 16 cases

The aim of this report is to determine clinical characteristics and outcome of Cystic Fibrosis [CF]. Cases of CF managed at Infantile Medecine A Department in Children's Hospital of Tunis during 13 years [1994 -2006] were reviewed. 16 children had CF. They were 8 males and 8 females. 13 patients were consanguineous and four had similar familial cases. The mean age at diagnosis was 19 months [10 days, 13 years]. 3/4 of patients were symptomatic within the first trimester of life. Revealing symptoms were: obstructive bronchopathy associated to chronic diarrhea [n = 6], oedema-anemia-hypotrophy-hypoprotidemia syndrome [n = 3], meconium ileus [n = 4], bronchiectasis [n = 2] and chronic diarrhea [n = l]. The diagnosis was confirmed by sweat test and genotypic data. The F508 del was the most frequent mutation [54%]. Clinical outcome was characterized by the occurrence of respiratory and nutritional complications: acute respiratory failure [n = 6], chronic respiratory failure [n = 3], chronic pseudomonas aeruginosa infection [n = 6] at a medium age of 3.8 years, recurrent haemoptysis [n = 2], pleural effusion [n = 2], a malnutrition [n = 10] and diabetes associated to puberty delay in one patient. Seven patients died at mean age of 4.4 years [6 months, 17.3 years]. Among surviving patients, six had no compromised nutritional status or lung function. Prenatal diagnosis was performed in three families. CF is characterized by earliest onset and severity of symptoms. Therapeutic insufficiency is the main cause of precocious complications and poor prognosis in our series

[Prevalence and causal factors of anemia in children in Tunisia]

Anemia continue to be prevalent among children under five years despite the improvement of socioeconomic and sanitary indicators. The purpose of the present cross-sectorial study is to assess the etiologic factors responsible for anemia. Knowledge of the relative importance of the different causes should form a basis for intervention strategies to prevent and control anemia. The survey covered 955 children under the age of five years, native of two regions with the highest prevalence of anemia, the Southwest and the District of Tunis. The results showed that 29% of children suffered from anemia. About 70% of them were iron deficient. The fractions of the deficiency in vitamin B 12 and in folates were insignificant Only 3% of children had chronic inflammation associated with [and possibly responsible for] their anemia A little fraction of anemia [=5%] was due to thalassemia or drepanocytosis. Picawasan important causal factor of iron deficiency anemia. The parasites identified in stool could not cause anemia

mucoviscidose chez l'enfant: Experience d'un service Tunisien de pediatrie generale

Cystic fibrosis was regarded a long time as exceptional in the Nord Africaine population and in particular in Tunisia what was at the origin of the ignorance of its various diagnostic and 'therapeutic aspects in our country. Nevertheless, with the development of the means of the diagnosis, several cases of cystic fibrosis were diagnossd these last years what will pose true problems of assumption of responsibility of these children like illustrates it well our experiment in the pediatric department of Sfax university hospital. In 10 years going of 1991 to 2000 we reported 7 cases of cystic fibrosis in the peditric department of Sfax university hospital. Our patients are 3 boys and 4 girls. The age of revelation of the disease varied from 3 months to 14 years with 4 years and 10 months an average age. All our patient had a respiratory symptomatology in the Foreground. The evolution was marked by the death of 4 patients at an average age of 5 years and half whereas the 3 surviving patients for the moment are more or less balanced on the nutritional level, digestive and respiratory. Cystic fibrosis is not exceptional in our area, its assumption of responsibility therapeutic is difficult, and it requires the collaboration of several experts and a good compliance of the child and of his family