Background: Cystic fibrosis is rare in Tunisia.Its diagnosis requires
experienced specialists. Its prognosis is poor in developing countries.
Objectives: To study the epidemiologic, clinical, genetic features and
the therapeutic challenges of cystic fibrosis in Tunisian children.
Methods: Covering a period of 21 years, this retrospective study
included all patients with a definite diagnosis of cystic fibrosis from
the Pediatrics Department B of The Children\u2019s Hospital of Tunis.
Results: Data from 32 children (14 boys and 18 girls) were collected.
The diagnosis was made during the first year of life in 28 cases.
Meconium ileus was found in 5 cases, respiratory manifestations in 22
cases, chronic diarrhea in 19 cases, faltering growth in 17 cases and a
pseudo Barter syndrome in 2 cases. The sweat chloride test was positive
in all cases. The most frequent mutation was F508del (56% of cases).
Respiratory complications marked the outcome. Among our 32 patients, 15
patients (50%) died at an average age of 5 years and 3 months, mainly
due to respiratory failure. The mean age of the surviving patients was
5 years. Conclusion: Cystic fibrosis prognosis is poor in our series
compared to developed countries due to the longer diagnostic delay and
the limited therapeutic options