African Health Sciences
Makerere University Medical School
Vol. 18, No. 3, 2018, pp. 664-670
Bioline Code: hs18083
Full paper language: English
Document type: Study
Document available free of charge
African Health Sciences, Vol. 18, No. 3, 2018, pp. 664-670
© Copyright 2018 - Boussetta et al.
Cystic fibrosis in Tunisian children: a review of 32 children|
Boussetta, Khedija; Khalsi, Fatma; Bahri, Yasmine; Belhadj, Imen; Tinsa, Faten; Messaoud, Taieb Ben & Hamouda, Samia
Background: Cystic fibrosis is rare in Tunisia.Its diagnosis requires experienced specialists. Its prognosis is poor in developing
Objectives: To study the epidemiologic, clinical, genetic features and the therapeutic challenges of cystic fibrosis in Tunisian
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’s 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.
Cystic fibrosis; children; Tunisia.