Khedija Boussetta¹, Fatma Khalsi¹, Yasmine Bahri¹, Imen Belhadj¹, Faten Tinsa¹,

Taieb Ben Messaoud², Samia Hamouda¹

1. Bechir Hamza Children’s Hospital of Tunis, Pediatrics Department B.
2. Bechir Hamza Children’s Hospital of Tunis, Biochemestry Department.

Abstract

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’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.

Keywords: Cystic fibrosis, children, Tunisia.

DOI: https://dx.doi.org/10.4314/ahs.v18i3.24

Cite as: Boussetta K, Khalsi F, Bahri Y, Belhadj I, Tinsa F, Messaoud TB, Hamouda S. Cystic fibrosis in Tunisian Children: a review of 32 children. Afri Health Sci. 2018;18(3): 664-670. https://dx.doi.org/10.4314/ahs.v18i3.24