• Samia Hamouda, FredjSondess HadjSHBechir Hamza Children's Hospital of Tunis, Department B., Sonia Hilioui, Fatma Khalsi, AmeurSalma BenSBHedi Chaker Hospital., Jihene Bouguila, Raoudha Boussoffara, Habib Besbes, Houda Ajmi, Nadia Mattoussi, Taieb Messaoud, Ahmed Mehrezi, Mongia Hachicha, Lamia Boughamoura, Mohamed Taher Sfar, Neji Gueddiche, Saoussen Abroug, Saida Ben Becheur, Sihem Barsaoui, Neji Tebib, Azza Samoud, Najoua Gandoura, Faten Tinsa, and Khadija Boussetta.
• Bechir Hamza Children's Hospital of Tunis, Department B.
• Afr Health Sci. 2020 Mar 1; 20 (1): 444452444-452.

AimTo establish a preliminary national report on clinical and genetic features of cystic fibrosis (CF) in Tunisian children as a first measure for a better health care organization.MethodsAll children with CF diagnosed by positive sweat tests between 1996 and 2015 in children's departments of Tunisian university hospitals were included. Data was recorded at diagnosis and during the follow-up from patients' medical records.ResultsIn 12 departments, 123 CF children were collected. The median age at diagnosis was 5 months with a median diagnosis delay of 3 months. CF was revealed mostly by recurrent respiratory tract infections (69.9%), denutrition (55.2%), and/or chronic diarrhea (41.4%). The mean sweat chloride concentration was 110.9mmol/L. At least one mutation was found in 95 cases (77.2%). The most frequent mutations were Phe508del (n=58) and E1104X (n=15). Fifty-five patients had a Pseudomonas Aeruginosa chronic colonization at a median age of 30 months. Cirrhosis and diabetes appeared at a mean age of 5.5 and 12.5 years respectively in 4 patients each. Sixty-two patients died at a median age of 8 months. Phe508del mutation and hypotrophy were associated with death (p=0.002 and p<0.001, respectively).ConclusionCF is life-shortening in Tunisia. Setting-up appropriate management is urgent.© 2020 Hamouda S et al.

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