Author:
Hamouda Samia,Hadj Fredj Sondess,Hilioui Sonia,Khalsi Fatma,Ben Ameur Salma,Bouguila Jihene,Boussoffara Raoudha,Besbes Habib,Ajmi Houda,Mattoussi Nadia,Messaoud Taieb,Mehrezi Ahmed,Hachicha Mongia,Boughamoura Lamia,Taher Sfar Mohamed,Gueddiche Neji,Abroug Saoussen,Ben Becheur Saida,Barsaoui Sihem,Tebib Neji,Samoud Azza,Gandoura Najoua,Tinsa Faten,Boussetta Khadija
Abstract
Aim: To 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.
Methods: All 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.
Results: In 12 departments, 123 CF children were collected. The median age at diagnosis was 5 months with a median diag- nosis 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).
Conclusion: CF is life-shortening in Tunisia. Setting-up appropriate management is urgent.
Keywords: Cystic fibrosis epidemiology; Tunisia.
Publisher
African Journals Online (AJOL)
Cited by
5 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献