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Cystic fibrosis in Tunisian children: a review of 32 children

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...

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Detalles Bibliográficos
Autores principales: Boussetta, Khedija, Khalsi, Fatma, Bahri, Yasmine, Belhadj, Imen, Tinsa, Faten, Messaoud, Taieb Ben, Hamouda, Samia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Makerere Medical School 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307014/
https://www.ncbi.nlm.nih.gov/pubmed/30602999
http://dx.doi.org/10.4314/ahs.v18i3.24
Descripción
Sumario: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.