Cystic fibrosis in Tunisian children: a review of 32 children.
Afr Health Sci
; 18(3): 664-670, 2018 Sep.
Article
in En
| MEDLINE
| ID: mdl-30602999
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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Cystic Fibrosis
Type of study:
Observational_studies
/
Risk_factors_studies
Limits:
Child, preschool
/
Female
/
Humans
/
Male
Country/Region as subject:
Africa
Language:
En
Journal:
Afr Health Sci
Journal subject:
MEDICINA
/
SERVICOS DE SAUDE
Year:
2018
Document type:
Article