title: [Cystic fibrosis: A report of 33 pediatric Tunisian cases].
abstract: BACKGROUND
  The frequency of cystic fibrosis is unknown in Tunisia, regarding the limited number of reported surveys and patients.
  
  
  OBJECTIVE
  to determine the clinical characteristics, outcome and genetic data of cystic fibrosis in Tunisian pediatric patients.
  
  
  METHODS
  Cases of cystic fibrosis managed at pediatric departments of Tunis, during 15 years (1997-2012), were reviewed.
  
  
  RESULTS
  33 children (23 males and 10 females) were enrolled. The Onset was within the first year of life in 26 patients. Revealing symptoms were the following: recurrent bronchopneumonia (28 cases), chronic diarrhea (17 cases), hepatomegaly (6 cases), malnutrition (15 cases), pseudo Bartter syndrome (3 cases), edemaanemia- hypoprotidemia (4 cases) and meconium ileus (4 cases). The diagnosis was confirmed by sweat test and genotypic data, the F508 del was the most frequent mutation (17 cases). Several complications had occurred during follow-up: chronic pseudomonas aeruginosa infection (15 cases), chronic respiratory failure (14 cases), recurrent hemoptysis (2 cases), pleural effusion (3 cases) and cirrhosis (2 cases). Ten patients died at a mean age of 7 years. One patient had pulmonary transplantation. Prenatal diagnosis was performed in 9 families.
  
  
  CONCLUSIONS
  In Tunisia, cystic fibrosis is not exceptional, but its diagnosis is delayed. Our survey is characterized by more severe earliest forms, difficult and insufficient therapeutic management. A Better medical awareness and a national action plan are needed.
authors.forename: Sonia
authors.initials: S
authors.lastname: Halioui-Louhaichi
authors.forename: Amel
authors.initials: A
authors.lastname: Ben Chehida
authors.forename: Rim
authors.initials: R
authors.lastname: Hassouna
authors.forename: Tayeb
authors.initials: T
authors.lastname: Massaoud
authors.forename: Marie Françoise
authors.initials: MF
authors.lastname: Ben Dridi
authors.forename: Sihem
authors.initials: S
authors.lastname: Barsaoui
authors.forename: Azza
authors.initials: A
authors.lastname: Gharbi-Sammoud
authors.forename: Neji
authors.initials: N
authors.lastname: Tebib
authors.forename: Ahmed
authors.initials: A
authors.lastname: Maherzi
mesh_terms: D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D057210:Delayed Diagnosis; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D014416:Tunisia / Q000453:epidemiology