Preliminary national report on cystic fibrosis epidemiology in Tunisia: the actual state of affairs.
Afr Health Sci
; 20(1): 444-452, 2020 Mar.
Article
em En
| MEDLINE
| ID: mdl-33402933
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 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).CONCLUSION:
CF is life-shortening in Tunisia. Setting-up appropriate management is urgent.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Fibrose Cística
Tipo de estudo:
Etiology_studies
/
Observational_studies
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Risk_factors_studies
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Screening_studies
Limite:
Adult
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Child
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Female
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Humans
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Infant
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Male
País/Região como assunto:
Africa
Idioma:
En
Revista:
Afr Health Sci
Assunto da revista:
MEDICINA
/
SERVICOS DE SAUDE
Ano de publicação:
2020
Tipo de documento:
Article