Cystic fibrosis in Jordan : clinical and genetic aspects
Author
Source
Issue
Vol. 23, Issue 4 (31 Dec. 2001)7 p.
Publisher
King Hamad University Hospital
Publication Date
2001-12-31
Country of Publication
Bahrain
No. of Pages
7
Main Subjects
Abstract EN
Objective : To describe the demographic characteristics, phenotype, genotype, microbiological data, treatment and outcome among Jordanian children with cystic fibrosis .
Design : A prospective Cohort Study .
Setting : Princess Rahma Teaching Hosptial (PRTH).
Methods : All patients with cystic fibrosis seen between 1995 and 2000 inclusive (n = 72).
Clinical and laboratory data were collected on these patients.
Results: There were 37 males and 35 females in the study group.
The mean age of all patients was 4.5 years; the mean age at presentation was 21.2 months; the mean age at diagnosis was 30.7 months and the mean delay of CF diagnosis was 9 months.
Pancreatic exocrine insufficiency was documented in 94 % of cases.
Twenty (27 %) children died, most below the age of 1 year.
Pseudomonas aeruginosa was isolated in 30 % of cases.
Therapeutic measures were suboptimal in the majority of cases.
Consanguineous marriage was present in 70% of cases.
Genetic screening of the study population revealed 20 different Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) mutations with Delta F508 mutation accounting only for 6.3 %.
Conclusion : There is a wide variability in the phenotype and genotype of patients with cystic fibrosis.
Jordanian CF patients have a severe clinical course of disease with genetic and environmental factors may be contributory.
American Psychological Association (APA)
Qaqish, Khalid S.. 2001. Cystic fibrosis in Jordan : clinical and genetic aspects. Bahrain Medical Bulletin،Vol. 23, no. 4.
https://search.emarefa.net/detail/BIM-602299
Modern Language Association (MLA)
Qaqish, Khalid S.. Cystic fibrosis in Jordan : clinical and genetic aspects. Bahrain Medical Bulletin Vol. 23, no. 4 (Dec. 2001).
https://search.emarefa.net/detail/BIM-602299
American Medical Association (AMA)
Qaqish, Khalid S.. Cystic fibrosis in Jordan : clinical and genetic aspects. Bahrain Medical Bulletin. 2001. Vol. 23, no. 4.
https://search.emarefa.net/detail/BIM-602299
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-602299