Cystic fibrosis in bahrain incidence, phenotype, and outcome

To identify the incidence and evaluate the causes of high mortality among Bahraini children with cystic fibrosis, we studied, retrospectively, 25 patients diagnosed as proven cases of cystic fibrosis at Sulmaniya Medical Center, the main referral center on the island, from January 1978 to December 1995. With extrapolation of the study data to the general population, the incidence of cystic fibrosis in Bahrain is at least one in 5800 and the prevalence is at least 3 in 100,000 population. Consanguineous marriage is documented in 80 per cent of the cases. Cystic fibrosis in Bahraini children is phenotypically severe, this is inferred from early age of presentation (mean of 2 months +/- 1 month) and early age of diagnosis (mean 4 +/- 1 month), development of pancreatic insufficiency by 1 year of age in all of the study patients, failure to thrive in 96 per cent of the patients, hypoalbuminemia in 48 per cent, meconium ileus in 20 per cent, progressive lung disease in 84 per cent at an early age, and early colonization with Pseudomonas aeruginosa. Age-specific mortality in the first year of life showed dramatic decline from 80 per cent between 1981 and 1985 to 50 per cent from 1986 to 1990, and to 9 per cent from 1991 to 1995. This improvement in survival is believed to be due to increased awareness among medical professionals and improved treatment.