Abstract
No large-scale studies of the incidence or disease severity of cystic fibrosis (CF) in black patients have been reported to date. In this study, the CF Foundation National Patient Registry was used to establish new incidence figures and to compare the clinical status of U.S. black (n = 601) and white patients (n = 17,755) with CF.
Results indicate that the incidence of CF is approximately 1 in 3,200 white and 1 in 15,000 black live births in the United States. Black patients with CF are currently, and were at diagnosis, younger and have poorer nutritional status and pulmonary function than white patients with CE Fewer have meconium ileus, but more have distal intestinal obstruction syndrome.
To control for genotype, each black Delta F508 homozygote (n = 47) was compared with four age- and sex-matched white Delta F508 homozygotes. Only the difference in nutritional status remained.
The Delta F508 mutation is associated with higher levels of meconium ileus than other genotypes, independent of race. In conclusion the clinical manifestations of CF are similar in black and white patients except for poorer nutritional status in black patients, which appears to be independent of age and genotype.