Elsevier

The Journal of Pediatrics

Volume 132, Issue 2, February 1998, Pages 255-259
The Journal of Pediatrics

Comparison of the clinical manifestations of cystic fibrosis in black and white patients,☆☆,,★★

https://doi.org/10.1016/S0022-3476(98)70441-XGet rights and content
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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 CF. Fewer have meconium ileus, but more have distal intestinal obstruction syndrome. To control for genotype, each black ΔF508 homozygote (n = 47) was compared with four age- and sex-matched white ΔF508 homozygotes. Only the difference in nutritional status remained. The ΔF508 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. (J Pediatr 1998;132:255-9)

Abbreviations

CF
Cystic fibrosis
CFTR
Cystic fibrosis transmembrane conductance regulator
DIOS
Distal intestinal obstruction syndrome
NCHS
National Center for Health Statistics

Cited by (0)

From the Department of Pediatrics and Center for Medical Genetics, Johns Hopkins University School of Medicine, Baltimore, Maryland; The Cystic Fibrosis Foundation, Bethesda, Maryland; the Department of Medical Genetics II, University Hospital Motol, Prague, Czech Republic; and the Department of Medicine, Division of Pulmonary Diseases, University of North Carolina at Chapel Hill, North Carolina.

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Supported by National Institute of Diabetes and Digestive and Kidney Diseases grants No. DK44003 to Dr. Cutting and No. K08 DK0215 to Dr. Hamosh; US-Czech Science and Technology Program grant (No. 94003) to Drs. Macek and Cutting; and IGA MZ CR (Nos. 2899-5, 3526-3, and 4124-3) and GA CR (No. 1148-6) to Dr. Macek.

Reprint requests: Ada Hamosh, MD, MPH, Center for Medical Genetics, CMSC 1004, Johns Hopkins University School of Medicine, 600 N. Wolfe St., Baltimore, MD 21287-3914.

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0022-3476/98/$5.00 + 0 9/21/83011