Abstract：
BACKGROUND:We have observed that Doctors often perceive that cystic fibrosis (CF) is exceptionally rare in non-whites, and that this bias has repeatedly resulted in diagnostic delay. We therefore compared the age at diagnosis, genetic features and relative prevalence of CF in non-whites and white patients in the West Midlands. METHODS:Analysis of data on all CF patients diagnosed in childhood and stored in the West Midlands CF register. RESULTS:Sixteen of the 514 children on the register were not of white European extraction, comprising 13 patients whose families originated from the Indian subcontinent, two of mixed AfroCaribbean/white European extraction and one of mixed Pakistani/white European extraction. The median age of diagnosis was similar in the white European and non-white patients (0.42 vs. 0.33 years, 95% CI for the difference of the medians -0.15, 0.37). However, in five cases with typical clinical features the diagnosis appears to have been delayed because of the child's racial origin (median age of diagnosis 3.87 years), and in five others the diagnosis was obvious (two siblings with CF, three had meconium ileus). There was a degree of consanguinity in nine cases. Five patients were homozygous or heterozygous for the delta F508 mutation, but no mutation could be identified in the remaining 11 patients. CONCLUSIONS:The possibility of CF needs to be considered in any patient with relevant clinical problems, regardless of racial origin. These findings need to be considered when planning any mass population screening programme for CF.
journal_name
Respir Medjournal_title
Respiratory medicineauthors
Spencer DA,Venkataraman M,Higgins S,Stevenson K,Weller PHdoi
10.1016/s0954-6111(05)80065-4subject
Has Abstractpub_date
1994-10-01 00:00:00pages
671-5issue
9eissn
0954-6111issn
1532-3064pii
S0954-6111(05)80065-4journal_volume
88pub_type
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