Improved detection of cystic fibrosis mutations in the heterogeneous U.S. population using an expanded, pan-ethnic mutation panel

Genet Med. 2001 May-Jun;3(3):168-76. doi: 10.1097/00125817-200105000-00004.

Abstract

Purpose: To determine the comparative frequency of 93 CFTR mutations in U.S. individuals with a clinical diagnosis of cystic fibrosis (CF).

Methods: A total of 5,840 CF chromosomes from Caucasians, Ashkenazi Jews, Hispanics, African Americans, Native Americans, Asians, and individuals of mixed race were analyzed using a pooled ASO hybridization strategy.

Results: Sixty-four mutations provided a sensitivity of 70% to 95% in all ethnic groups except Asians, and at least 81% when the U.S. population was considered as a whole.

Conclusions: For population-based carrier screening for CF in the heterogeneous U.S. population, which is characterized by increasing admixture, a pan-ethnic mutation panel of 50 to 70 CFTR mutations may provide a practical test that maximizes sensitivity.

MeSH terms

  • Asian People
  • Black People
  • Chromosomes
  • Cystic Fibrosis / diagnosis*
  • Cystic Fibrosis / ethnology
  • Cystic Fibrosis / genetics*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
  • DNA Mutational Analysis
  • Genetics, Population
  • Humans
  • Mutation*
  • Sensitivity and Specificity
  • United States
  • White People

Substances

  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator