A data-driven evaluation of the size and content of expanded carrier screening panels

Genet Med. 2019 Sep;21(9):1931-1939. doi: 10.1038/s41436-019-0466-5. Epub 2019 Feb 28.

Abstract

Purpose: The American College of Obstetricians and Gynecologists (ACOG) proposed seven criteria for expanded carrier screening (ECS) panel design. To ensure that screening for a condition is sufficiently sensitive to identify carriers and reduce residual risk of noncarriers, one criterion requires a per-condition carrier rate greater than 1 in 100. However, it is unestablished whether this threshold corresponds with a loss in clinical detection. The impact of the proposed panel design criteria on at-risk couple detection warrants data-driven evaluation.

Methods: Carrier rates and at-risk couple rates were calculated in 56,281 patients who underwent a 176-condition ECS and were evaluated for panels satisfying various criteria. Condition-specific clinical detection rates were estimated via simulation.

Results: Different interpretations of the 1-in-100 criterion have variable impact: a compliant panel would include between 3 and 38 conditions, identify 11-81% fewer at-risk couples, and detect 36-79% fewer carriers than a 176-condition panel. If the carrier rate threshold must be exceeded in all ethnicities, ECS panels would lack prevalent conditions like cystic fibrosis. Simulations suggest that the clinical detection rate remains >84% for conditions with carrier rates as low as 1 in 1000.

Conclusion: The 1-in-100 criterion limits at-risk couple detection and should be reconsidered.

Keywords: clinical detection rate; clinical guidelines; clinical utility; expanded carrier screening; genetic testing.

MeSH terms

  • DNA Copy Number Variations / genetics
  • Female
  • Genetic Carrier Screening / methods*
  • Genetic Carrier Screening / standards
  • Genetic Counseling / methods*
  • Genetic Counseling / standards
  • Genetic Diseases, Inborn / diagnosis
  • Genetic Diseases, Inborn / genetics*
  • Genetic Testing*
  • Guidelines as Topic
  • Heterozygote
  • Humans
  • Male
  • Mutation / genetics