Literature DB >> 25077647

Molecular genetic testing for cystic fibrosis: laboratory performance on the College of American Pathologists external proficiency surveys.

Elaine Lyon1, Iris Schrijver2, Karen E Weck3, Andrea Ferreira-Gonzalez4, C Sue Richards5, Glenn E Palomaki6.   

Abstract

BACKGROUND: Molecular testing for cystic fibrosis mutations is widespread and routine in reproductive decision making and diagnosis. Our objective was to assess the level of performance of laboratories for this test.
METHODS: The College of American Pathologists administers external proficiency testing with multiple DNA samples distributed biannually. RESULTS are analyzed, reviewed, and graded by the joint College of American Pathologists/American College of Medical Genetics and Genomics Biochemical and Molecular Genetics Committee. Assessment is based on genotype and associated clinical interpretation.
RESULTS: Overall, 357 clinical laboratories participated in the proficiency testing survey between 2003 and 2013 (322 in the United States and 35 international). In 2013, US participants reported performing nearly 120,000 tests monthly. Analytical sensitivity and specificity of US laboratories were 98.8% (95% confidence interval: 98.4-99.1%) and 99.6% (95% confidence interval: 99.4-99.7%), respectively. Analytical sensitivity improved between 2003 and 2008 (from 97.9 to 99.3%; P = 0.007) and remained steady thereafter. Clinical interpretation matched the intended response for 98.8, 86.0, and 91.0% of challenges with no, one, or two mutations, respectively. International laboratories performed similarly. DISCUSSION: Laboratory testing for cystic fibrosis in the United States has improved since 2003, and these data demonstrate a high level of quality. Neither the number of samples tested nor test methodology affected performance.

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Year:  2014        PMID: 25077647     DOI: 10.1038/gim.2014.93

Source DB:  PubMed          Journal:  Genet Med        ISSN: 1098-3600            Impact factor:   8.822


  9 in total

1.  Clinical sensitivity of prenatal screening for cystic fibrosis via CFTR carrier testing in a United States panethnic population.

Authors:  Glenn E Palomaki; Stacey C FitzSimmons; James E Haddow
Journal:  Genet Med       Date:  2004 Sep-Oct       Impact factor: 8.822

2.  Development of genomic reference materials for cystic fibrosis genetic testing.

Authors:  Victoria M Pratt; Michele Caggana; Christina Bridges; Arlene M Buller; Lisa DiAntonio; W Edward Highsmith; Leonard M Holtegaard; Kasinathan Muralidharan; Elizabeth M Rohlfs; Jack Tarleton; Lorraine Toji; Shannon D Barker; Lisa V Kalman
Journal:  J Mol Diagn       Date:  2009-04-09       Impact factor: 5.568

Review 3.  Genotype and phenotype in cystic fibrosis.

Authors:  J Zielenski
Journal:  Respiration       Date:  2000       Impact factor: 3.580

Review 4.  Cystic fibrosis: molecular diagnosis, population screening, and public policy.

Authors:  W W Grody
Journal:  Arch Pathol Lab Med       Date:  1999-11       Impact factor: 5.534

5.  Correlation between genotype and phenotype in patients with cystic fibrosis.

Authors: 
Journal:  N Engl J Med       Date:  1993-10-28       Impact factor: 91.245

6.  A mutation in CFTR produces different phenotypes depending on chromosomal background.

Authors:  S Kiesewetter; M Macek; C Davis; S M Curristin; C S Chu; C Graham; A E Shrimpton; S M Cashman; L C Tsui; J Mickle
Journal:  Nat Genet       Date:  1993-11       Impact factor: 38.330

7.  Laboratory standards and guidelines for population-based cystic fibrosis carrier screening.

Authors:  W W Grody; G R Cutting; K W Klinger; C S Richards; M S Watson; R J Desnick
Journal:  Genet Med       Date:  2001 Mar-Apr       Impact factor: 8.822

8.  Analytic validity of cystic fibrosis testing: a preliminary estimate.

Authors:  Glenn E Palomaki; Linda A Bradley; C Sue Richards; James E Haddow
Journal:  Genet Med       Date:  2003 Jan-Feb       Impact factor: 8.822

9.  Cystic fibrosis population carrier screening: 2004 revision of American College of Medical Genetics mutation panel.

Authors:  Michael S Watson; Garry R Cutting; Robert J Desnick; Deborah A Driscoll; Katherine Klinger; Michael Mennuti; Glenn E Palomaki; Bradley W Popovich; Victoria M Pratt; Elizabeth M Rohlfs; Charles M Strom; C Sue Richards; David R Witt; Wayne W Grody
Journal:  Genet Med       Date:  2004 Sep-Oct       Impact factor: 8.822

  9 in total
  3 in total

1.  CAP/ACMG proficiency testing for biochemical genetics laboratories: a summary of performance.

Authors:  Devin Oglesbee; Tina M Cowan; Marzia Pasquali; Timothy C Wood; Karen E Weck; Thomas Long; Glenn E Palomaki
Journal:  Genet Med       Date:  2017-06-29       Impact factor: 8.822

2.  GenomeTrakr proficiency testing for foodborne pathogen surveillance: an exercise from 2015.

Authors:  Ruth E Timme; Hugh Rand; Maria Sanchez Leon; Maria Hoffmann; Errol Strain; Marc Allard; Dwayne Roberson; Joseph D Baugher
Journal:  Microb Genom       Date:  2018-06-15

3.  Cost-effectiveness of the CFTR gene-sequencing test for asymptomatic carriers in the Colombian population

Authors:  Ernesto Andrade; Jorge Díaz
Journal:  Biomedica       Date:  2020-06-15       Impact factor: 0.935

  3 in total

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