Newborn cystic fibrosis screening in southeastern Mexico: Birth prevalence and novel CFTR gene variants

J Med Screen. 2018 Sep;25(3):119-125. doi: 10.1177/0969141317722808. Epub 2017 Oct 9.

Abstract

Objective: To use the results of the first five years of a cystic fibrosis newborn screening program to estimate the cystic fibrosis birth prevalence and spectrum of cystic fibrosis transmembrane conductance regulator ( CFTR) gene variants in Yucatan, Mexico.

Methods: Screening was performed from 2010 to 2015, using two-tier immunoreactive trypsinogen testing, followed by a sweat test. When sweat test values were >30 mmol/L, the CFTR gene was analyzed.

Results: Of 96,071 newborns screened, a second sample was requested in 119 cases. A sweat test was performed in 30 newborns, and 9 possible cases were detected (seven confirmed cystic fibrosis and two inconclusive). The most frequently detected CFTR pathogenic variant (5/14 cystic fibrosis alleles, 35.7%) was p.(Phe508del); novel p.(Ala559Pro) and p.(Thr1299Hisfs*29) pathogenic variants were found.

Conclusions: Cystic fibrosis birth prevalence in southeastern Mexico is 1:13,724 newborns. Immunoreactive trypsinogen blood concentration is influenced by gestational age and by the time of sampling. The spectrum of CFTR gene variants in Yucatan is heterogeneous.

Keywords: CFTR gene pathogenic variants; Cystic fibrosis; Mexico; immunoreactive trypsinogen; newborn screening.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alleles
  • Cystic Fibrosis / blood*
  • Cystic Fibrosis / diagnosis*
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
  • Female
  • Humans
  • Infant, Newborn
  • Male
  • Mexico / epidemiology
  • Mutation*
  • Neonatal Screening / methods*
  • Prevalence
  • Reproducibility of Results
  • Sweat
  • Trypsinogen / blood

Substances

  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Trypsinogen