Genetic counselling after carrier detection by newborn screening when one parent carries F508 and the other R117H

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Genetic counselling after carrier detection by newborn screening when one parent carries DeltaF508 and the other R117H.

Newborn screening (NBS) for cystic fibrosis (CF) has been carried out in Victoria, Australia since 1989. The primary screen is immunoreactive trypsinogen (IRT) followed by DeltaF508 mutation analysis. As part of this process, carrier babies are detected and their parents are routinely offered carrier testing as part of their follow up. The DeltaF508 parent is identified and the other parent has...

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The very low penetrance of cystic fibrosis for the R117H mutation: a reappraisal for genetic counselling and newborn screening.

BACKGROUND Cystic fibrosis (CF) is caused by compound heterozygosity or homozygosity of CF transmembrane conductance regulator gene (CFTR) mutations. Phenotypic variability associated with certain mutations makes genetic counselling difficult, notably for R117H, whose disease phenotype varies from asymptomatic to classical CF. The high frequency of R117H observed in CF newborn screening has als...

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ژورنال

عنوان ژورنال: Archives of Disease in Childhood

سال: 2003

ISSN: 0003-9888,1468-2044

DOI: 10.1136/adc.88.10.886