Journal
JOURNAL OF CYSTIC FIBROSIS
Volume 18, Issue 2, Pages 190-193Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.jcf.2018.12.006
Keywords
Analytical variation; Biological variation; Cystic fibrosis; Index of individuality; Reference change value; Sweat chloride
Categories
Funding
- Cystic Fibrosis Foundation Therapeutics, Inc. [MARTIN17XXO]
- National Institutes of Health [R01DK095738]
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Fifty-nineCF infants' sweat chloride concentrations were analyzed to answer the questions: What is the biological and analytical variation in sweat chloride concentrations collected from the 32 infants homozygous for the F508 deletion? Do sweat chloride concentrations change in the first year of life beyond the variance previously established for adults with similar CFTR mutations? The biological and analytical variation of the infants' sweat chloride concentration was similar to that seen in adult CF patients. While there was a statistically significant difference between sweat chloride concentration in early (89.8 mmol/L) and late (95.0 mmol/L) infancy, this change is not likely clinically significant. This suggests that sweat chloride concentrations in CF patients do not change in a meaningful way during the first year of life. Determining variability in infants with CF is the necessary first step for future design of clinical trials of CFTR modulators in younger patients. (C) 2018 Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society.
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