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Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
Published on: April 11, 2018
Menarchal determinants in cystic fibrosis
American Journal of Diseases of Children (1960)
|December 1, 1980
Summary
Girls with cystic fibrosis (CF) experience delayed menarche, occurring later and with less body fat compared to healthy peers. This delay is linked to illness severity, not a critical body fat percentage.
Area of Science:
- Pediatrics
- Endocrinology
- Genetics
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Puberty onset, including menarche, is crucial for female reproductive health.
- Body composition and hormonal factors influence pubertal development.
Purpose of the Study:
- To investigate the timing of menarche in girls with Cystic Fibrosis (CF).
- To examine the relationship between menarche, body fat index (FI), and illness severity in CF.
- To assess hormonal profiles (gonadotropin, estradiol) related to delayed puberty in CF.
Main Methods:
- Retrospective analysis of medical records for 63 girls with CF who reached menarche.
- Comparison with 17 nonmenarchal girls with CF of similar age.
- Measurement of age at menarche, fat index (FI), clinical scores, and hormone levels.
Main Results:
- Girls with CF had menarche at a mean age of 14.5 years with a mean FI of 20.1%, significantly later and with less fat than normal girls.
- Menarchal age correlated with CF illness severity; amenorrheic girls were sicker and smaller.
- Gonadotropin and estradiol levels were within normal ranges in girls with CF.
Conclusions:
- Delayed menarche in Cystic Fibrosis is strongly associated with the severity of the illness.
- A "critical body composition" is unlikely to be the primary initiator of puberty in CF.
- Further research is needed to fully understand pubertal development in chronic childhood diseases.
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