Abstract
Children with CMS (Treacher-Collins Syndrome, hemifacial microsomia, Robin Sequence, Stickler Syndrome and other syndromes of clefting) and obstructive airway disease have poor sleep-entrained growth hormone (GH) secretion and may grow poorly. We studied GH release in four patients with CMS and compared it to seven children with idiopathic short stature (ISS) by measuring GH in blogd, every 20 min. during sleep. All patients had GH concentrations ≥10 ng/ml during standard provocative testing and had subnormal (<2 SD) height and height velocity percentiles for age. No patient had significant apnea or oxygen desaturation during the sleep period, GH secretory rates/per hour of sleep obtained by calculating area under the curve divided by sleep time (hrs) for CMS vs ISS were 8.4±4.0 vs 20.8±9.4pg/hr (mean ± SD) (p<0.025). There was no difference in the mean amplitude of the highest GH peak obtained during sleep: 13.4±4,7 ng/ml vs 19.2±6,6 ng/ml for ICM vs ISS (p>0.05). CMS patients had histories of poor sleep at home but no apnea was demonstrated during overnight studies.
These data suggest that children with CMS who grow poorly may have insufficient GH secretion during sleep.
Conclusions: Children with CMS and short stature require nocturnal GH secretion studies as part of their evaluation because nighttime GH secretion may be deficient and they may therefore benefit from treatment with growth hormone.
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Louis, Y., Wu, R., Thorpy, M. et al. ABNORMAL NOCTURNAL GROWTH HORMONE (GH) SECRETION IN SHORT CHILDREN WITH CRANIOFACIAL MALFORMATIONS SYNDROMES (CMS). Pediatr Res 21 (Suppl 4), 231 (1987). https://doi.org/10.1203/00006450-198704010-00387
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DOI: https://doi.org/10.1203/00006450-198704010-00387