ABNORMAL NOCTURNAL GROWTH HORMONE (GH) SECRETION IN SHORT CHILDREN WITH CRANIOFACIAL MALFORMATIONS SYNDROMES (CMS)

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 ( 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.