At. Soliman et al., DEFECTIVE GROWTH-HORMONE SECRETION IN CHILDREN WITH PYCNODYSOSTOSIS AND IMPROVED LINEAR GROWTH AFTER GROWTH-HORMONE TREATMENT, Archives of Disease in Childhood, 75(3), 1996, pp. 242-244
Short stature is a characteristic feature of pycnodysostosis. We repor
t defective growth hormone secretion in response to provocation and lo
w insulin-like growth factor-I (IGF-I) concentration in five out of si
x patients with pycnodysostosis. Physiological replacement with growth
hormone increased IGF-I concentration and improved linear growth in t
hese children.