Growth Hormone (GH) Secretory Characteristics and Effectiveness of GH Treatment in Achondroplasia

The growth hormone (GH) secretory characteristics and the effectiveness of GH in the treatment of short stature were examined in 22 patients with achondroplasia (seven males and 15 females, aged three to 12 years).The GH response to provocation tests was abnormal in five patients; four showed a subnormal (<10ng/ml) response to L-DOPA and one showed a subnormal (<20ng/ml) response to GRF. The mean GH concentration during sleep was low (<5ng/ml) in three patients, who also showed reduced IGF-I levels and delayed bone age. These three patients were considered to have GH neurosecretory dysfunction syndrome (GHND).In 13 patients who were treated with GH for more than six months, height velocity was significantly increased compared with that before therapy (4.1±0.8cm/year v. 7.8±1.8 cm/year).These data indicate that GH may be effective in the treatment of short stature in chondroplasia.