Municchi G, Pasquino A M, Pucarelli I, Cianfarani S, Passeri F
Pediatric Department, University La Sapienza, Rome, Italy.
Horm Res. 1995;44(4):164-7. doi: 10.1159/000184618.
Final height of 4 patients with Noonan syndrome and short stature treated with growth hormone (GH) is reported. Four prepubertal girls (chronological age 12.3-15.1 years, bone age 11.0-11.5 years) were treated with recombinant human growth hormone (0.5 IU/kg/week s.c.) for at least 3 years. Stimulated GH secretion was normal, spontaneous nocturnal GH secretion was low in 1 patient. Final height, as standard deviation score according to Ranke-specific standards for Noonan syndrome, improved in 3 patients and 2 of the exceeded their corrected midparental height.
报告了4例努南综合征伴身材矮小患者接受生长激素(GH)治疗后的最终身高。4例青春期前女孩(实际年龄12.3 - 15.1岁,骨龄11.0 - 11.5岁)接受重组人生长激素(0.5 IU/kg/周,皮下注射)治疗至少3年。其中1例患者刺激后的GH分泌正常,自发性夜间GH分泌较低。根据努南综合征的Ranke特定标准,以标准差评分表示的最终身高在3例患者中有所改善,其中2例超过了校正后的父母身高中位数。