低生长激素性巨人症

报告 1例低生长激素水平且垂体大小正常的巨人症。患者 ,女 ,11岁 ,因生长过速 6年入院。查体 :身高 181cm,体重 77kg,指间距 187cm。骨龄 11.1岁 ,血清基础生长激素水平小于 1ng/ml,血清 T3 、T4、FT3 、FT4、TSH、E2 、L H、FSH、PRL、PTC及血浆 ACTH水平正常。胰岛素低血糖刺激试验、精氨酸刺激试验显示生长激素无反应。双手及头颅 X片显示尺、桡骨、掌指骨粗大 ,双手指骨骨皮质变薄 ,指骨增长 ,颅骨增大 ,颅板增厚 ,枕骨粗隆呈倒钩状。心三位片显示左、右心室轻度增大。头颅 MRI显示垂体大小正常 ,无异常信号。对于该例患者 ,非脉冲式的 GH分泌和组织对 GH敏感性增加也许导致 IGF- 1过度分泌 ,以及未分类的促生长因子和具生物活性的抗生长激素受体抗体的存在可能是导致临床上出现巨人症的原因

Gigantism with Low Serum Level of Growth Hormone:A Case Report

Gigantism with low or normal basal concentrations of growth hormone (GH) is a rare condition, possibly due to abnormal GH secretory patterns, enhanced tissue sensitivity to GH, or the existence of an unidentified growth promoting factor. Here we report an 11 year old female case of gigantism with a normal pituitary gland. Her height was 181 cm, body weight 77kg, and bone age 11.1 years. Her basal serum GH levels were lower than 1 ng/ml. The levels of T 3, T 4, FT 3, FT 4, TSH, E 2, LH, FSH, PRL, PTC and ACTH were normal. Serum GH response to insulin induced hypoglycemia or arginine stimulation tests was blunted. In this case , non pulsatile GH secretion and enhanced tissue sensitivity to GH may induce hypersecretion of IGF 1 and the existence of an unidentified growth promoting factor or biologically active anti GH receptor antibodies may cause clinical gigantism.