Andler W, Stolecke H, Sirang H
Helv Paediatr Acta. 1978 Nov;33(4-5):393-400.
A 10-month-old boy with the clinical features of the diencephalic syndrome of emaciation due to a suprasellar spongioblastoma is described. The patient showed high basal levels of growth hormone (GH greater than 80 muU/ml on several occasions). In addition, elevated concentration of plasma testosterone (125.5 ng/100ml) was combined with a relatively high LH-increase to LHRH (45.6 mU/ml). After completion of irradiation basal GH-levels had been normalized, and GH responses to insulin induced hypoglycemia (IIH) and propranolol-glucagon (PG) were adequate. Complete clinical remission of emaciation occurred soon after radiation therapy and went parallel with the normalization of GH-regulation.
本文描述了一名10个月大的男孩，因鞍上海绵状细胞瘤出现消瘦型间脑综合征的临床特征。该患者基础生长激素水平较高（多次检测生长激素大于80 μU/ml）。此外，血浆睾酮浓度升高（125.5 ng/100ml），同时促黄体生成素对促性腺激素释放激素（LHRH）的反应相对较高（45.6 mU/ml）。放疗结束后，基础生长激素水平恢复正常，生长激素对胰岛素诱发低血糖（IIH）和普萘洛尔-胰高血糖素（PG）的反应正常。放疗后不久，消瘦症状完全临床缓解，且与生长激素调节的正常化同步。
