Badawy S Z, Pisarska M D, Wasenko J J, Buran J J
Department of Obstetrics and Gynecology, State University of New York Health Science Center, Syracuse 13210.
J Reprod Med. 1994 Aug;39(8):643-8.
Congenital hypopituitarism may be due to hypothalamic failure. The case presented below belonged to this category. In addition, the demonstration of absent septum pellucidum placed this case in the category of suprasellar dysplasia. The patient was 21 years old, with primary amenorrhea and lack of development of secondary sex characteristics. The laboratory findings confirmed the diagnosis of hypothyroidism, hypocortisolism, hypogonadotropism, hyperprolactinemia and normal growth hormone. Stimulation studies revealed a subnormal response of cortisol to adrenocorticotrophic hormone stimulation, subnormal response of follicle stimulating hormone and luteinizing hormone to gonadotropin releasing hormone stimulation, normal response of prolactin to thyrotropin releasing hormone stimulation and exaggerated response of thyroid stimulating hormone to thyrotropin releasing hormone stimulation. The patient was treated with thyroid supplementation. Magnetic resonance imaging showed a hypoplastic infundibulum, ectopic neurohypophysis, small anterior pituitary gland and absent septum pellucidum. Congenital hypopituitarism may be part of a large spectrum of midline brain abnormalities.
先天性垂体功能减退可能是由于下丘脑功能衰竭所致。下面介绍的病例就属于这一类型。此外，透明隔缺如的表现使该病例属于鞍上发育异常的范畴。患者21岁，原发性闭经，第二性征未发育。实验室检查结果证实了甲状腺功能减退、皮质醇缺乏、促性腺激素缺乏、高催乳素血症以及生长激素正常的诊断。刺激试验显示，皮质醇对促肾上腺皮质激素刺激的反应低于正常，促卵泡激素和促黄体生成素对促性腺激素释放激素刺激的反应低于正常，催乳素对促甲状腺激素释放激素刺激的反应正常，促甲状腺激素对促甲状腺激素释放激素刺激的反应过度。患者接受了甲状腺补充治疗。磁共振成像显示垂体柄发育不全、异位神经垂体、垂体前叶较小以及透明隔缺如。先天性垂体功能减退可能是广泛的中线脑异常的一部分。
