Kikuchi K, Fujisawa I, Momoi T, Yamanaka C, Kaji M, Nakano Y, Konishi J, Mikawa H, Sudo M
Department of Pediatrics, Kyoto University School of Medicine, Japan.
J Clin Endocrinol Metab. 1988 Oct;67(4):817-23. doi: 10.1210/jcem-67-4-817.
We compared 1.5 T magnetic resonance (MR) image findings with hypothalamic-pituitary function in 11 patients with idiopathic pituitary dwarfism, each of whom had a history of perinatal abnormalities, and 1 patient with posttraumatic pituitary dwarfism. MR imaging revealed transection of the pituitary stalk in all patients and the formation of an ectopic posterior lobe at the proximal stump in 9 patients, none of whom had polydipsia or polyuria. Three patients without an ectopic posterior lobe had diabetes insipidus. The 5 patients who had small pituitary glands (less than 2 mm in height) had hypothyroidism with low serum TSH concentrations and low serum cortisol responses to insulin-induced hypoglycemia; however, 7 patients with normal-sized pituitary glands had normal thyroid and adrenal function. The serum GH response to GHRH did not correlate with the size of the pituitary gland. The patients with small pituitary glands had delayed or prolonged serum TSH responses to TRH and impaired serum LH and FSH responses to GnRH; 4 of the patients with normal-sized pituitary glands had normal serum TSH, LH, and FSH responses. Only 2 patients had high basal serum PRL concentrations. The endocrinological data suggest that reestablishment of the hypothalamo-hypophyseal portal circulation, which cannot be seen by MR imaging, may occur. We suggest that the primary cause of idiopathic pituitary dwarfism in many patients is injury to the pituitary stalk at birth.
我们比较了11例特发性垂体性侏儒症患者及1例创伤后垂体性侏儒症患者的1.5T磁共振（MR）图像结果与下丘脑 - 垂体功能。11例特发性垂体性侏儒症患者均有围产期异常病史。MR成像显示所有患者垂体柄横断，9例患者在近端残端形成异位后叶，这些患者均无烦渴或多尿症状。3例无异位后叶的患者患有尿崩症。5例垂体小（高度小于2mm）的患者有甲状腺功能减退，血清促甲状腺激素（TSH）浓度低，对胰岛素诱导的低血糖血清皮质醇反应低；然而，7例垂体大小正常的患者甲状腺和肾上腺功能正常。血清生长激素（GH）对生长激素释放激素（GHRH）的反应与垂体大小无关。垂体小的患者对促甲状腺激素释放激素（TRH）的血清TSH反应延迟或延长，对促性腺激素释放激素（GnRH）的血清促黄体生成素（LH）和促卵泡生成素（FSH）反应受损；4例垂体大小正常的患者血清TSH、LH和FSH反应正常。仅2例患者基础血清催乳素（PRL）浓度高。内分泌学数据提示可能发生了MR成像无法显示的下丘脑 - 垂体门脉循环重建。我们认为许多患者特发性垂体性侏儒症的主要原因是出生时垂体柄损伤。
