Brown R S, Bhatia V, Hayes E
Department of Pediatrics, University of Massachusetts Medical School, Worcester 01655.
J Clin Endocrinol Metab. 1991 Jan;72(1):12-8. doi: 10.1210/jcem-72-1-12.
Between 1980 and 1989, five infants (four males and one female) with congenital hypopituitarism were born within a relatively sparsely populated area of central Massachusetts, an incidence 25-fold greater than expected. In all infants the pituitary stalk was undetectable on magnetic resonance imaging, and a bright spot on T-1 weighted imaging, probably representing posterior pituitary tissue, was found ectopically located inferior to the median eminence. The height of the anterior pituitary remnant on magnetic resonance imaging varied from undetectable to 4 mm, and similarly, on hormonal testing a spectrum of deficiencies of GH, TSH, ACTH, and gonadotropin was observed. The magnitude of the hypopituitarism was less in the three patients whose anterior pituitary remnant was greater than 2 mm, and in them, TRH testing resulted in exaggerated TSH and PRL responses, suggestive of hypothalamic disease. In contrast, in the other two patients in whom the anterior pituitary remnant was less than 2 mm in height, hypopituitarism was more severe, and TRH administration resulted in little if any TSH or PRL response, characteristic of pituitary disease. The size of the anterior pituitary remnant correlated significantly with that of the ectopic posterior pituitary bright spot (P less than 0.005). We conclude that patients with congenital hypopituitarism may exhibit a spectrum of hormonal responses, varying from findings typical of hypothalamic to those characteristic of primary pituitary disease. The pattern of hormonal responsiveness is dependent on the quantity of residual anterior pituitary tissue and probably results from abnormal transport of the hypothalamic hypophyseal releasing hormones rather than to any putative hypothalamic abnormality. The significant correlation between the size and function of the anterior pituitary gland and the size of the ectopic posterior pituitary remnant suggests that the fetal pituitary gland may secrete a factor necessary for the growth and descent of the neuroepithelium to form the infundibulum and posterior pituitary gland.
1980年至1989年间，马萨诸塞州中部一个人口相对稀少的地区出生了5名先天性垂体功能减退的婴儿（4名男性和1名女性），发病率比预期高25倍。在所有婴儿中，磁共振成像上均未检测到垂体柄，并且在T1加权成像上发现一个亮点，可能代表垂体后叶组织，异位位于正中隆起下方。磁共振成像上垂体前叶残余的高度从无法检测到4毫米不等，同样，在激素检测中，观察到生长激素、促甲状腺激素、促肾上腺皮质激素和促性腺激素存在一系列缺乏情况。垂体前叶残余大于2毫米的3名患者垂体功能减退的程度较轻，对他们进行促甲状腺激素释放激素检测时，促甲状腺激素和催乳素反应增强，提示下丘脑疾病。相比之下，垂体前叶残余高度小于2毫米的另外两名患者垂体功能减退更严重，注射促甲状腺激素释放激素后促甲状腺激素或催乳素几乎没有反应，这是垂体疾病的特征。垂体前叶残余的大小与异位垂体后叶亮点的大小显著相关（P小于0.005）。我们得出结论，先天性垂体功能减退患者可能表现出一系列激素反应，从典型的下丘脑疾病表现到原发性垂体疾病的特征。激素反应模式取决于垂体前叶残余组织的数量，可能是由于下丘脑垂体释放激素的异常转运，而不是任何假定的下丘脑异常。垂体前叶大小和功能与异位垂体后叶残余大小之间的显著相关性表明，胎儿垂体可能分泌一种神经上皮生长和下降所必需的因子，以形成漏斗和垂体后叶。
