Den Ouden D T, Kroon M, Hoogland P H, Geelhoed-Duijvestijn P H L M, Wit J M
Department of Internal Medicine, MCH Westeinde Hospital, 2501 CK The Hague, The Netherlands.
J Clin Endocrinol Metab. 2002 Dec;87(12):5430-4. doi: 10.1210/jc.2002-020672.
A 43-yr-old male was referred because of an x-ray made after a fall, which showed open epiphysis of the arm. The man had always been short for his age; during childhood he once consulted a pediatrician because of short stature, but thereafter he never sought medical attention. At age 18 yr he was not allowed to join the army because of his height of 147 cm. He continued to grow steadily and finally reached 193 cm. He had no complaints and considered himself reasonably fit. Physical examination showed a disproportional man with a body mass index of 29.3 kg/m(2) and Tanner stage P1G1. Laboratory investigations showed hormone levels consistent with multiple pituitary deficiency, with dynamic tests consistent with hypothalamic or pituitary stalk disease. Magnetic resonance scanning of the brain showed a small anterior pituitary remnant, no pituitary stalk, and an ectopic neurohypophysis. This case of untreated panhypopituitarism shows a particular growth curve with an average growth velocity of 2 cm/yr, resembling patients with estrogen receptor mutation or aromatase deficiency. A literature study of other adult patients with untreated panhypopituitarism shows a variable growth pattern. Some speculations about possible reasons for this variability in clinical characteristics are presented.
一名43岁男性因跌倒后拍摄的X线片显示手臂骨骺开放而前来就诊。该男子的身高一直低于同龄人；儿童时期，他曾因身材矮小咨询过儿科医生，但此后他从未寻求过医疗关注。18岁时，由于身高仅147厘米，他未被允许参军。他继续稳步生长，最终身高达到193厘米。他没有不适症状，自认为身体状况尚可。体格检查显示该男子身材比例失调，体重指数为29.3kg/m²，性发育处于坦纳1期阴毛和生殖器1期。实验室检查显示激素水平符合多种垂体功能减退，动态试验结果符合下丘脑或垂体柄疾病。脑部磁共振扫描显示垂体前叶有一小残余部分，无垂体柄，且神经垂体异位。这例未经治疗的全垂体功能减退病例呈现出一种特殊的生长曲线，平均生长速度为每年2厘米，类似于雌激素受体突变或芳香化酶缺乏的患者。对其他未经治疗的成年全垂体功能减退患者的文献研究显示，其生长模式各不相同。文中对这种临床特征变异性的可能原因进行了一些推测。
