Müller B, Trepp R, Diem P, Christ E
Abteilung für Endokrinologie und Diabetologie, Inselspital, Universität Bern, Germany.
Dtsch Med Wochenschr. 2002 Dec 6;127(49):2610-2. doi: 10.1055/s-2002-35929.
A 59-year-old woman was examined because of weight gain, increasing fatigue and secondary amenorrhoea, which occurred after a complicated delivery at age 18. The finding of an increased TSH concentration was initially considered as primary hypothyroidism and substitution therapy was commenced. Because of the concomitant secondary amenorrhoea the patient was referred for additional endocrinological investigations.
Biochemical analysis confirmed the increase in TSH concentrations, and revealed a gonadotropin deficiency, a decrease in IGF-I concentration and a free urinary cortisol concentration at the lower end of the normal range. Dynamic testing of pituitary function (insulin tolerance test) confirmed a severe growth hormone deficiency and partial secondary adrenal insufficiency. An MRI study of the pituitary showed an empty sella with some remaining pituitary tissue at the bottom of the sella.
DIAGNOSIS, TREATMENT AND CLINICAL COURSE: The laboratory findings of pituitary insufficiency with an empty sella on MRI scan suggested Sheehan's syndrome despite an increase in thyrotropin level. Pituitary replacement therapy was started with hydrocortone, combined estrogens and progesterone in addition to levothyroxin, which considerably improved clinical symptoms.
A history of secondary amenorrhoea after a complicated delivery including significant bleeding or septic complications suggest Sheehan's syndrome, which can result in partial or complete panhypopituitarism. In such circumstances the pituitary hormone levels are usually reduced. TSH concentrations, however, may be increased.
一名59岁女性因体重增加、疲劳加重及继发性闭经前来就诊,这些症状于她18岁时复杂分娩后出现。促甲状腺激素(TSH)浓度升高最初被认为是原发性甲状腺功能减退,并开始进行替代治疗。由于同时存在继发性闭经,患者被转诊接受进一步的内分泌检查。
生化分析证实TSH浓度升高,并显示促性腺激素缺乏、胰岛素样生长因子-I(IGF-I)浓度降低以及尿游离皮质醇浓度处于正常范围下限。垂体功能动态试验(胰岛素耐量试验)证实存在严重的生长激素缺乏和部分继发性肾上腺功能不全。垂体磁共振成像(MRI)检查显示为空蝶鞍,蝶鞍底部仍有一些残留的垂体组织。
诊断、治疗及临床病程:MRI扫描显示空蝶鞍且伴有垂体功能不全的实验室检查结果提示希恩综合征,尽管促甲状腺激素水平升高。开始使用氢化可的松、复方雌激素和孕激素以及左甲状腺素进行垂体替代治疗,这使临床症状得到显著改善。
复杂分娩后出现继发性闭经病史,包括大量出血或败血症并发症,提示可能患有希恩综合征,该综合征可导致部分或完全性垂体功能减退。在这种情况下,垂体激素水平通常会降低。然而,TSH浓度可能会升高。