Extensive investigation of 114 patients with Sheehan's syndrome: a continuing disorder

Extensive investigation of 114 patients with Sheehan's syndrome: a continuing disorder
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DOI:
10.1530/eje-14-0244
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发表时间:
2014-09-01
影响因子:
5.8
通讯作者:
Kelestimur, Fahrettin
Kelestimur, Fahrettin
中科院分区:
医学1区
文献类型:
--
作者:
Diri, Halit;Tanriverdi, Fatih;Kelestimur, Fahrettin

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目的:希恩综合征(SS)是产后垂体坏死引起的垂体功能低下的众所周知的原因。由于其在西方社会很少见,其诊断常常被忽视。我们的目的是调查大量 SS 患者的临床、实验室和放射学方面的情况。研究设计:对 114 名 SS 患者的医疗记录进行回顾性评估。此外,通过磁共振成像检查将29名健康女性的蝶鞍体积与患者的蝶鞍体积进行了比较。结果:SS患者的平均诊断延迟时间为19.7年。结果发现,52.6%的患者在诊断时有非特异性主诉,30.7%有与肾上腺功能不全相关的主诉,9.6%有与性腺功能减退症相关的主诉。诊断时,55.3%的患者患有全垂体功能减退症,44.7%的患者患有部分垂体功能减退症。人们发现,缺乏激素的数量逐年增加。基础催乳素低于 4.0 ng/ml 的患者对 TRH 测试没有足够的催乳素反应,而所有基础催乳素高于 7.8 ng/ml 的患者都有足够的反应。 SS 组的平均蝶鞍体积 (340.5 +/- 214 mm(3)) 显着低于健康组 (602.5 +/- 192 mm(3))。结论:SS 是欠发达国家和发展中国家垂体功能低下的常见原因。诊断延迟的主要原因似乎是患者出现非特异性主诉和忽视 SS 的频率较高。此外,TRH 刺激试验被发现对识别 PRL 缺乏具有很高的敏感性和特异性。此外,较小的蝶鞍尺寸可能在 SS 的发病机制中发挥重要作用。
Objective: Sheehan's syndrome (SS) is a well-known cause of hypopituitarism resulting from postpartum pituitary necrosis. Because of its rarity in Western society, its diagnosis is often overlooked. We aimed to investigate the clinical, laboratory, and radiological aspects of SS in a large number of patients.Study design: A retrospective assessment of the medical records of 114 patients with SS was conducted. In addition, sella turcica volumes of 29 healthy women were compared with those of patients by magnetic resonance imaging examinations.Results: The mean period of diagnostic delay was 19.7 years in patients with SS. It was found that 52.6% of patients had nonspecific complaints, 30.7% had complaints related to adrenal insufficiency, and 9.6% had complaints related to hypogonadism when diagnosed. At the time of diagnosis, 55.3% of the patients had panhypopituitarism, while 44.7% had partial hypopituitarism. The number of deficient hormones was found to be increased over the years. None of the patients whose basal prolactin was below 4.0 ng/ml had adequate prolactin responses to TRH test, while all patients whose basal prolactin was above 7.8 ng/ml had adequate responses. Mean sella volume was found to be significantly lower in the SS group (340.5 +/- 214 mm(3)) than that in the healthy group (602.5 +/- 192 mm(3)).Conclusions: SS is a common cause of hypopituitarism in underdeveloped and developing countries. The main reasons for diagnostic delay seem to be the high frequency of patients with nonspecific complaints and neglect of SS. In addition, the TRH stimulation test was found to have a high sensitivity and specificity to recognize PRL deficiency. Furthermore, small sella size may have an important contributing role in the etiopathogenesis of SS.