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对 114 例席汉氏综合征患者的广泛调查:一种持续性疾病。

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

机构信息

Departments of EndocrinologyRadiologyErciyes University Medical School, 38039 Kayseri, TurkeyDepartment of EndocrinologyOndokuz Mayis University Medical School, Samsun, Turkey.

Departments of EndocrinologyRadiologyErciyes University Medical School, 38039 Kayseri, TurkeyDepartment of EndocrinologyOndokuz Mayis University Medical School, Samsun, Turkey

出版信息

Eur J Endocrinol. 2014 Sep;171(3):311-8. doi: 10.1530/EJE-14-0244. Epub 2014 Jun 10.

DOI:10.1530/EJE-14-0244
PMID:24917653
Abstract

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.

摘要

目的

席汉氏综合征(SS)是一种由产后垂体坏死引起的已知的垂体功能减退症病因。由于在西方社会较为罕见,其诊断往往被忽视。我们旨在研究大量 SS 患者的临床、实验室和影像学表现。

研究设计

对 114 例 SS 患者的病历进行回顾性评估。此外,通过磁共振成像检查比较了 29 名健康女性的鞍底体积与患者的鞍底体积。

结果

SS 患者的平均诊断延迟时间为 19.7 年。发现 52.6%的患者有非特异性症状,30.7%的患者有肾上腺功能不全相关症状,9.6%的患者有性腺功能减退相关症状。在诊断时,55.3%的患者存在全垂体功能减退症,而 44.7%的患者存在部分垂体功能减退症。随着时间的推移,缺乏的激素数量有所增加。基础催乳素低于 4.0ng/ml 的患者中无一例 TRH 试验催乳素反应充足,而基础催乳素高于 7.8ng/ml 的患者均有充足的反应。发现 SS 组的平均鞍底体积(340.5±214mm³)明显小于健康组(602.5±192mm³)。

结论

SS 是不发达国家和发展中国家垂体功能减退症的常见病因。诊断延迟的主要原因似乎是患者有较高的非特异性症状发生率,且容易忽视 SS。此外,TRH 刺激试验对识别催乳素缺乏具有较高的敏感性和特异性。此外,小的鞍底大小可能对 SS 的发病机制有重要作用。

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