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新疆席汉氏综合征:97例患者的临床特征及实验室评估

Sheehan's syndrome in Xinjiang: Clinical characteristics and laboratory evaluation of 97 patients.

作者信息

Du Guo-li, Liu Zhong-hua, Chen Min, Ma Rui, Jiang Sheng, Shayiti Miriguli, Zhu Jun, Yusufu Aibibai

机构信息

Department of Endocrinology, First Affiliated Hospital of Xinjiang Medical University, Urumqi, China.

Department of Endocrinology, the Eastern Section of Linyi People's Hospital, Linyi, China.

出版信息

Hormones (Athens). 2015 Oct-Dec;14(4):660-7. doi: 10.14310/horm.2002.1624.

Abstract

OBJECTIVE

To evaluate the clinical and hormonal characteristics of patients with Sheehan's syndrome in Xinjiang, China.

METHODS

97 cases diagnosed as Sheehan's syndrome in our hospital from 1999 to 2013 were retrospectively reviewed. The medical history, physical examination findings and hormonal profiles were documented and analyzed.

RESULTS

The mean age at diagnosis was 43.7±12.4 years, with a mean diagnostic delay of 9.1±9.5 years (range, 1 month-35 years). 10 of our patients (10.3%) had a home birth. 96 of our patients (99.0%) had a history of obstetric hemorrhage. The most common clinical presentation included amenorrhea (80/97, 82.5%), agalactia (2/97, 74.2%) and loss of axillary or pubic hair (83/97, 85.6%). Seventy two of our patients (74.2%) failed to lactate and 80 of our patients (82.5%) failed to resume menstruation. Hypothalamic dysfunction included the hypothalamic-pituitary-gonadal axis (HPG) (LH deficiency: 77/83 patients, 92.8%; FSH deficiency: 73/83 patients, 88%; E2 deficiency: 62 of 82 patients,75.6%), the hypothalamic-pituitary-thyroid (HPT) axis (TSH deficiency: 77/93 patients, 82.8%, TT3 deficiency: 70/ 87 patients, 80.5%, TT4 deficiency: 72/87 patients, 82.8%) and the hypothalamus-pituitary-adrenal (HPA) axis (ACTH deficiency: 19/37 patients, 51.4%, cortisol deficiency: 49/64, 76.6%).

CONCLUSIONS

Sheehan's syndrome is still common in Xinjiang, especially in rural areas. Long diagnosis delay in most of the patients indicates that women might be lacking correct diagnosis and treatment. Physicians need to be aware of the most important clues for diagnosis such as lack of lactation in the postpartum period and failure to resume menstruation.

摘要

目的

评估中国新疆席汉氏综合征患者的临床及激素特征。

方法

回顾性分析1999年至2013年在我院确诊为席汉氏综合征的97例患者。记录并分析其病史、体格检查结果及激素水平。

结果

确诊时的平均年龄为43.7±12.4岁,平均诊断延迟时间为9.1±9.5年(范围1个月至35年)。10例患者(10.3%)在家中分娩。96例患者(99.0%)有产科出血史。最常见的临床表现包括闭经(80/97,82.5%)、无乳(2/97,74.2%)和腋毛或阴毛脱落(83/97,85.6%)。72例患者(74.2%)无乳汁分泌,80例患者(82.5%)月经未复潮。下丘脑功能障碍包括下丘脑 - 垂体 - 性腺轴(HPG)(促黄体生成素缺乏:77/83例患者,92.8%;促卵泡生成素缺乏:73/83例患者,88%;雌二醇缺乏:82例患者中的62例,75.6%)、下丘脑 - 垂体 - 甲状腺轴(HPT)(促甲状腺激素缺乏:77/93例患者,82.8%;总三碘甲状腺原氨酸缺乏:70/87例患者,80.5%;总甲状腺素缺乏:72/87例患者,82.8%)和下丘脑 - 垂体 - 肾上腺轴(HPA)(促肾上腺皮质激素缺乏:19/37例患者,51.4%;皮质醇缺乏:49/64例患者,76.6%)。

结论

席汉氏综合征在新疆仍然常见,尤其是在农村地区。大多数患者诊断延迟延迟表明女性可能缺乏正确的诊断和治疗。医生需要了解产后无乳和月经未复潮等重要诊断线索。

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