目的：探讨希恩综合征的病因、临床表现、激素分泌特点和治疗。方法收集1999年1月-2013年5月新疆医科大学第一附属医院确诊的97例希恩综合征患者的临床资料，包括一般资料、临床表现及体格检查、实验室检查、垂体前叶及靶腺激素水平、影像学检查、治疗及效果。结果97例希恩综合征患者确诊年龄为（43.7±12.4）岁，距离产科病理事件的时间为（9.1±9.5）年。96例（99.0％）患者有产后大出血病史。83例（85.6％）有腋毛、阴毛脱落，80例（82.5％）有产后闭经，79例（81.4％）有头发、眉毛稀疏。实验室检查：血红蛋白降低占74.4％（67/90），红细胞沉降率增快占56.0％（14/25），低血糖占26.4％（23/87），血清总三碘甲状腺原氨酸（ TT3）水平降低占80.5％（70/87），总甲状腺素（TT4）水平降低占82.8％（72/87），游离三碘甲状腺原氨酸（FT3）水平降低占82.9％（34/41），游离甲状腺素（FT4）水平降低占90.2％（37/41），促甲状腺激素水平降低占82.8％（77/93），催乳素水平降低占57.3％（47/82），促卵泡生成素水平降低占88.0％（73/83），黄体生成素水平降低占92.8％（77/83），促肾上腺皮质激素（10：00）水平降低占51.4％（19/37）。27例患者行垂体磁共振成像（ MRI）检查，其中5例未见异常，2例显示垂体体积略缩小，20例显示垂体体积明显萎缩，呈部分性或完全性空泡蝶鞍。18例患者行妇科B超检查，15例显示子宫体积变小。85例（87.6％）患者给予相应靶腺激素替代治疗，不适症状明显好转。结论产后大出血为希恩综合征的常见病因，以靶腺激素缺失为主要临床表现，临床表现隐匿，易误诊、漏诊，重症患者可发生垂体前叶功能减退危象。治疗上应积极应用相应激素替代治疗。%syndrome. Methods The clinical data of 97 patients diagnosed as Sheehan syndrome at the First Affiliated Hospital of Xinjiang Medical University from January 1999 to May 2013 were collected including general information,clinical manifestation, physical examination,general laboratory test,anterior pituitary and target gland hormone level,imaging examination, treatment and effects. Results The mean age of the 97 patients was(43. 7 ± 12. 4) years at the diagnosis and approximately (9. 1 ± 9. 5) years past from the obstetric events. 96 patients( 99. 0%) suffered a postpartum hemorrhage,83 patients (85. 6%)had armpit hair and pubic hair lost,80 patients(82. 5%) had postnatal amenorrhea and 79 cases(81. 4%) had sparse hair and eyebrow. Laboratory examination results:lower hemoglobin 74. 4% (67/90),rapid erythrocyte sedimentation rate 56. 0% (14/25),hypoglycemia 26. 4% (23/87),TT3 decrease 80. 5% (70/87),TT4 decrease 82. 8% (72/87), FT3 decrease 82. 9% (34/41),FT4 decrease 90. 2% (37/41),Thyroid stimulating hormone decrease 82. 8% (77/93), PRL decrease 57. 3% (47/82),serum testosterone level decrease 88. 0% (73/83),LH decrease 92. 8% (77/83),ACTH (10:00)level decrease 51. 4% (19/37). 27 cases received pituitary magnetic resonance imaging examination:pituitary volume in 5 was normal,in 2 was slightly reduced,in 20 was in a state of significant atrophy showing partly or complete empty sella. The gynecologic ultrasonic examination of 18 cases showed that uterine volume of 12 cases was reduced. 85 cases(87. 6%) were administered corresponding target gland hormone instead of treatment and had the uncomfortable symptom improvement to a large degree. Conclusion Postpartum hemorrhage is a common cause of Sheehan syndrome and target gland hormone missing is the main clinical manifestation. For the clinical manifestations of this disease is imperceptible,it is easy to be misdiagnosed and missed,which may lead to anterior pituitary dysfunction of the severe patients. Hormone replacement treatment should be actively applied.
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