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目的 :探讨119例席汉综合征患者的临床特征。方法 :收集2002年1月~2014年11月在中南大学湘雅二医院内分泌科确诊为席汉综合征的119例住院患者的临床资料。结果 :119例席汉综合征患者中,确诊平均年限为(18.1±11.0)年。确诊席汉综合征患者再孕12例(10%),其中长期接受激素替代6例(5%),再孕后病情缓解1例(0.8%)。并发垂体危象64例(54%),以垂体危象为首发症状者48例(75%),无垂体危象55例(46%)。促性腺激素减退症为101例(97%),促甲状腺激素减退症占103例(89%),促肾上腺皮质激素减退症占51例(100%)。尿崩症3例(3%)。49例行垂体MRI,其中,空泡蝶鞍27例(55%),垂体萎缩19例(39%)。40例行骨密度检查,骨质疏松10例(25%),低骨量23例(58%)。结论 :席汉综合征的病情轻重不一,临床表现存在多样性,早期正确诊断至关重要。一方面诊断后及时靶腺激素替代治疗可避免垂体危象、骨质疏松症等的发生,另一方面病情轻者有可能再孕,再孕后病情可改善或缓解。
Objective: To investigate the clinical features of 119 cases of syndromes of Xihan syndrome. Methods: The clinical data of 119 hospitalized patients diagnosed as Feh-Han syndrome in Department of Endocrinology, Second Xiangya Hospital of Central South University from January 2002 to November 2014 were collected. Results: Among the 119 patients with ICH, the mean duration of diagnosis was (18.1 ± 11.0) years. Six patients (10%) were diagnosed with syndrome of reinstatement syndrome, including 6 cases (5%) receiving long-term hormone replacement and 1 case (0.8%) remission after re-birth. 64 cases (54%) had pituitary crisis, 48 cases (75%) had pituitary crisis as the first symptom, and 55 cases (46%) did not have pituitary crisis. Hypogonadism was found in 101 (97%), thyroid-stimulating hormone (103) (89%), and adrenocorticotropic hormone in 51 (100%). Diabetes insipidus in 3 cases (3%). Pituitary MRI was performed in 49 cases, of which 27 cases (55%) had vacuole sellae and 19 cases (39%) had pituitary atrophy. Forty patients underwent bone mineral density examination, osteoporosis in 10 (25%) and low bone mass in 23 (58%). Conclusion: The syndrome of Xi Han syndrome varies in severity, the diversity of clinical manifestations, early diagnosis is essential. On the one hand after the diagnosis of timely replacement of target gland hormone therapy to avoid pituitary crisis, osteoporosis and other occurrences, on the other hand mild condition may be pregnant, pregnancy condition can be improved or alleviated.