论文部分内容阅读
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。
Objective To investigate the etiology, clinical manifestations, hormone secretion and treatment of Schwann syndrome. Methods The clinical data of 97 patients with Schwann syndrome diagnosed in the First Affiliated Hospital of Xinjiang Medical University from January 1999 to May 2013 were collected, including general information, clinical manifestations and physical examination, laboratory tests, anterior pituitary and target Gonadal hormone levels, imaging examination, treatment and effect. Results The age at diagnosis of 97 cases of Shein syndrome was (43.7 ± 12.4) years old and the time from obstetric pathology was (9.1 ± 9.5) years. 96 patients (99.0%) had a history of postpartum hemorrhage. 83 cases (85.6%) had armpit hair, pubic hair shedding, 80 cases (82.5%) had amenorrhea and 79 cases (81.4%) had hair and eyebrows sparse. Laboratory tests showed that hemoglobin was decreased by 74.4% (67/90), erythrocyte sedimentation rate was 56.0% (14/25), hypoglycemia was 26.4% (23/87), serum total triiodothyronine (TT3 ) Decreased 80.5% (70/87), the total thyroxine (TT4) level decreased 82.8% (72/87), the free triiodothyronine (FT3) level decreased 82.9% (34/41) The level of free thyroxine (FT4) decreased by 90.2% (37/41), the level of thyroid-stimulating hormone decreased by 82.8% (77/93), the level of prolactin decreased by 57.3% (47/82) and the level of follicle-stimulating hormone decreased Accounting for 88.0% (73/83), 92.8% (77/83) reduction of luteinizing hormone and 51.4% (19/37) reduction of adrenocorticotrophic hormone (10:00). Pituitary magnetic resonance imaging (MRI) was performed in 27 patients. No abnormalities were found in 5 of the 27 patients. The pituitary volume was slightly reduced in 2 patients and the pituitary volume was significantly decreased in 20 patients. The lesions were partially or completely vacuolar sella. 18 patients underwent gynecological B-ultrasound, 15 patients showed smaller uterine volume. 85 patients (87.6%) were given the corresponding target gland hormone replacement therapy, the symptoms improved significantly. Conclusion The postpartum hemorrhage is a common etiology of Shein syndrome. The main clinical manifestations of the target hypnotic hormone deficiency are hidden clinical manifestations, misdiagnosis, missed diagnosis and critically ill patients. Anterior pituitary dysfunction may occur in critically ill patients. The treatment should be actively applied the corresponding hormone replacement therapy.