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目的通过对小脑扁桃体下疝畸形(Chiari畸形)合并垂体前叶激素缺乏患者临床特征进行分析,加强对本病的认识。方法分析2010年9月—2014年6月中国人民解放军总医院内分泌科住院的3例Chiari畸形合并垂体前叶激素缺乏患者一般资料、临床表现、实验室检查结果和影像学检查结果。结果 3例患者外生殖器阴毛稀疏或无,阴茎短小;患者1、3身材矮小,3例患者骨龄均落后2~3年、心功能未见异常。内分泌功能检查显示,患者1生长激素(GH)、促肾上腺皮质激素(ACTH)、促甲状腺激素(TSH)、促黄体生成素(LH)、促卵泡刺激素(FSH)均缺乏,患者2垂体前叶激素LH、FSH缺乏,患者3垂体前叶激素GH缺乏。MRI显示3例患者均表现为小脑扁桃体下疝畸形,患者1、3伴脊髓空洞症;患者1垂体前叶高度降低,垂体后叶正常短T1信号影未显示,垂体柄未显示;患者2垂体略变窄,高度约4 mm,左翼见稍长T1信号影,增强扫描后呈相对稍低T1信号影;患者3 T1信号影未见明显异常。结论 Chiari畸形患者临床表现多样,可以合并垂体前叶激素缺乏,MRI检查可伴脊髓空洞症或T1信号影异常。
Objective To analyze the clinical features of the anterior pituitary hormones deficit associated with cerebellar tonsil hernia deformity (Chiari’s deformity) and to enhance its understanding of the disease. Methods From September 2010 to June 2014, general information, clinical manifestations, laboratory findings and imaging findings of 3 Chiari malformation patients with anterior pituitary hormone deficiency who were hospitalized in Department of Endocrinology, Chinese PLA General Hospital were analyzed. Results In 3 cases, the external genital pubic hair was sparse or absent, and the penis was short. Patients 1 and 3 were short stature. The bone age of 3 patients lasted 2 to 3 years and their cardiac function was abnormal. Endocrine function tests showed that patients had a lack of growth hormone (GH), adrenocorticotropic hormone (ACTH), thyrotropin (TSH), luteinizing hormone (LH) and follicle stimulating hormone (FSH) Lutein LH, FSH deficiency, lack of anterior pituitary hormone GH in patients with 3. MRI showed three cases of patients with cerebellar tonsil hernia deformity, patients with 1,3 syringomyelia; patients 1 anterior pituitary height was reduced, the normal short posterior pituitary T1 signal was not shown, the pituitary stalk was not displayed; patient 2 pituitary Slightly narrower, a height of about 4 mm, the left wing to see a slightly longer T1 signal shadow, enhanced scan showed a relatively lower T1 signal shadow; patient 3 T1 signal shadow no significant abnormalities. Conclusions Chiari malformation patients with various clinical manifestations may be combined with anterior pituitary hormone deficiency, MRI may be associated with syringomyelia or T1 signal abnormalities.