Van Wyk-Grumbach综合征1例报告及文献复习

Van Wyk and Grumbach syndrome:A case report and literature review

目的:分析1例Van Wyk-Grumbach综合征(VWGS)患者的临床资料,探讨其发病机制、临床特点、诊断、鉴别诊断和治疗方法。方法:回顾性分析1例VWGS患者的病史、辅助检查及诊疗经过。结果:治疗前该患者表现为甲状腺功能低下、双侧卵巢囊肿、垂体泌乳素(PRL)水平明显升高和垂体占位性病变。甲状腺素替代治疗后患者甲状腺功能、PRL基本恢复正常,卵巢囊肿明显缩小。结论:VWGS患者的临床表现比较特殊,常被误诊为垂体肿瘤或卵巢肿瘤而行手术治疗,掌握其临床特点有助于正确诊断,确诊后以甲状腺素替代治疗为主,可避免不必要的手术。

Objective：To analyze the clinical materials of the patient with Van Wyk and Grumbach syndrome （VWGS）,and to explore the pathogenesis,clinical characteristics,diagnosis,differential diagnosis and treatment method.Methods：The clinical data of a patient with VWGS was analyzed retrospectively.Results：The patient presented autoimmune thyroiditis, sever long-standing hypothyroidism, bilateral ovarian cysts, hyperprolactinaemia and a secondary pituitary adenoma. After thyroxine replacement treatment, the thyroid function and prolactin （PRL）level recovered to the normal levels and the ovarian cyst was shrunk within 1 month. Conclusion：The clinical manifestation of VWGS is special.And it’s easy to misdiagnose this syndrome as pituitary adenoma or ovary cyst and then operation is performed.Thyroid hormone replacement can completely resolve the symptoms and hormone abnormalities.Grasping the clinical characteristics of this syndrome and making correct diagnosis is critical to avoid unnecessary operation.