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A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding

机译:一例非典型麦昆-奥尔布赖特综合症伴阴道流血

摘要

AbstractudBackground: McCune-Albright syndrome (MAS) is a rare non-inherited disorder characterized byudthe clinical triad of precocious puberty, cafe-au-lait skin lesions, and fibrous dysplasia of bone.udCase Presentation: We report a girl with MAS, presenting initially with vaginal bleeding at the ageudof 17 months. Ultrasonography revealed unilateral ovarian cysts and ureteral and ovarianudenlargement. Considering the clinical and paraclinical findings, the patient diagnosed as a case ofudgonadotropin-independent precocious puberty was treated with medroxy-progestrone acetateud(MPA) for three months. During the follow up, recurrent episodes of bleeding, ovarian activationudand cyst formation, as well as breast size development were reported. At the age of 5.5 years,udfibrous dysplasia was detected, which in coexistence with precocious puberty confirmed theuddiagnosis of MAS. The patient had no cafe-au-lait skin macles during follow up.udConclusion: Considering that clinical manifestations of MAS appear later in the course of recurrentudperiods of ovarian activation and cyst formation, a careful clinical observation and follow up ofudpatients is necessary and the diagnosis of MAS must be kept in mind in cases with gonadotropinindependentudprecocious puberty.
机译:摘要 ud背景:McCune-Albright综合征(MAS)是一种罕见的非遗传性疾病,其特征是性早熟,咖啡色皮肤损伤和骨纤维增生的临床三联征。 udCase Presentation:我们报告一个女孩使用MAS时,最初在17个月大时出现阴道流血。超声检查显示单侧卵巢囊肿以及输尿管和卵巢肿大。考虑到临床和临床外发现,对诊断为 udgonadotropin独立性早熟的患者进行了醋酸甲羟孕酮 ud(MPA)治疗三个月。在随访期间,据报道有反复发作的出血,卵巢活化 udand囊肿形成以及乳房增大的情况。在5.5岁时,检测到纤维异常,并与性早熟并存,证实了MAS的诊断。结论:考虑到MAS的临床表现出现在卵巢活化和囊肿形成的反复发作过程中,请仔细观察并随访患者。对于非促性腺激素依赖性/性早熟的患者,必须牢记MAS的诊断。

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