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首页> 外文期刊>Iranian journal of pediatrics >A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding
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A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding

机译：非典型麦昆-奥尔布赖特综合征伴阴道流血一例

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BackgroundMcCune-Albright syndrome (MAS) is a rare non-inherited disorder characterized by the clinical triad of precocious puberty, cafe-au-lait skin lesions, and fibrous dysplasia of bone.Case PresentationWe report a girl with MAS, presenting initially with vaginal bleeding at the age of 17 months. Ultrasonography revealed unilateral ovarian cysts and ureteral and ovarian enlargement. Considering the clinical and paraclinical findings, the patient diagnosed as a case of gonadotropin-independent precocious puberty was treated with medroxy-progestrone acetate (MPA) for three months. During the follow up, recurrent episodes of bleeding, ovarian activation and cyst formation, as well as breast size development were reported. At the age of 5.5 years, fibrous dysplasia was detected, which in coexistence with precocious puberty confirmed the diagnosis of MAS. The patient had no cafe-au-lait skin macles during follow up.ConclusionConsidering that clinical manifestations of MAS appear later in the course of recurrent periods of ovarian activation and cyst formation, a careful clinical observation and follow up of patients is necessary and the diagnosis of MAS must be kept in mind in cases with gonadotropin-independent precocious puberty.
机译：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异常增生的临床三联征。病例介绍我们报告了一个患有MAS的女孩，最初表现为阴道出血。在17个月大时。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，对诊断为促性腺激素独立性早熟的患者进行了醋酸甲羟孕酮（MPA）治疗三个月。在随访期间，据报道有反复发作的出血，卵巢活化和囊肿形成以及乳房变大的情况。在5.5岁时，发现了纤维异常增生，与性早熟并存证实了MAS的诊断。该患者在随访过程中未见咖啡因黄斑。结论考虑到MAS的临床表现在卵巢活化和囊肿形成的复发期中较晚出现，因此需要对患者进行仔细的临床观察和随访，并进行诊断在非促性腺激素依赖性性早熟的情况下，必须牢记MAS的使用。

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