Departments of Pediatric Endocrinology and Diabetology, University of Turin, Regina Margherita Children's Hospital, Turin, Italy.
Horm Res Paediatr. 2012;78(3):151-7. doi: 10.1159/000342641. Epub 2012 Sep 20.
To date, there is no agreement about the frequency or the features of thyroid abnormalities in McCune-Albright syndrome (MAS). The aim of our study was to detect thyroid abnormalities in a cohort of MAS children and adolescents and to give indications for their treatment and follow-up.
In 36 patients, 22 females and 14 males, thyroid function and sonographic features of thyroid were evaluated every 6-12 months.
Three males and 1 female had hyperthyroidism: 2 with nodular, 2 with diffuse goiters. They were treated with methimazole (0.2-0.5 mg/kg/day) with good clinical and biochemical responses. The remaining 32 patients were euthyroid, even if 7 displayed sonographic alterations, of whom 5 had nodular goiter with nodules >1 cm, and 2 micronodular goiter. Fine-needle aspiration biopsy was performed in 2 patients with nodules >1 cm, 1 showing hemorrhagic nodule and 1 colloid cystic nodule.
Prevalence of thyroid alterations in the studied MAS series was 31%. 64% of 11 patients with thyroid alterations had nodular goiters, with nodules >1 cm. As the onset of thyroid disease ranged from 1 to 20 years, a strict monitoring of thyroid function is recommended every 6 months. Satisfactory treatment can be obtained and maintained with antithyroid drugs.
迄今为止,关于 McCune-Albright 综合征(MAS)患者甲状腺异常的频率或特征尚无共识。我们研究的目的是检测 MAS 患儿和青少年的甲状腺异常,并为其治疗和随访提供建议。
对 36 例患者(22 名女性和 14 名男性)进行了甲状腺功能和甲状腺超声特征的评估,每 6-12 个月评估一次。
3 名男性和 1 名女性患有甲状腺功能亢进症:2 例为结节性甲状腺肿,2 例为弥漫性甲状腺肿。他们接受了甲巯咪唑(0.2-0.5 mg/kg/天)治疗,取得了良好的临床和生化反应。其余 32 例患者甲状腺功能正常,尽管 7 例存在超声改变,其中 5 例为>1 cm 的结节性甲状腺肿,2 例为微结节性甲状腺肿。对 2 例>1 cm 的结节患者进行了细针穿刺活检,其中 1 例为出血性结节,1 例为胶样囊性结节。
在研究的 MAS 系列中,甲状腺改变的患病率为 31%。11 例甲状腺改变患者中 64%有>1 cm 的结节性甲状腺肿。由于甲状腺疾病的发病年龄从 1 岁到 20 岁不等,建议每 6 个月监测一次甲状腺功能。使用抗甲状腺药物可以获得并维持满意的治疗效果。