儿童Van Wyk-Grumbach综合征1例报告并文献复习

发布时间：2018-12-13 05:51

【摘要】：目的探讨Van Wyk-Grumbach综合征(VWGS)的早期诊断和治疗。方法回顾分析1例VWGS患儿的临床资料,并复习相关文献。结果患儿,女,9岁,因身高增长缓慢、肥胖3年,伴乳房增大6个月、阴道出血3个月就诊。游离甲状腺素(FT4)0.46 ng/d L,促甲状腺素(TSH)150 m IU/L;GnRH激发试验提示性腺轴未启动;血泌乳素、雌二醇明显升高;骨龄延迟;彩色超声示子宫、卵巢增大,可见卵巢囊肿;垂体MRI示腺垂体增生。予左甲状腺素钠治疗2个月后甲状腺功能恢复正常,复查彩超示双卵巢明显缩小,体质量减轻6 kg,未再阴道出血;3个月后双卵巢至正常大小,鞍区MRI占位缩小;6个月后复查彩色超声示子宫、卵巢大小正常,无卵巢囊肿,鞍区MRI占位消失。结论 VWGS是长期未经治疗的严重原发性甲状腺功能减退症少见的并发症,好发于青春期女童,甲状腺素替代治疗有效。
[Abstract]:Objective to investigate the early diagnosis and treatment of Van Wyk-Grumbach syndrome (VWGS). Methods the clinical data of one case with VWGS were retrospectively analyzed and the related literature was reviewed. Results the female, 9 years old, had a slow increase in height, obesity for 3 years, breast enlargement for 6 months and vaginal bleeding for 3 months. Free thyroxine (FT4) 0.46 ng/d / L, thyrotropin (TSH) 150m IU/L;GnRH excitation test showed that gonadal axis was not activated, serum prolactin and estradiol were significantly increased, bone age was delayed, and serum prolactin and estradiol were significantly increased. Color ultrasound showed uterus, ovarian enlargement, ovarian cyst, pituitary MRI showed adenohypophysis hyperplasia. The thyroid function returned to normal after 2 months of treatment with levathyroxine sodium. The color Doppler ultrasound showed that the diovaries were obviously reduced and the body mass was reduced for 6 kg, without vaginal bleeding, and after 3 months the size of the two ovaries reached the normal size and the MRI occupied the Sellar region decreased. After 6 months, color ultrasound showed normal ovarian size, no ovarian cyst, and MRI space occupying in Sellar region disappeared. Conclusion VWGS is a rare complication of severe primary hypothyroidism without treatment for a long time. It is more common in adolescent girls. Thyroxine replacement therapy is effective.
【作者单位】：江西省儿童医院内分泌遗传代谢科;
【分类号】：R725.8

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