中国实用儿科杂志

中国实用儿科杂志 ›› 2025, Vol. 40 ›› Issue (9): 788-791.DOI: 10.19538/j.ek2025090616

• 病例报告 • 上一篇    下一篇

中枢性性早熟伴血管炎相关性烟雾综合征1例报告

  

  1. 中山大学附属第一医院  a 儿科内分泌，b 放射科，广东  广州  510080
  • 出版日期:2025-09-06 发布日期:2025-10-21
  • 通讯作者: 陈秋莉，电子信箱：13682244543@163.com

Central precocious puberty with vasculitis-related moyamoya syndrome：one case report

  1. *Department of Endocrinology Pediatrics，the First Affiliated Hospital，SunYat-Sen University，Guangzhou  510080，China
  • Online:2025-09-06 Published:2025-10-21

摘要： 1例3岁1月龄女性患儿，因“乳房发育伴生长加速1年半”于2020年11月就诊于中山大学附属第一医院儿科，经检查确诊为中枢性性早熟（central precocious puberty，CPP）。入院后行垂体磁共振成像（MRI）、磁共振血管成像（MRA）、磁共振波谱（MRS）、弥散加权成像（DWI）及磁敏感加权成像（SWI）等检查，诊断为血管炎相关性烟雾综合征。因促性腺激素释放激素类似物（GnRHa）治疗后患儿骨龄（BA）控制欠佳，查17羟孕酮（17OHP）偏高，行促肾上腺皮质激素（ACTH）激发试验，考虑有非经典型先天性肾上腺皮质增生症（NCCAH）可能。该例CPP伴血管炎相关性烟雾综合征罕见，提示血管炎相关性烟雾综合征亦可能为CPP病因。

关键词: 中枢性性早熟, 血管炎相关性烟雾综合征, 非经典型先天性肾上腺皮质增生症

Abstract: A 3-year-old and 1-month-old female child visited the Department of Pediatric Endocrinology of the First Affiliated Hospital of Sun Yat-sen University in November 2020 due to“breast development with accelerated growth for 1.5 years”. After examination，she was diagnosed with central precocious puberty（CPP）. After admission，pituitary magnetic resonance imaging（MRI），magnetic resonance angiography（MRA），magnetic resonance spectroscopy（MRS），diffusion-weighted imaging（DWI）+susceptibility weighted imaging（SWI） and other examinations were performed，and the diagnosis was vasculitis-related smoke syndrome. Due to poor control of bone age（BA） in the child after treatment with gonadotropin-releasing hormone analog（GnRHa），17-hydroxyprogesterone（17OHP） was checked. The level of 17OHP was elevated，and the adrenocorticotrophic hormone（ACTH） stimulation test was performed，which indicated the possibility of non-classical congenital adrenal hyperplasia（NCCAH）. This case of CPP accompanied by vasculitisassociated smoke syndrome is rare，suggesting that vasculitis-associated smoke syndrome can also be a cause of CPP.

Key words: central precocious puberty, vasculitis-related moyamoya syndrome, non-classical congenital adrenal hyperplasia