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儿童自身免疫性上腺功能缺陷:多腺体综合征2型的暗示?
Marta Arrigoni1, Paolo Cavarzere2, Lara Nicolussi Principe1
1Pediatric Division, Department of Pediatrics, University Hospital of Verona, Piazzale Stefani 1, 37126, Verona, Italy.
自体免疫多腺体综合征2型 (APS2型) 在儿童中很少见,并且经常潜伏地呈现. 对上腺功能不全的早期发现和专业护理对于管理这种情况至关重要.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 自免疫性疾病 自免疫性疾病
- 内分泌系统疾病 内分泌系统疾病
背景情况:
- 儿童的原发性上腺功能缺陷 (PAI) 通常是由受损的类固醇生成引起的.
- 自身免疫性上腺炎是PAI在儿童时期的不常见原因,与成人PAI不同.
- 2型自身免疫多腺体综合征 (APS),其特征是自身免疫阿迪逊病,1型糖尿病和/或哈西莫托病,在儿童群体中很少见.
研究的目的:
- 突出儿童2型APS的罕见性和潜在潜伏性.
- 强调早期诊断和治疗儿科患者上腺功能衰竭的重要性.
- 强调有自身免疫性疾病的儿童需要定期的随访.
主要方法:
- 一个8岁女孩被诊断患有阿迪森氏病和自身免疫性甲状腺炎的病例报告.
- 描述了由上腺功能衰竭危机标志着的初始疾病过程.
- 在第三级医院进行管理调整,从而改善疾病控制.
主要成果:
- 该患者出现了早期出现的亚迪逊病和自身免疫性甲状腺炎.
- 最初的管理涉及解决多次上腺功能衰竭危机.
- 在一个专门的中心优化治疗导致了更好的疾病管理.
结论:
- 2型APS在儿童中很少被诊断出来,因为它有可能延长潜伏期.
- 建议在三级中心早期识别和专家管理上腺功能不全.
- 对于患有自身免疫性疾病的患者来说,定期监测是必不可少的,因为儿科病例中不完整或未被诊断的形式的频率很高.
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