上腺功能障碍的谱在血红蛋白E/贝塔血症中
Arijit Singha1, Pradip Mukhopadhyay1, Sujoy Ghosh1
1Department of Endocrinology and Metabolism, Institute of Post Graduate Medical Education and Research, 244 AJC Bose Road, Kolkata, 700020, India.
The Journal of clinical endocrinology and metabolism
|September 29, 2023
概括
上腺功能不充分 (AI) 是常见的血红蛋白E (HbE) /贝塔血症,影响41%的研究患者. 它的患病率与疾病严重程度或输血需求无关.
科学领域:
- 内分泌学 在内分泌学.
- 血液学 血液学 血液学
- 内部医学 内部医学
背景情况:
- 上腺功能缺陷 (AI) 的评估,包括矿物质皮质醇轴,以前没有在血红蛋白E (HbE) /β血症中进行研究.
- 这项研究解决了在这个特定患者群体内了解AI患病率的差距.
研究的目的:
- 为了确定患有HbE/β血病的患者中AI的患病率.
- 调查AI患病率是否与疾病严重程度或HbE/β血症中输血要求相关.
主要方法:
- 一项观察性,横截面研究涉及104名患有HbE/β血症的患者.
- 评估了清晨早期的血清皮质醇,血ACTH和血阿尔多 / 水平.
- 使用1μg和250μg短赛纳测试,对患者的基线皮质醇在5-18μg/dL之间进行分类,以对上腺功能进行分类.
主要成果:
- 在41%的患者 (n=43) 中检测到上腺功能不足 (AI),其中83.7%是初级AI,16.3%是二级AI.
- 根据特定的荷尔蒙标准,31%的患者 (n=33) 诊断出亚临床AI.
- 根据输血依赖 (P=.56) 或疾病严重程度 (P=.52) 观察到AI患病率没有显著差异.
结论:
- 上腺功能不充分是HbE/β血症的常见并发症.
- 在这种情况下,AI的发生是独立于输血依赖和疾病严重程度.
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