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β地中海贫血患者肾上腺皮质功能不全:一项荟萃分析。

Adrenal Insufficiency in Patients with Beta Thalassemia: A Meta-Analysis.

机构信息

Department of Endocrinology, Hippocration General Hospital, GR-11527 Athens, Greece.

Thalassemia and Sickle Cell Unit, Expertise Center of Hemoglobinopathies and Their Complications, Hippocration General Hospital, GR-11527 Athens, Greece.

出版信息

Medicina (Kaunas). 2024 Sep 25;60(10):1571. doi: 10.3390/medicina60101571.

Abstract

: Adrenal insufficiency (AI) can be a significant concern in patients with transfusion-dependent homozygous beta thalassemia (bThal) due to the chronic disease burden and frequent blood transfusions that these patients require. The prevalence of AI in this population remains unclear, with studies often lacking control groups for comparison. This meta-analysis aimed to estimate the proportion of patients with transfusion-dependent bThal who exhibit evidence of AI. : A systematic review following PRISMA guidelines identified 19 studies for analysis. : Despite the variability in the diagnostic methods used to ascertain AI, the meta-analysis revealed that approximately one-third of patients had evidence of AI, with the prevalence rising to 50% in studies focused on adults with bThal. : These findings suggest an increased risk of AI in patients with bThal compared to the general population. Clinicians should consider tailored management strategies, including glucocorticoid coverage during surgical procedures, to mitigate the risk of adrenal crises in this vulnerable patient group. Further research is needed to optimize adrenal surveillance and management in patients with bThal.

摘要

肾上腺功能不全(AI)可能是依赖输血的纯合子β地中海贫血(bThal)患者的一个重要问题,因为这些患者需要长期的疾病负担和频繁的输血。由于研究通常缺乏对照组进行比较,因此该人群中 AI 的患病率尚不清楚。本荟萃分析旨在估计依赖输血的 bThal 患者中出现 AI 证据的比例。

按照 PRISMA 指南进行的系统评价确定了 19 项研究进行分析。

尽管用于确定 AI 的诊断方法存在差异,但荟萃分析显示,大约三分之一的患者有 AI 证据,在专门针对 bThal 成年患者的研究中,患病率上升至 50%。

这些发现表明,与一般人群相比,bThal 患者发生 AI 的风险增加。临床医生应考虑制定针对个体的管理策略,包括在手术过程中使用糖皮质激素覆盖,以降低这一脆弱患者群体发生肾上腺危象的风险。需要进一步研究来优化 bThal 患者的肾上腺监测和管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c061/11509364/e58a13da52cd/medicina-60-01571-g001.jpg

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