Vasculopathy among children and adolescents with sickle cell disease: the crosstalk with annexin A1, vitamin D, and myocardial iron overload.

IF 2.3 4区医学 Q2 HEMATOLOGY

Expert Review of Hematology Pub Date : 2025-06-01 Epub Date: 2025-04-18 DOI:10.1080/17474086.2025.2495670

Mohsen Saleh ElAlfy, Eman Abdel Rahman Ismail, Sara Mostafa Makkeyah, Ahmed Samir, Dina Husseiny Salama, Nanis Mohammed Salah Eldin, Dina Mohamed Fathy ElMaghraby, Nada Ayman Gad, Marwa Fath Ahmed Ali, Fatma Soliman Elsayed Ebeid

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引用次数: 0

Abstract

Background: Annexin A1 plays an important role in myocardial defense against ischemia-reperfusion injury. We aimed to evaluate the role of annexin A1 as a potential marker of vasculopathy in children and adolescents with sickle cell disease (SCD) and its relation to myocardial iron content (MIC) and vitamin D status.

Research design and methods: Forty-one patients with SCD were compared with 40 age- and sex-matched healthy controls, and underwent assessment of serum annexin A1, vitamin D, Doppler echocardiography and cardiac magnetic resonance (CMR).

Results: Six (14.6%) SCD patients had cardiac disease, five (12.2%) had abnormal MIC (≥1.16) and 10 (24.4%) had pulmonary hypertension risk. Annexin A1 levels were significantly lower among patients with SCD compared with healthy controls (p < 0.001). SCD patients with pulmonary hypertension risk, evidence of diastolic dysfunction, and nephropathy as well as those with serum ferritin ≥ 2500 µg/L and vitamin D deficiency had lower Annexin A1 levels than those without. Serum annexin A1 levels were negatively correlated to urinary albumin creatinine ratio (UACR) and Tei index while positively correlated to vitamin D among SCD patients.

Conclusions: Annexin A1 could be a promising marker of vasculopathy and may provide a biochemical explanation for vitamin D deficiency in SCD.

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儿童和青少年镰状细胞病患者的血管病变：与膜联蛋白A1、维生素D和心肌铁超载的串扰

背景：膜联蛋白A1在心肌防御缺血再灌注损伤中起重要作用。我们的目的是评估膜联蛋白A1作为儿童和青少年镰状细胞病（SCD）血管病变的潜在标志物的作用及其与心肌铁含量（MIC）和维生素D状态的关系。研究设计和方法：将41例SCD患者与40例年龄和性别匹配的健康对照进行比较，并进行血清膜联蛋白A1、维生素D、多普勒超声心动图和心脏磁共振（CMR）的评估。结果：SCD患者合并心脏疾病6例（14.6%），MIC异常5例（12.2%）（≥1.16），合并肺动脉高压危险10例（24.4%）。与健康对照组相比，SCD患者的膜联蛋白A1水平明显降低(p结论：膜联蛋白A1可能是血管病变的一个有希望的标志物，可能为SCD患者维生素D缺乏提供生化解释。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Expert Review of Hematology HEMATOLOGY-

CiteScore

4.70

自引率

3.60%

发文量

审稿时长

6-12 weeks

期刊介绍： Advanced molecular research techniques have transformed hematology in recent years. With improved understanding of hematologic diseases, we now have the opportunity to research and evaluate new biological therapies, new drugs and drug combinations, new treatment schedules and novel approaches including stem cell transplantation. We can also expect proteomics, molecular genetics and biomarker research to facilitate new diagnostic approaches and the identification of appropriate therapies. Further advances in our knowledge regarding the formation and function of blood cells and blood-forming tissues should ensue, and it will be a major challenge for hematologists to adopt these new paradigms and develop integrated strategies to define the best possible patient care. Expert Review of Hematology (1747-4086) puts these advances in context and explores how they will translate directly into clinical practice.