The relationship between liver stiffness by two-dimensional shear wave elastography and iron overload status in transfusion-dependent patients.

IF 1.2 4区 医学 Q4 HEMATOLOGY
Pediatric Hematology and Oncology Pub Date : 2024-09-01 Epub Date: 2024-07-09 DOI:10.1080/08880018.2024.2353900
Pimporn Puttawibul, Supika Kritsaneepaiboon, Thirachit Chotsampancharoen, Polathep Vichitkunakorn
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引用次数: 0

Abstract

Increased liver stiffness (LS) can be result of increased liver iron concentration (LIC) which may not yet be reflected in the liver fibrotic status. The objective of our study was to examine relationship between hemochromatosis, LS, and serum ferritin level in transfusion-dependent patients. We recruited all 70 transfusion-dependent patients, whose median age was 15, referred for evaluating LIC status by magnetic resonance imaging (MRI) followed by two-dimensional ultrasonography shear wave elastography (2D-SWE). Thalassemia beta affected the majority of the patients. The optimal cut point for prediction of severe hemochromatosis using median SWE (kPa) and SWV (m/s) was ≥ 7.0 kPa and ≥ 1.54 m/s, respectively, with sensitivity of 0.76 (95% confidence interval [CI] 0.55, 0.91) and, specificity of 0.69 (95%CI 0.53, 0.82). When combing the optimal cut point of SWE (kPa) at ≥ 7.0 and serum ferritin ≥ 4123 ng/mL, the sensitivity increased to 0.84 (95%CI 0.64, 0.95) with specificity of 0.67 (95%CI 0.50, 0.80), positive predictive value (PPV) of 0.60 (95%CI 0.42, 0.76), and negative predictive value (NPV) of 0.88 (95%CI 0.71, 0.96). Simultaneous tests of 2D-SWE and serum ferritin for prediction of severe hemochromatosis showed the highest sensitivity of 84% (95%CI 0.64-0.95), as compared to 2D-SWE alone at 76% (95%CI 0.55, 0.91) or serum ferritin alone at 44% (95%CI 0.24-0.65). We recommend measuring both 2D-SWE and serum ferritin in short interval follow up patients. Adding 2D-SWE to management guideline will help in deciding for aggressive adjustment of iron chelating medication and increased awareness of patients having severe hemochromatosis.

通过二维剪切波弹性成像检测输血依赖型患者肝脏硬度与铁超负荷状态之间的关系。
肝硬变(LS)的增加可能是肝铁浓度(LIC)增加的结果,而肝铁浓度的增加可能尚未反映在肝纤维化状态中。我们的研究旨在探讨输血依赖型患者血色素沉着病、肝硬变和血清铁蛋白水平之间的关系。我们招募了所有 70 名输血依赖型患者(中位年龄为 15 岁),通过磁共振成像(MRI)和二维超声剪切波弹性成像(2D-SWE)评估 LIC 状态。大多数患者都患有地中海贫血。使用中位 SWE(kPa)和 SWV(m/s)预测重度血色素沉着病的最佳切点分别是≥7.0 kPa 和≥1.54 m/s,灵敏度为 0.76(95% 置信区间 [CI] 0.55,0.91),特异性为 0.69(95%CI 0.53,0.82)。将 SWE (kPa) ≥ 7.0 和血清铁蛋白 ≥ 4123 ng/mL 的最佳切点结合起来,灵敏度增加到 0.84(95%CI 0.64,0.95),特异性为 0.67(95%CI 0.50,0.80),阳性预测值 (PPV) 为 0.60(95%CI 0.42,0.76),阴性预测值 (NPV) 为 0.88(95%CI 0.71,0.96)。同时检测 2D-SWE 和血清铁蛋白预测重度血色病的灵敏度最高,为 84% (95%CI 0.64-0.95),而单独检测 2D-SWE 的灵敏度为 76% (95%CI 0.55, 0.91) 或单独检测血清铁蛋白的灵敏度为 44% (95%CI 0.24-0.65)。我们建议在短间隔随访患者中同时测量 2D-SWE 和血清铁蛋白。将 2D-SWE 纳入管理指南将有助于决定是否积极调整除铁药物,并提高对严重血色沉着病患者的认识。
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来源期刊
CiteScore
2.60
自引率
5.90%
发文量
71
审稿时长
6-12 weeks
期刊介绍: PHO: Pediatric Hematology and Oncology covers all aspects of research and patient management within the area of blood disorders and malignant diseases of childhood. Our goal is to make PHO: Pediatric Hematology and Oncology the premier journal for the international community of clinicians and scientists who together aim to define optimal therapeutic strategies for children and young adults with cancer and blood disorders. The journal supports articles that address research in diverse clinical settings, exceptional case studies/series that add novel insights into pathogenesis and/or clinical care, and reviews highlighting discoveries and challenges emerging from consortia and conferences. Clinical studies as well as basic and translational research reports regarding cancer pathogenesis, genetics, molecular diagnostics, pharmacology, stem cells, molecular targeting, cellular and immune therapies and transplantation are of interest. Papers with a focus on supportive care, late effects and on related ethical, legal, psychological, social, cultural, or historical aspects of these fields are also appreciated. Reviews on important developments in the field are welcome. Articles from scientists and clinicians across the international community of Pediatric Hematology and Oncology are considered for publication. The journal is not dependent on or connected with any organization or society. All submissions undergo rigorous peer review prior to publication. Our Editorial Board includes experts in Pediatric Hematology and Oncology representing a wide range of academic and geographic diversity.
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