Nalesso Federico, Rigato Matteo, Cirella Irene, Protti Maria Paola, Zanella Ruggero, Rossi Bartolomeo, Putti Maria Caterina, Martino Francesca K, Calò Lorenzo A
Department of Medicine, University of Padova, 35128 Padova, Italy.
Haematology-Oncology Clinic, Women and Child's Health Department, University of Padua, 35122 Padua, Italy.
J Clin Med. 2022 Nov 15;11(22):6752. doi: 10.3390/jcm11226752.
Beta-thalassemia syndromes are the most common inherited monogenic disorders worldwide. The most common pathophysiologic and clinical renal disease manifestations of in β-TM patients is the tubular dysfunctions related to iron overload, chronic anemia, and the need for chronic iron chelation therapy. The aim of this pilot study is to apply an innovative ultrasound and Doppler technique to assess the Renal Functional Reserve (RFR) in β-TM patients, and to evaluate its reliability in iron overload tubulopathy. Ultrasound assessment of intra-parenchymal renal resistive index variation (IRRIV) has recently been proposed as a safe and reproducible technique to identify RFR presence. We define the preserved RFR when the Delta Renal Resistive Index (RRI) is >0.05 (baseline RRI—minimum RRI value during stress) in the Renal Stress Test (RST). Nineteen β-TM patients were enrolled for this study. In our series, we found a strong negative correlation between mean ferritin values and Delta RRI (R = −0.51, p = 0.03). This pilot study suggested the RST as reliable tool for assessing the RFR by ultrasound. Specifically, RST could help in clinical practice suggesting the patient’s management and iron chelation therapy.
β地中海贫血综合征是全球最常见的遗传性单基因疾病。β-TM患者最常见的病理生理和临床肾脏疾病表现是与铁过载、慢性贫血以及慢性铁螯合治疗需求相关的肾小管功能障碍。本初步研究的目的是应用一种创新的超声和多普勒技术评估β-TM患者的肾功能储备(RFR),并评估其在铁过载肾小管病中的可靠性。肾实质内电阻抗指数变化(IRRIV)的超声评估最近被提议作为一种识别RFR存在的安全且可重复的技术。我们将肾应激试验(RST)中Δ肾电阻抗指数(RRI)>0.05(基线RRI - 应激期间最小RRI值)定义为RFR保留。本研究纳入了19例β-TM患者。在我们的系列研究中,我们发现平均铁蛋白值与ΔRRI之间存在强烈的负相关(R = -0.51,p = 0.03)。这项初步研究表明RST是通过超声评估RFR的可靠工具。具体而言,RST有助于临床实践中指导患者管理和铁螯合治疗。