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The Assessment of Renal Functional Reserve in β-Thalassemia Major Patients by an Innovative Ultrasound and Doppler Technique: A Pilot Study

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...

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Autores principales: Nalesso, Federico, Rigato, Matteo, Cirella, Irene, Protti, Maria Paola, Zanella, Ruggero, Rossi, Bartolomeo, Putti, Maria Caterina, Martino, Francesca K., Calò, Lorenzo A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9699445/
https://www.ncbi.nlm.nih.gov/pubmed/36431228
http://dx.doi.org/10.3390/jcm11226752
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author Nalesso, Federico
Rigato, Matteo
Cirella, Irene
Protti, Maria Paola
Zanella, Ruggero
Rossi, Bartolomeo
Putti, Maria Caterina
Martino, Francesca K.
Calò, Lorenzo A.
author_facet Nalesso, Federico
Rigato, Matteo
Cirella, Irene
Protti, Maria Paola
Zanella, Ruggero
Rossi, Bartolomeo
Putti, Maria Caterina
Martino, Francesca K.
Calò, Lorenzo A.
author_sort Nalesso, Federico
collection PubMed
description 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.
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spelling pubmed-96994452022-11-26 The Assessment of Renal Functional Reserve in β-Thalassemia Major Patients by an Innovative Ultrasound and Doppler Technique: A Pilot Study Nalesso, Federico Rigato, Matteo Cirella, Irene Protti, Maria Paola Zanella, Ruggero Rossi, Bartolomeo Putti, Maria Caterina Martino, Francesca K. Calò, Lorenzo A. J Clin Med Article 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. MDPI 2022-11-15 /pmc/articles/PMC9699445/ /pubmed/36431228 http://dx.doi.org/10.3390/jcm11226752 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Nalesso, Federico
Rigato, Matteo
Cirella, Irene
Protti, Maria Paola
Zanella, Ruggero
Rossi, Bartolomeo
Putti, Maria Caterina
Martino, Francesca K.
Calò, Lorenzo A.
The Assessment of Renal Functional Reserve in β-Thalassemia Major Patients by an Innovative Ultrasound and Doppler Technique: A Pilot Study
title The Assessment of Renal Functional Reserve in β-Thalassemia Major Patients by an Innovative Ultrasound and Doppler Technique: A Pilot Study
title_full The Assessment of Renal Functional Reserve in β-Thalassemia Major Patients by an Innovative Ultrasound and Doppler Technique: A Pilot Study
title_fullStr The Assessment of Renal Functional Reserve in β-Thalassemia Major Patients by an Innovative Ultrasound and Doppler Technique: A Pilot Study
title_full_unstemmed The Assessment of Renal Functional Reserve in β-Thalassemia Major Patients by an Innovative Ultrasound and Doppler Technique: A Pilot Study
title_short The Assessment of Renal Functional Reserve in β-Thalassemia Major Patients by an Innovative Ultrasound and Doppler Technique: A Pilot Study
title_sort assessment of renal functional reserve in β-thalassemia major patients by an innovative ultrasound and doppler technique: a pilot study
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9699445/
https://www.ncbi.nlm.nih.gov/pubmed/36431228
http://dx.doi.org/10.3390/jcm11226752
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