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Thalass. Rep., Volume 12, Issue 1 (March 2022) – 5 articles

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6 pages, 165 KiB  
Editorial
Publisher’s Note: Page Numbers Changed to Article Numbers for Articles Published in Thalassemia Reports Volumes 1–11
by Thalassemia Reports Editorial Office
Thalass. Rep. 2022, 12(1), 24-29; https://doi.org/10.3390/thalassrep12010005 - 23 Feb 2022
Viewed by 1513
Abstract
From Volume 1 (2011) to Volume 11 (2021), Thalassemia Reports [...] Full article
10 pages, 1092 KiB  
Article
Random Forest Clustering Identifies Three Subgroups of β-Thalassemia with Distinct Clinical Severity
by Angela Vitrano, Khaled M. Musallam, Antonella Meloni, Sebastiano Addario Pollina, Mehran Karimi, Amal El-Beshlawy, Mahmoud Hajipour, Vito Di Marco, Saqib Hussain Ansari, Aldo Filosa, Paolo Ricchi, Adriana Ceci, Shahina Daar, Efthymia Vlachaki, Sylvia Titi Singer, Zaki A. Naserullah, Alessia Pepe, Salvatore Scondotto, Gabriella Dardanoni, Fedele Bonifazi, Vijay G. Sankaran, Elliott Vichinsky, Ali T. Taher, Aurelio Maggio and International Working Group on Thalassemia (IWG-THAL)add Show full author list remove Hide full author list
Thalass. Rep. 2022, 12(1), 14-23; https://doi.org/10.3390/thalassrep12010004 - 18 Feb 2022
Cited by 3 | Viewed by 2379
Abstract
In this work, we aimed to establish subgroups of clinical severity in a global cohort of β-thalassemia through unsupervised random forest (RF) clustering. We used a large global dataset of 7910 β-thalassemia patients and evaluated 19 indicators of phenotype severity (IPhS) to determine [...] Read more.
In this work, we aimed to establish subgroups of clinical severity in a global cohort of β-thalassemia through unsupervised random forest (RF) clustering. We used a large global dataset of 7910 β-thalassemia patients and evaluated 19 indicators of phenotype severity (IPhS) to determine their contribution and relatedness in grouping β-thalassemia patients into clusters using RF analysis. RF clustering suggested that three clusters with minimal overlapping exist (classification error rate: 4.3%), and six important IPhS were identified: the current age of the patient, the mean serum ferritin level, the age at diagnosis, the age at first transfusion, the age at first iron chelation, and the number of complications. Cluster 3 represented patients with early initiation of transfusion and iron chelation, considerable iron overload, and early mortality from heart failure. Patients in Cluster 2 had lower serum ferritin levels, although they had a higher number of complications manifesting overtime. Patients in Cluster 1 represented a subgroup with delayed or absent transfusion and iron chelation, but with a high morbidity rate. Hepatic disease and cancer were dominant causes of death in patients in Cluster 1 and 2. Our findings established that patients with β-thalassemia can be clustered into three groups based on six parameters of phenotype severity. Full article
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2 pages, 380 KiB  
Editorial
The New Voice for the New Era of Thalassemia Reports
by Aurelio Maggio
Thalass. Rep. 2022, 12(1), 12-13; https://doi.org/10.3390/thalassrep12010003 - 13 Jan 2022
Viewed by 1819
Abstract
The recent transfer of Thalassemia Reports, the only journal fully dedicated on Thalassemia, from PagePress to MDPI was great news for those who contributed to the spread of the journal [...] Full article
10 pages, 852 KiB  
Article
HeGRI: A Novel Index of Serum Hepcidin Suppression in Relation to the Degree of Renal Dysfunction among β-Thalassemia Major Patients
by Burhan A. Zaman, Suzan O. Rasool, Nashwan M. R. Ibrahim and Deldar M. Abdulah
Thalass. Rep. 2022, 12(1), 2-11; https://doi.org/10.3390/thalassrep12010002 - 15 Dec 2021
Viewed by 2572
Abstract
Background: The progressive renal function inadequacy results in altered hepcidin metabolism due to a shifting of its renal elimination, which consequently affects enteric iron absorption and iron stores’ availability. This study aimed to investigate and correlate renal function, iron status, and hepcidin in [...] Read more.
Background: The progressive renal function inadequacy results in altered hepcidin metabolism due to a shifting of its renal elimination, which consequently affects enteric iron absorption and iron stores’ availability. This study aimed to investigate and correlate renal function, iron status, and hepcidin in patients with β-thalassemia major through a novel index. Methods: In this 1:1 case–control study, serum hepcidin, serum ferritin, iron study, hematological and renal function parameters were compared between 60 β-thalassemia major patients with iron overload and 61 healthy individuals (2–30 years old). Results: The concentrations of serum hepcidin (21.898 vs. 9.941 ng/mL; p < 0.001) and eGFR (179.71 vs. 132.95; p < 0.001) were significantly higher in β-thalassemia major patients compared to the controls. The serum hepcidin levels decreased with increasing levels of total iron-binding capacity (TIBC; β = −0.442; p = 0.024), transferrin saturation (β = −0.343; p = 0.023), serum creatinine (β = −0.625; p = 0.0030), and eGFR (β = −0.496; p = 0.011). The mean hepcidin/ferritin ratio was significantly lower in the β-thalassemia major cases (0.0069 vs. 0.3970; p < 0.001). The novel hepcidin/eGFR ratio index (HeGRI) was significantly higher in the patient group compared to controls (0.12 vs. 0.09; p = 0.031), respectively. Conclusions: These results suggest that HeGRI could be a potential index of the appropriateness of serum hepcidin suppression associated with the degree of renal dysfunction among β-thalassemia major patients. Full article
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1 pages, 173 KiB  
Editorial
Publisher’s Note: Continued Publication of Thalassemia Reports by MDPI
by Enric Sayas
Thalass. Rep. 2022, 12(1), 1; https://doi.org/10.3390/thalassrep12010001 - 10 Dec 2021
Viewed by 1738
Abstract
Thalassemia Reports (ISSN: 2039-4365) was launched in 2011 and has become the premier peer-reviewed international medical journal devoted entirely to the study, diagnosis, and treatment of thalassemia [...] Full article
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