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. 2023 Dec 2;12(23):7476.
doi: 10.3390/jcm12237476.

Nephrological Complications in Hemoglobinopathies: SITE Good Practice

Giovan Battista Ruffo  1 , Rodolfo Russo  2 , Tommaso Casini  3 , Letizia Lombardini  4 , Valeria Orecchia  5 , Vincenzo Voi  6 , Raffaella Origa  7 , Gian Luca Forni  8 , Monia Marchetti  9 , Antonia Gigante  10 , Giacomo Garibotto  11 , Aurelio Maggio  12 , Lucia De Franceschi  13
Affiliations

Nephrological Complications in Hemoglobinopathies: SITE Good Practice

Giovan Battista Ruffo et al. J Clin Med. .

Abstract

Background: Hemoglobinopathies, among which thalassemic syndromes (transfusion-dependent and non-transfusion dependent thalassemias) and sickle cell disease (SCD), are the most widespread monogenic diseases worldwide. Hemoglobinopathies are endemic and spread-out all-over Italy, as result of internal and external migration flows. Nowadays, the increase therapeutic options associated to the general aging of patients with hemoglobinopathies related to the improvement in clinical management, contribute to the abnormalities in kidney function going from blood and urine test alterations to chronic kidney disease and end stage renal disease.

Methods: Here, we carried out a revision of the literature as panel of recognized experts in hemoglobinopathies with the consultancy and the revision of two nephrologists on kidney alteration and kidney disease in patients with TDT, NTDT and SCD. This is part of the action of the Italian society for the study of thalassemia and hemoglobinopties (SITE). The purpose of this "good practice (GP)" is to provide recommendations for follow-up and therapy for the management of kidney alterations in patients with TDT, NTDT and SCD. The literature review covers the period 1.1.2016 to 31.12.2022. In consideration of the rarity of these diseases, the analysis was extended from 5 to 7 years. Moreover, in the absence of relevant scientific papers in the identified time frame, we referred to pivotal or population studies, when available. Finally, in the absence of evidence-based data from prospective and randomized trials, the authors had to refer to expert opinion (expert consensus) for many topics.

Results: We generated question and answer boxes to offer a friendly consultation, using color code strategy and focused answers.

Conclusions: The present GP will help in improving the clinical management, and the quality of care of patients with hemoglobinopathies.

Keywords: dynamic programming; hedging; post-decision state variable; risk management; transaction costs.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
PRISMA flow-diagram for screening process of the literature. #: number; PRISMA: Preferred Reporting Items for Systematic Reviews and Meta-Analyses.
See this image and copyright information in PMC

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