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Nephrological Complications in Hemoglobinopathies: SITE Good Practice
Giovan Battista Ruffo1, Rodolfo Russo2, Tommaso Casini3
1U.O. Ematologia con Talassemia, ARNAS Civico Di Cristina Benfratelli, 90127 Palermo, Italy.
Journal of Clinical Medicine
|December 9, 2023
Summary
This study provides recommendations for managing kidney issues in patients with hemoglobinopathies like thalassemia and sickle cell disease. Adhering to these guidelines can improve patient care and clinical outcomes.
Area of Science:
- Hematology
- Nephrology
- Genetics
Background:
- Hemoglobinopathies, including thalassemia syndromes and sickle cell disease (SCD), are prevalent monogenic disorders globally and endemic to Italy.
- Improved clinical management and aging populations with hemoglobinopathies are linked to increasing kidney function abnormalities, ranging from altered tests to chronic and end-stage renal disease.
Purpose of the Study:
- To establish "good practice (GP)" recommendations for the follow-up and therapy of kidney alterations in patients with transfusion-dependent thalassemia (TDT), non-transfusion-dependent thalassemia (NTDT), and SCD.
- To offer expert guidance on managing renal complications in these patient groups.
Main Methods:
- A literature review was conducted by hemoglobinopathy experts and nephrologists, covering publications from January 1, 2016, to December 31, 2022.
- The review extended to 5-7 years due to disease rarity and included pivotal studies or expert consensus when evidence-based data was limited.
Main Results:
- Question and answer boxes were developed to facilitate accessible consultation.
- A color-coded strategy and focused answers were employed for clarity.
Conclusions:
- The developed "good practice (GP)" guidelines aim to enhance clinical management strategies for patients with hemoglobinopathies.
- Implementation of these recommendations is expected to improve the overall quality of care for individuals affected by these genetic blood disorders.
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