Thalassaemia (part 1)

Alli, N; Patel, Moosa

doi:10.7196/samj.2021.v111i6.15724

Cited by 3 publications

(2 citation statements)

References 17 publications

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“…Since HbA constitutes the major fraction of total Hb, the α-and β-thalassaemias represent the bulk of clinically significant thalassaemias, and are therefore the focus of this 2-part CME series. In this issue (part 1), [2] the discourse centres around epidemiology, pathophysiology, clinical exposition and diagnosis. Management issues are discussed in the forthcoming issue (part 2).…”

Section: Cmementioning

confidence: 99%

Thalassaemia (part 1)

2021

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Section: Cmementioning

confidence: 99%

Thalassaemia (part 1)

2021

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“…The combination of inefficient hemoglobin production, hemolysis, and ineffective erythropoiesis (i.e., destruction of red blood cell precursors in the bone marrow) results in severe anemia [5]. Lifelong regular blood transfusions and iron chelation are the cornerstones of disease management [6,7]. The introduction of routine transfusion therapy for the correction of anemia 60 years ago transformed β-TM from a fatal childhood illness into a chronic disorder.…”

Section: Introductionmentioning

confidence: 99%

Low Vitamin D Levels Are Associated with Increased Cardiac Iron Uptake in Beta-Thalassemia Major

Meloni,

Pistoia,

Vassalle

et al. 2023

Diagnostics

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We evaluated the association of vitamin D and parathormone (PTH) levels with cardiac iron and function in beta-thalassemia major (β-TM) patients. Two-hundred and seventy-eight TM patients (39.04 ± 8.58 years, 56.8% females) underwent magnetic resonance imaging for the assessment of iron overload (T2* technique), biventricular function parameters (cine images), and replacement myocardial fibrosis (late gadolinium enhancement technique). Vitamin D levels were deficient (<20 ng/dL) in 107 (38.5%) patients, insufficient (20–30 ng/dL) in 96 (34.5%) patients, and sufficient (≥30 ng/dL) in 75 (27.0%) patients. Deficient vitamin D patients had a significantly higher frequency of myocardial iron overload (MIO; global heart T2* < 20 ms) than patients with sufficient and insufficient vitamin D levels and a significantly higher left ventricular end-diastolic volume index and mass index than patients with sufficient vitamin D levels. PTH was not associated with cardiac iron, function, or fibrosis. In the multivariate regression analysis, vitamin D, serum ferritin, and pancreatic iron levels were the strongest predictors of global heart T2* values. In receiver operating characteristic curve analysis, a vitamin D level ≤ 17.3 ng/dL predicted MIO with a sensitivity of 81.5% and a specificity of 75.3% (p < 0.0001). In TM, the periodic and regular assessment of vitamin D levels can be beneficial for the prevention of cardiac iron accumulation and subsequent overt dysfunction.

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