Key result
Early treatment with hydroxyurea or transfusion at age <6 years in patients with sickle cell disease resulted in extracellular volume fraction levels comparable to normal controls.
Why the study?
Does early initiation of hydroxyurea or transfusion prevent increased extracellular volume fraction in patients with sickle cell disease?
Does early initiation of hydroxyurea or transfusion prevent increased extracellular volume fraction in patients with sickle cell disease?
Early initiation of disease-modifying therapy in sickle cell disease may prevent the development of diffuse myocardial fibrosis as measured by ECV.
Cardiovascular disease is a major cause of mortality in patients with sickle cell disease (SCD). Niss et al previously reported that cardiac magnetic resonance in 25 patients showed universal myocardial fibrosis, which they correlated with increased extracellular volume fraction (ECV). In the current study, they compared patients with SCD who were treated with hydroxyurea or transfusion at age <6 years to a group of patients with SCD without therapy. They documented that patients treated early had ECV levels comparable to normal controls, potentially preventing subsequent myocardial fibrosis.
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Niss et al. (2022) conducted an editorial in Sickle cell disease (SCD). Hydroxyurea or transfusion at age <6 years vs. Patients with SCD without therapy was evaluated on Extracellular volume fraction (ECV) levels. Early treatment with hydroxyurea or transfusion at age <6 years in patients with sickle cell disease resulted in extracellular volume fraction levels comparable to normal controls.
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