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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
[Cardiac complications of sickle cell disease in children]
I Astadicko1, M F Dresse1, M C Seghaye1
1Service de Pédiatrie, CHU de Liège, Belgique.
Insights
Sickle cell disease (SCD) can cause serious heart problems, including cardiomyopathy and pulmonary hypertension, particularly in children. Early diagnosis and management are key to improving patient care and outcomes.
Area of Science:
- Hematology
- Cardiology
- Genetics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder affecting hemoglobin.
- Key manifestations include hemolysis, infections, and vaso-occlusive crises leading to organ damage.
- Cardiac complications significantly contribute to SCD morbidity and mortality.
Purpose of the Study:
- To review the pathophysiology of cardiac complications in SCD.
- To highlight diagnostic tools for early recognition of cardiac issues in SCD patients.
- To improve patient care strategies for SCD-related cardiac conditions.
Main Methods:
- Literature review focusing on pathophysiological aspects.
- Analysis of diagnostic tools for cardiac complications in SCD.
- Synthesis of current knowledge on SCD cardiac manifestations.
Main Results:
- Cardiac issues in SCD arise from chronic anemia, vaso-occlusion, iron overload, and organ damage.
- In children, dilated or restrictive cardiomyopathy and pulmonary arterial hypertension are primary concerns.
- While acute events are rare, chronic cardiac damage impacts long-term health.
Conclusions:
- Cardiac complications are a critical, often under-recognized, aspect of SCD.
- Early identification through appropriate diagnostics is essential for effective management.
- Understanding pathophysiology aids in developing better care protocols for SCD patients.
Abstract:
Sickle cell disease (SCD) is a genetic disorder due to an abnormal gene coding for the chain ? of the hemoglobin. The main clinical manifestations related to the major forms of SCD (SS-, SC-, and S-thalassemia) are chronic hemolysis, susceptibility to infections and vasoconstrictive crisis causing micro-emboli and/or infarction responsible for acute or chronic organ lesions. The latest are enhanced by tissue iron overload due to repeated blood transfusions. Cardiac complications are an important part of morbidity and mortality of SCD. They are due to chronic anemia, vaso-occlusive crisis, iron overload, pulmonary, renal and hepatic damage. In children, cardiac complications of SCD are not sufficiently identified. They principally consist in dilated or restrictive cardiomyopathy and pulmonary arterial hypertension. Sudden death and acute cardiac failure due to myocardial infarction or arrhythmias have become exceptional. This review focuses on the pathophysiological aspects of cardiac complications of SCD and on the diagnostic tools allowing their early recognition and improvement of patient care.
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