Blood Transfusion Vs. Hydroxyurea for Stroke Prevention in Children With Sickle Cell Anemia: A Systematic Review and Meta-Analysis

Hafiz, Tamara A; Aldharman, Sarah S; AlSubaie, Ruby N; Alzahrani, Lena D; Albalawi, Ibrahim Ahmed J; Alali, Yara A; Yousuf, Maisaa M; Alharbi, Hayat M; Alamri, Nawaf S; Jamil, Syed Furrukh

doi:10.7759/cureus.31778

Cited by 3 publications

(3 citation statements)

References 47 publications

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“…The pathophysiological alterations that result in a stroke in a patient with SCD diverge from those in other stroke patients, necessitating distinct treatment approaches (particularly for IS). In SCD cases, the primary objective is to reduce hemoglobin S levels, a goal achievable through methods such as exchange transfusion or, albeit challenging to execute rapidly, simple blood transfusion [85,90,99,100].…”

Section: Stroke Management In Patients With Scdmentioning

confidence: 99%

Overview of the Association Between the Pathophysiology, Types, and Management of Sickle Cell Disease and Stroke

Hakami,

Alhazmi,

Busayli

et al. 2023

Cureus

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Sickle cell disease (SCD) is a genetic blood disorder that affects hemoglobin and increases stroke risk, particularly in childhood. This review examines the pathophysiological association between SCD and stroke, the classification of stroke types, risk factors, diagnosis, management, prevention, and prognosis. A comprehensive literature search was conducted via PubMed, Scopus, and Cochrane databases. Relevant studies on SCD and stroke pathophysiology, classification, epidemiology, diagnosis, treatment, and prevention were identified.Sickle cell disease causes red blood cells to become rigid and sickle-shaped, obstructing blood vessels. Recurrent sickling alters cerebral blood flow and damages vessel walls, often leading to ischemic or hemorrhagic strokes (HS). These occur most frequently in childhood, with ischemic strokes (IS) being more common. Key risk factors include a prior transient ischemic attack (TIA), low hemoglobin, and a high leukocyte count. Neuroimaging is essential for diagnosis and determining stroke type. Primary prevention centers on blood transfusions and hydroxyurea for those at high risk. Acute treatment involves promptly restoring blood flow and managing complications. However, significant knowledge gaps remain regarding stroke mechanisms, optimizing screening protocols, and improving long-term outcomes. This review synthesizes current evidence on SCD and stroke to highlight opportunities for further research and standardizing care protocols across institutions. Ultimately, a holistic perspective is critical for mitigating the high risk of debilitating strokes in this vulnerable patient population.

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Section: Stroke Management In Patients With Scdmentioning

confidence: 99%

Overview of the Association Between the Pathophysiology, Types, and Management of Sickle Cell Disease and Stroke

Hakami,

Alhazmi,

Busayli

et al. 2023

Cureus

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“…Children with SCA are at a heightened risk of experiencing overt and silent (asymptomatic) strokes 4 . Overt strokes result in observable neurological deficits, while silent strokes, often detected through neuroimaging studies, can lead to subtle cognitive impairments 5 . These neurological events can profoundly impact a child’s development and quality of life 5 .…”

Section: Introductionmentioning

confidence: 99%

Hydroxyurea for secondary stroke prevention in children with sickle cell anaemia: a systematic review of clinical evidence and outcomes

Aderinto,

Olatunji,

Kokori

et al. 2024

Annals of Medicine &Amp; Surgery

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Background: Stroke remains one of the leading complications of Sickle cell anaemia (SCA) in children. Traditionally, SCA treatment focused on symptom relief. However, the high incidence of strokes in children has prompted a reevaluation of treatment, particularly hydroxyurea, for secondary stroke prevention. This study assesses hydroxyurea’s effectiveness and safety in preventing secondary strokes in pediatric SCA patients. Methods: This systematic review followed a predefined protocol registered with PROSPERO. Comprehensive searches were conducted across PubMed, Embase, Scopus, MEDLINE, Google Scholar, and the Cochrane Library up to August 2023. Studies were included involving pediatric SCA patients at risk of secondary stroke, assessing hydroxyurea as the primary intervention. Results: A total of 6 studies meeting inclusion criteria were included. The effectiveness of hydroxyurea in preventing secondary strokes, with variable responses reported across studies. Adverse effects, including mild neutropenia, are associated with hydroxyurea treatment but with variability in reported toxicity levels. Conclusion: Hydroxyurea holds promise in preventing recurrent strokes in children with SCA, though its efficacy and safety profiles vary among individuals. Optimal dosages and treatment durations require further investigation, necessitating vigilant monitoring of haematological parameters. Future research should refine dosing strategies, consider individual patient characteristics, assess long-term effects, and explore ancillary benefits beyond stroke prevention.

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“…Extensive research and clinical experience spanning three decades have established hydroxyurea as a great therapeutic agent for inducing alpha-2 gamma-2 hemoglobin in individuals with sickle cell anemia (SCA). It has demonstrated efficacy across all age groups, from infants to adults, and offers additional benefits such as mild suppression of bone marrow function, increased red blood cell size, reduced cellular adhesion, improved blood flow, and the potential to enhance nitric oxide release in affected areas [12].…”

Section: Introductionmentioning

confidence: 99%

Review on Hydroxyurea Usage in Young Children with Sickle Cell Disease: Examining Hemoglobin Induction, Potential Benefits, Responses, Safety, and Effectiveness

Mkwambe,

Deng,

Zhao

2024

IJCM

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Sickle cell disease (SCD) is a prevalent condition, particularly in the countries of sub-Saharan Africa, where the presence of specific genes associated with Malaria contributes to its high prevalence. Patients with sickle cell disease frequently experience painful episodes necessitating hospitalization, and their hemoglobin levels are typically lower than those of the general population. There are different treatment options available to manage complications, such as transfusing blood, hydroxyurea, and strong anti-pains. However, with all these treatments, patients still commonly experience pain crises and suffer from organ damage. Hydroxyurea, the sole approved medication for sickle cell anemia in developed and developing countries, is widely used in children despite being primarily indicated for adults. Multiple studies have demonstrated the efficacy of hydroxyurea in inducing HbF production in young children with SCD. Elevated HbF levels have been associated with improved clinical outcomes, including a reduction in vaso-occlusive crises, acute chest syndrome, and the need for blood transfusions. Furthermore, increased HbF levels have been shown to ameliorate disease-related organ damage, such as pulmonary hypertension and sickle cell retinopathy. The response to hydroxyurea treatment in young children with SCD is variable. Some patients achieve substantial increases in HbF levels and experience significant clinical benefits, while others may have a more modest response. Factors influencing the response include baseline HbF levels, genetic modifiers, treatment adherence, and dose optimization. Safety is a crucial consideration when using hydroxyurea in young children. Studies have shown that hydroxyurea is generally well-tolerated, with the most common adverse effects being myelosuppression, gastrointestinal symptoms, and dermatological manifestations. However,

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Blood Transfusion Vs. Hydroxyurea for Stroke Prevention in Children With Sickle Cell Anemia: A Systematic Review and Meta-Analysis

Cited by 3 publications

References 47 publications

Overview of the Association Between the Pathophysiology, Types, and Management of Sickle Cell Disease and Stroke

Overview of the Association Between the Pathophysiology, Types, and Management of Sickle Cell Disease and Stroke

Hydroxyurea for secondary stroke prevention in children with sickle cell anaemia: a systematic review of clinical evidence and outcomes

Review on Hydroxyurea Usage in Young Children with Sickle Cell Disease: Examining Hemoglobin Induction, Potential Benefits, Responses, Safety, and Effectiveness

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