Hydroxyurea (hydroxycarbamide) for sickle cell disease

Rankine-Mullings, Angela; Nevitt, Sarah J

doi:10.1002/14651858.cd002202.pub3

Cited by 19 publications

(15 citation statements)

References 183 publications

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“… 31 Although SCD pregnancy has been recognized as high-risk for over 50 years, disease-specific studies of SCD pregnancy pathophysiology and of interventions to affect pregnancy outcomes are limited or inconclusive. 25 , 32 , 33 This study relies on billing codes because no contemporary registry yet contains the obstetric data needed to analyze a nationally representative sample of pregnant people with SCD. Yet, the evidence that such a study might be transformative is clear, given the field-defining output from the Cooperative Study of Sickle Cell Disease (conducted 1977 to 1998).…”

Section: Discussionmentioning

confidence: 99%

“…In high- and low-income settings, more people with SCD are surviving into their reproductive years . Although SCD pregnancy has been recognized as high-risk for over 50 years, disease-specific studies of SCD pregnancy pathophysiology and of interventions to affect pregnancy outcomes are limited or inconclusive . This study relies on billing codes because no contemporary registry yet contains the obstetric data needed to analyze a nationally representative sample of pregnant people with SCD.…”

Section: Discussionmentioning

confidence: 99%

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Severe Maternal Morbidity and Mortality in Sickle Cell Disease in the National Inpatient Sample, 2012-2018

et al. 2023

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ImportancePregnancy outcomes are historically poor among people with sickle cell disease (SCD) in the US, most of whom have Black race. Whether outcomes have improved is unknown.ObjectiveTo tabulate adverse pregnancy outcomes among patients with SCD, comparing outcomes of deliveries among Black people with SCD with those of Black people without SCD and a control non-Black population, and to measure the association of racial disparities with adverse outcomes in SCD pregnancies.Design, Setting, and ParticipantsThis cross-sectional study was a secondary analysis involving data from National Inpatient Sample, a nationally representative sample of 20% of acute hospital admissions in the US, between 2012 and 2018. The data set included all admissions with codes for delivery of a pregnancy among people aged 11 to 55 years. Data were analyzed from September 2021 to August 2022.ExposuresSCD, racial disparities.Main Outcomes and MeasuresSevere maternal morbidity (SMM) as measured by the US Centers for Disease Control and Prevention’s index alongside other outcomes; multiple logistic regression was used to compare the odds for adverse pregnancy outcomes.ResultsThe sample included 5 401 899 deliveries, including 3901 deliveries among people with SCD and 742 164 deliveries among people with Black race. Compared with the non-Black control group, patients with SCD and Black patients were younger (mean [SD] age: SCD, 27.2 [5.9] years; Black, 27.1 [6.1] years vs 28.7 [5.9] years) and more likely to have public insurance (SCD, 2609 deliveries [67.3%]; Black, 496 828 deliveries [65.4%] vs 1 880 198 deliveries [40.8%]). The maternal mortality rate in deliveries among people with SCD was 26 times greater than in the non-Black control group and more than 10 times greater than among Black pregnant people without SCD (Per 10 000 deliveries: SCD 13.3; 95% CI, 5.7-31.2; Black race, 1.2; 95% CI, 1.0-1.5; non-Black control 0.5; 95% CI, 0.5-0.6). Compared with the control group, SCD deliveries had higher odds of SMM (adjusted odds ratio [aOR], 7.22; 95% CI, 6.25-8.34; P &lt; .001), especially cerebrovascular events (aOR, 22.00; 95% CI, 15.25-31.72; P &lt; .001) and thromboembolism (aOR, 17.34; 95% CI, 11.55-26.03; P &lt; .001). Racial disparities explained a median (IQR) 28.9% (21.2%-33.1%) of the increased risk in deliveries to people with SCD and between 40% and 50% of the increased risk for acute kidney failure (excess risk [ER], 56.9%; 95% CI, 54.3%-59.3%), intrauterine fetal demise (ER, 47.8%; 95% CI, 46.6%-49.1%), and eclampsia (ER, 42.1%; 95% CI, 37.9%-46.1%).Conclusions and RelevanceIn this large cross-sectional study of pregnancy outcomes in people with SCD, the risk for SMM was higher compared with deliveries among people without SCD, especially for thrombotic events, organ failure, and death. Racial disparities were associated with adverse outcomes. Our findings compel scientific, clinical, and political effort to improve outcomes for pregnant people with SCD.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Severe Maternal Morbidity and Mortality in Sickle Cell Disease in the National Inpatient Sample, 2012-2018

et al. 2023

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“…The requirement for ongoing transfusions for the primary prevention of strokes makes them unfeasible in countries with limited resources. Notable updates by Jamaican researchers to the Cochrane database emphasized the efficacy of hydroxyurea in primary stroke prevention in at-risk SCD patients by maintaining TCD velocities [ 11 , 12 ].…”

Section: Reviewmentioning

confidence: 99%

Transcranial Doppler Ultrasonography-Related Research in the Caribbean Region

Mandalaneni¹,

Venkatapathappa²,

Koshy³

et al. 2023

Cureus

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Transcranial Doppler (TCD) ultrasonography is a non-invasive ultrasound technique that uses highfrequency sound waves to measure blood flow velocities in the cerebral vasculature. This review analyzes TCD research in the Caribbean region using a bibliometric analysis of 29 articles from PubMed. The articles were analyzed using Microsoft Excel 2016 and the VOSviewer software (Van Eck and Waltman, Leiden University, Centre for Science and Technology Studies (CWTS), www.vosviewer.com) and characterized various aspects of TCD research, including countries, research themes, authorship, journals, affiliations, and keywords. The majority of the 29 publications came from Cuba (38%), followed by the French West Indies (22%) and Jamaica (20%). Most TCD research focused on sickle cell disease (SCD), accounting for 45% of the studies, followed by 21% of articles on vasospasm and subarachnoid hemorrhage. The use of TCD in brain death and neuro-intensive care was also explored, constituting 17% of the studies. Alternative TCDmonitored treatment options for SCD, such as stem cell transplantation and hydroxyurea, were also frequently investigated. The most productive institutions were Hospital Clínico-Quirúrgico Hermanos Ameijeiras in Havana, Cuba, the Sickle Cell Unit at the University of West Indies (UWI) Mona in Jamaica, the Medical-Surgical Research Center (CIMEQ) in Havana, Cuba, and the SCD Reference Center in Guadeloupe and Martinique in the French West Indies. TCD has been identified as a cost-effective tool for real-time monitoring of cerebral blood flow in many clinical settings, including stroke and SCD, which are prevalent in the Caribbean. Although there is an increase in the trend of using TCD for neuromonitoring in the Caribbean, gaps still exist. Capacity-building initiatives, such as training programs for healthcare providers and the development of local TCD research networks, can improve access to TCD in resource-constrained settings to treat and neuromonitor patients cost-effectively.

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“…Hydroxyurea appears to be safe for long-term use, but study of its secondary effects, such as infertility, is ongoing. [69][70][71] In the randomized, controlled Pediatric Hydroxyurea Phase 3 Clinical Trial (BABY HUG) trial, starting hydroxyurea in early childhood was associated with a slower rise in TCD, compared to placebo. 72,73 An ongoing clinical trial is evaluating the up-front use of hydroxyurea for primary stroke prophylaxis in regions without access to chronic transfusion.…”

Section: Transfusion Complications and Iron Overloadmentioning

confidence: 99%

Managing the Cerebrovascular Complications of Sickle Cell Disease: Current Perspectives

Light¹,

Boucher²,

Baskin‐Miller³

et al. 2023

JBM

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The importance of protecting brain function for people with sickle cell disease (SCD) cannot be overstated. SCD is associated with multiple cerebrovascular complications that threaten neurocognitive function and life. Without screening and preventive management, 11% of children at 24% of adults with SCD have ischemic or hemorrhagic strokes. Stroke screening in children with SCD is well-established using transcranial Doppler ultrasound (TCD). TCD velocities above 200 cm/s significantly increase the risk of stroke, which can be prevented using chronic red blood cell (RBC) transfusion. RBC transfusion is also the cornerstone of acute stroke management and secondary stroke prevention. Chronic transfusion requires long-term management of complications like iron overload. Hydroxyurea can replace chronic transfusions for primary stroke prevention in a select group of patients or in populations where chronic transfusions are not feasible. Silent cerebral infarction (SCI) is even more common than stroke, affecting 39% of children and more than 50% of adults with SCD; management of SCI is individualized and includes careful neurocognitive evaluation. Hematopoietic stem cell transplant prevents cerebrovascular complications, despite the short- and long-term risks. Newer disease-modifying agents like voxelotor and crizanlizumab, as well as gene therapy, may treat cerebrovascular complications, but these approaches are investigational.

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Hydroxyurea (hydroxycarbamide) for sickle cell disease

Cited by 19 publications

References 183 publications

Severe Maternal Morbidity and Mortality in Sickle Cell Disease in the National Inpatient Sample, 2012-2018

Severe Maternal Morbidity and Mortality in Sickle Cell Disease in the National Inpatient Sample, 2012-2018

Transcranial Doppler Ultrasonography-Related Research in the Caribbean Region

Managing the Cerebrovascular Complications of Sickle Cell Disease: Current Perspectives

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