The natural history of stroke in sickle cell disease

Powars, Darleen R.; Wilson, B. J.; Imbus, Charles; Pegelow, Charles H.; Allen, J. R. L.

doi:10.1016/0002-9343(78)90772-6

Cited by 554 publications

(334 citation statements)

References 25 publications

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“…Almost a quarter of individuals with SCA will experience an overt stroke by 45 years of age [61]. Powars et al established that among individuals with SCD and stroke, 50% have a second stroke within 2 years, and 66% within 9 years of the initial event [62].…”

Section: Stroke Prevention In Scamentioning

confidence: 99%

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

2015

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Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimistic that the next 40 years will be focused on meeting current challenges in SCD by addressing chronic complications of SCD to reduce mortality and improve quality of life in a growing population of adults with SCD in high-income countries, while simultaneously decreasing the disparity of medical care between high and low-income countries.

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Section: Stroke Prevention In Scamentioning

confidence: 99%

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

2015

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“…Most strokes in children with SCA (symptomatic or asymptomatic) are the result of infarction; intracranial hemorrhage becomes more common later in life. 4 Even with adequate chronic transfusion therapy or successful bone marrow transplantation, the child who has had a stroke is left with significant physical or neuropsychologic deficits. Because of the lifelong morbidity from primary stroke, considerable effort has been made to identify patients at risk for stroke and to intervene in a preventive manner.…”

Section: Introductionmentioning

confidence: 99%

Distinct HLA associations by stroke subtype in children with sickle cell anemia

Hoppe¹,

Klitz²,

Noble³

et al. 2003

Blood

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Children with sickle cell anemia (SCA) carry a 200-fold increased risk for cerebral infarction. Stroke can be the result of small-vessel (SV) or large-vessel (LV) disease. However, it is unknown whether these subtypes result from the same pathophysiologic processes. Complete HLA genotyping was performed on 231 eligible children previously enrolled in the Cooperative Study of Sickle Cell Disease (CSSCD). Cerebral infarction on magnetic resonance imaging (MRI) was documented in 71 patients, and 160 patients had negative findings on MRI. Based on MRI/magnetic resonance angiography (MRA) findings, infarct size, and location, 36 patients were classified as having LV stroke and 35 as having SV stroke. When comparing the total MRI؉ group with the MRI؊ group, HLA DPB1*0401 was associated with increased stroke risk (P ‫؍‬ .01), whereas DPB1*1701 (P ‫؍‬ .02) conferred protection from stroke. These DPB1 associations with stroke were attributed to the SV stroke group, in whom DPB1*0401 was associated with susceptibility (P ‫؍‬ .003) and DPB1*1701 with protection from stroke (P ‫؍‬ .06). In the LV stroke subgroup, HLA-A*0102 (P ‫؍‬ .02) and -A*2612 (P ‫؍‬ .007) conferred susceptibility, whereas -A*3301(P ‫؍‬ .04) protected from stroke. These results suggest that specific HLA alleles influence stroke risk and appear to contribute differently to SV and LV stroke subtypes. The distinct HLA associations with SV and LV stroke suggest that different pathologic processes may be involved in the development of stroke in children with SCA. If these results are confirmed in a larger study, HLA type may serve as a useful marker for the early identification of SCA patients at high risk for stroke. (Blood. 2003;

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“…Initial evidence implicating the need for secondary prevention of strokes was demonstrated by the seminal study by Powers et al showing that 50% of the individuals with SCD and overt stroke will have a second stroke within 2 years and 66% within 10 years. 20 In addition, a temporal clustering occurred, with 80% of the strokes being observed within 36 months of the first stroke. Subsequently, in a landmark single-arm intervention study, Sarnaik et al demonstrated that a transfusion program that consisted of keeping the maximum hemoglobin S levels Ͻ 25% and the baseline line hemoglobin between 10 and 12 g/dL was beneficial in preventing recurrent strokes.…”

Section: Secondary Prevention Of Overt Strokes Regular Blood Transfusmentioning

confidence: 99%

“…Regardless of whether patients receive blood transfusion therapy, all large case series in patients with strokes have demonstrated that the 2-to 3-year window after an initial stroke has the highest incidence rate for stroke recurrence. 20,22,23 In the largest hydroxyurea trial for secondary prevention of strokes, hydroxyurea was started well after the highest stroke recurrence risk period, with a mean interval of approximately 5 years after the initial stroke. Reliable comparison of incidence rates from different observational studies for secondary prevention of strokes cannot be done unless the time after the initial stroke is considered in the estimate of the recurrence incidence rate.…”

Section: Hydroxyurea Therapymentioning

confidence: 99%

Secondary Prevention of Overt Strokes in Sickle Cell Disease: Therapeutic Strategies and Efficacy

DeBaun

2011

Hematology

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Overt strokes, previously one of the most common neurological complications in sickle cell disease (SCD), have become far less frequent with routine transcranial Doppler (TCD) assessment followed by regular blood transfusion therapy. Nevertheless, children and adults with SCD continue to have overt strokes, and in the foreseeable future will continue to require secondary prevention of strokes. With the exception of the most recently completed "Stroke With Transfusions Changing to Hydroxyurea" Trial (SWiTCH; NCT00122980), randomized trials providing best evidence for long-term management of overt strokes in SCD is lacking. Instead of randomized clinical trials, a series of observational and single-arm studies have predominated. This review assesses the best available evidence for acute and chronic management of overt stroke and the efficacy of regular blood transfusion therapy, hydroxyurea therapy, and hematopoietic stem cell transplantation (HSCT), including matched sibling donor and unrelated HSCT.

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The natural history of stroke in sickle cell disease

Cited by 554 publications

References 25 publications

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

Distinct HLA associations by stroke subtype in children with sickle cell anemia

Secondary Prevention of Overt Strokes in Sickle Cell Disease: Therapeutic Strategies and Efficacy

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