The Challenge of Creating an Evidence-Based Guideline for Sickle Cell Disease

DeBaun, Michael R.

doi:10.1001/jama.2014.11103

Cited by 14 publications

(14 citation statements)

References 17 publications

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“…178,179 In light of the limited evidence, recent recommendations did not support neuroimaging screening in asymptomatic children, adolescents, and adults with sickle cell disease.…”

Section: Population Screening For Silent Cerebrovascular Diseasementioning

confidence: 99%

Prevention of Stroke in Patients With Silent Cerebrovascular Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association/American Stroke Association

Smith¹,

Saposnik²,

Biessels³

et al. 2017

Stroke

294

296

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Abstract-Two decades of epidemiological research shows that silent cerebrovascular disease is common and is associated with future risk for stroke and dementia. It is the most common incidental finding on brain scans. To summarize evidence on the diagnosis and management of silent cerebrovascular disease to prevent stroke, the Stroke Council of the American Heart Association convened a writing committee to evaluate existing evidence, to discuss clinical considerations, and to offer suggestions for future research on stroke prevention in patients with 3 cardinal manifestations of silent cerebrovascular disease: silent brain infarcts, magnetic resonance imaging white matter hyperintensities of presumed vascular origin, and cerebral microbleeds. The writing committee found strong evidence that silent cerebrovascular disease is a common problem of aging and that silent brain infarcts and white matter hyperintensities are associated with future symptomatic stroke risk independently of other vascular risk factors. In patients with cerebral microbleeds, there was evidence of a modestly increased risk of symptomatic intracranial hemorrhage in patients treated with thrombolysis for acute ischemic stroke but little prospective evidence on the risk of symptomatic hemorrhage in patients on anticoagulation. There were no randomized controlled trials targeted specifically to participants with silent cerebrovascular disease to prevent stroke. Primary stroke prevention is indicated in patients with silent brain infarcts, white matter hyperintensities, or microbleeds. Adoption of standard terms and definitions for silent cerebrovascular disease, as provided by prior American Heart Association/American Stroke Association statements and by a consensus group, may facilitate diagnosis and communication of findings from radiologists to clinicians. (Stroke. 2017;48:e44-e71.

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“…178,179 In light of the limited evidence, recent recommendations did not support neuroimaging screening in asymptomatic children, adolescents, and adults with sickle cell disease.…”

Section: Population Screening For Silent Cerebrovascular Diseasementioning

confidence: 99%

Prevention of Stroke in Patients With Silent Cerebrovascular Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association/American Stroke Association

Smith¹,

Saposnik²,

Biessels³

et al. 2017

Stroke

294

296

View full text Add to dashboard Cite

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“…These improvements are anticipated to improve quality of life and result in greater cost-effectiveness as compared with episodic, emergency department (ED)-based care. [15][16][17][18] Patients with SCD treated at SCD centers use ED and inpatient facilities less frequently, have decreased health care costs overall, 15,16,19 and are more likely to be prescribed hydroxyurea, 20 which has been demonstrated to improve psychosocial outcomes including healthrelated quality of life. 21 Comprehensive care of SCD requires that affected individuals have a continual bidirectional relationship with their medical team.…”

Section: Introductionmentioning

confidence: 99%

Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects

et al. 2020

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Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is a medically and socially complex, multisystem illness that affects individuals throughout the lifespan. Given improvements in care, most children with SCD survive into adulthood. However, access to adult sickle cell care is poor in many parts of the United States, resulting in increased acute care utilization, disjointed care delivery, and early mortality for patients. A dearth of nonmalignant hematology providers, the lack of a national SCD registry, and the absence of a centralized infrastructure to facilitate comparative quality assessment compounds these issues. As part of a workshop designed to train health care professionals in the skills necessary to establish clinical centers focused on the management of adults living with SCD, we defined an SCD center, elucidated required elements of a comprehensive adult SCD center, and discussed different models of care. There are also important economic impacts of these centers at an institutional and health system level. As more clinicians are trained in providing adult-focused SCD care, center designation will enhance the ability to undertake quality improvement and compare outcomes between SCD centers. Activities will include an assessment of the clinical effectiveness of expanded access to care, the implementation of SCD guidelines, and the efficacy of newly approved targeted medications. Details of this effort are provided.

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“…There is an ongoing debate over the rationale of screening for SCIs in patients with SCD [14,15]. While the Silent Cerebral Infarct Transfusion (SIT) randomized controlled trial showed that chronic red blood cell transfusions reduce the risk of recurrent infarction, this benefit was incomplete with some children in the transfusion therapy arm also developing infarct recurrence [16].…”

Section: Introductionmentioning

confidence: 99%

Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis

Houwing

Grohssteiner

Dremmen

et al. 2020

BMC Med

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Background and purpose Silent cerebral infarcts (SCIs) are the most common neurological complication in children and adults with sickle cell disease (SCD). In this systematic review, we provide an overview of studies that have detected SCIs in patients with SCD by cerebral magnetic resonance imaging (MRI). We focus on the frequency of SCIs, the risk factors involved in their development and their clinical consequences. Methods The databases of Embase, MEDLINE ALL via Ovid, Web of Science Core Collection, Cochrane Central Register of Trials via Wiley and Google Scholar were searched from inception to June 1, 2019. Results The search yielded 651 results of which 69 studies met the eligibility criteria. The prevalence of SCIs in patients with SCD ranges from 5.6 to 80.6% with most studies reported in the 20 to 50% range. The pooled prevalence of SCIs in HbSS and HbSβ0 SCD patients is 29.5%. SCIs occur more often in patients with the HbSS and HbSβ0 genotype in comparison with other SCD genotypes, as SCIs are found in 9.2% of HbSC and HbSβ+ patients. Control subjects showed a mean pooled prevalence of SCIs of 9.8%. Data from included studies showed a statistically significant association between increasing mean age of the study population and mean SCI prevalence. Thirty-three studies examined the risk factors for SCIs. The majority of the risk factors show no clear association with prevalence, since more or less equal numbers of studies give evidence for and against the causal association. Conclusions This systematic review and meta-analysis shows SCIs are common in patients with SCD. No clear risk factors for their development were identified. Larger, prospective and controlled clinical, neuropsychological and neuroimaging studies are needed to understand how SCD and SCIs affect cognition.

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The Challenge of Creating an Evidence-Based Guideline for Sickle Cell Disease

Cited by 14 publications

References 17 publications

Prevention of Stroke in Patients With Silent Cerebrovascular Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association/American Stroke Association

Prevention of Stroke in Patients With Silent Cerebrovascular Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association/American Stroke Association

Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects

Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis

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