Prevention of a First Stroke by Transfusions in Children with Sickle Cell Anemia and Abnormal Results on Transcranial Doppler Ultrasonography

Adams, Robert J.; McKie, Virgil C.; Hsu, Lewis; Files, Beatrice; Vichinsky, Elliott; Pegelow, Charles; Abboud, Miguel; Gallagher, Dianne; Kutlar, Abdullah; Nichols, Fenwick T.; Bonds, Duane R.; Brambilla, Donald; Woods, Gerald; Olivieri, Nancy; Driscoll, Catherine; Miller, Scott; Wang, Winfred; Hurlett, Anne; Scher, Charles; Berman, Brian; Carl, Elizabeth; Jones, Anne M.; Roach, E. Steve; Wright, Elizabeth; Zimmerman, Robert A.; Waclawiw, Myron

doi:10.1056/nejm199807023390102

Cited by 1,719 publications

(1,532 citation statements)

References 22 publications

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“…A further limitation of NO bioavailability from either heightened destruction or diminished production may predispose to vaso-occlusion. The finding of impaired response of conduit vessels to NO may be particularly important in the carotid circulation-contributing to the reversible cerebral vasculopathy and propensity for stroke observed in children with HbSS [22]. Consistent with this idea is the finding that continued NO production is important for maintaining cerebral blood flow during experimental stroke in the presence of sickle erythrocytes [23].…”

Section: Discussionmentioning

confidence: 92%

Sickle cell anemia is associated with reduced nitric oxide bioactivity in peripheral conduit and resistance vessels

et al. 2003

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The purpose of the study was to examine endothelium-dependent and -independent vasodilation of conduit and resistance vessels in sickle cell anemia (HbSS). We measured the effects of intra-arterial infusion of methacholine, sodium nitroprusside, and N G -monomethyl-L-arginine (L-NMMA) on forearm blood flow using venous occlusion plethysmography in eight patients with HbSS and 11 HbAA controls. We assessed vasodilation of the conduit brachial artery using ultrasound in 17 patients with HbSS and 41 nonanemic controls. Forearm blood flow response to methacholine was similar in HbSS and HbAA, while the response to sodium nitroprusside was greater in those with HbSS. Nitric oxide synthase inhibition with L-NMMA attenuated methacholine-induced forearm blood flow to a lesser extent in HbSS compared to HbAA, suggesting diminished nitric oxide (NO) contribution to endothelium-dependent vasodilation. Flow-mediated and nitroglycerin-induced dilation were impaired in HbSS compared to controls and were not affected by the antioxidant vitamin C or the precursor substrate for NO synthesis L-arginine. NO bioactivity is reduced but differs in peripheral conduit and resistance vessels in HbSS. Resistance vessels have preserved response to exogenous NO donors but have diminished contribution of NO to endothelium-dependent vasodilation. Conduit vessels demonstrate impaired vasodilation to endogenous and exogenous NO. Am.

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Section: Discussionmentioning

confidence: 92%

Sickle cell anemia is associated with reduced nitric oxide bioactivity in peripheral conduit and resistance vessels

et al. 2003

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“…The pivotal Stroke Prevention Trial in Sickle Cell Anemia (STOP) trial utilized this strategy for primary stroke prevention and demonstrated that children with elevated TCD measurements (!200 cm/sec) receiving regular blood transfusion therapy (defined as blood transfusions every 3 to 6 weeks with a maximum hemoglobin S target of 30%) had a 92% relative risk reduction in the rate of overt strokes [63]. The STOP II study reported that once started on regular blood transfusion therapy for an elevated TCD measurement, stopping the transfusion would frequently result in either an overt stroke or an increase in the TCD velocity to levels associated with a high rate of stroke [64].…”

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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“…African-Americans constitute 47% of the population in St. Louis City and 14% in St. Louis County [3] but only about 5% of the blood donor population in the metropolitan area is African-American. Finding ways to increase the number of minority donors in the United States is important, particularly for children with sickle cell disease (SCD) requiring monthly blood transfusion therapy for primary and secondary prevention of strokes [4,5].…”

Section: Introductionmentioning

confidence: 99%

Barriers and motivators to blood and cord blood donations in young African-American women

Grossman

Watkins

Fleming³

et al. 2005

Am. J. Hematol.

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The primary aim of this study was to assess potential barriers and motivators to blood and cord blood donation among African-American women. A telephone survey of AfricanAmerican women, ages 18-30 years, in the St. Louis metropolitan area was performed. The survey was administered by trained telemarketing personnel using a ComputerAssisted Direct Interview (CADI) system. One hundred sixty-two women were surveyed. Common barriers to blood donation were inconvenience of donor sites (19%), fear of needles (16%), and too much time required to donate (15%). Potential motivators were increasing awareness of need for blood (43%), increasing the number of convenient donor locations (19%), and encouragement by spiritual leaders to have blood drives at their church (17%). Lack of awareness was the only identified barrier to cord blood donation. Most women surveyed (88%) indicated that they definitely or probably would donate cord blood. Strategies to increase the proportion of African-American blood and cord blood donations may include educating potential donors about the process and benefits of donation to particular patient populations and engaging church leadership in supporting blood and cord blood donations. Am.

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Prevention of a First Stroke by Transfusions in Children with Sickle Cell Anemia and Abnormal Results on Transcranial Doppler Ultrasonography

Abstract: Transfusion greatly reduces the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcranial Doppler ultrasonography.

Cited by 1,719 publications

References 22 publications

Sickle cell anemia is associated with reduced nitric oxide bioactivity in peripheral conduit and resistance vessels

Sickle cell anemia is associated with reduced nitric oxide bioactivity in peripheral conduit and resistance vessels

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

Barriers and motivators to blood and cord blood donations in young African-American women

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