Journal ArticleDOI
Effect of Hydroxyurea on the Frequency of Painful Crises in Sickle Cell Anemia
Samuel Charache,Michael L. Terrin,Richard D. Moore,George J. Dover,Franca B. Barton,Susan V. Eckert,Robert P. McMahon,Duane Bonds +7 more
TLDR
Hydroxyurea therapy can ameliorate the clinical course of sickle cell anemia in some adults with three or more painful crises per year and Maximal tolerated doses of hydroxyurea may not be necessary to achieve a therapeutic effect.Abstract:
Background In a previous open-label study of hydroxyurea therapy, the synthesis of fetal hemoglobin increased in most patients with sickle cell anemia, with only mild myelotoxicity. By inhibiting sickling, increased levels of fetal hemoglobin might decrease the frequency of painful crises. Methods In a double-blind, randomized clinical trial, we tested the efficacy of hydroxyurea in reducing the frequency of painful crises in adults with a history of three or more such crises per year. The trial was stopped after a mean follow-up of 21 months. Results Among 148 men and 151 women studied at 21 clinics, the 152 patients assigned to hydroxyurea treatment had lower annual rates of crises than the 147 patients given placebo (median, 2.5 vs. 4.5 crises per year, P<0.001). The median times to the first crisis (3.0 vs. 1.5 months, P = 0.01) and the second crisis (8.8 vs. 4.6 months, P<0.001) were longer with hydroxyurea treatment. Fewer patients assigned to hydroxyurea had chest syndrome (25 vs. 51, P<0.001), and...read more
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Journal ArticleDOI
Sickle cell disease
TL;DR: New strategies for specific therapy, including expanded use of chronic transfusions, bone marrow transplantation, and hydroxyurea, now offer hope for prevention of many or all of the hemolytic and vaso-occlusive manifestations of sickle cell disease.
Journal ArticleDOI
Prevention of a First Stroke by Transfusions in Children with Sickle Cell Anemia and Abnormal Results on Transcranial Doppler Ultrasonography
Robert J. Adams,Virgil McKie,Lewis L. Hsu,B Files,Elliott Vichinsky,Charles H. Pegelow,Miguel R. Abboud,Dianne Gallagher,Abdullah Kutlar,Fenwick T. Nichols,Duane Bonds,Donald Brambilla +11 more
TL;DR: Transfusion greatly reduces the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcranial Doppler ultrasonography.
Journal ArticleDOI
Cerebrovascular Accidents in Sickle Cell Disease: Rates and Risk Factors
Kwaku Ohene-Frempong,Steven J. Weiner,Lynn A. Sleeper,Scott T. Miller,Stephen H. Embury,John W. Moohr,Doris L. Wethers,Charles H. Pegelow,Frances M. Gill +8 more
TL;DR: The highest rates of prevalence of CVA and incidence were in sickle cell anemia (SS) patients, but CVA occurred in all common genotypes, and the incidence of infarctive CVA was lowest in SS patients 20 to 29 years of age and higher in children and older patients.
Journal ArticleDOI
Primary Prevention of Ischemic Stroke A Guideline From the American Heart Association/American Stroke Association Stroke Council: Cosponsored by the Atherosclerotic Peripheral Vascular Disease Interdisciplinary Working Group; Cardiovascular Nursing Council; Clinical Cardiology Council; Nutrition, Physical Activity, and Metabolism Council; and the Quality of Care and Outcomes Research Interdisciplinary Working Group: The American Academy of Neurology affirms the value of this guideline.
Larry B. Goldstein,Robert J. Adams,Mark J. Alberts,Lawrence J. Appel,Lawrence M. Brass,Cheryl Bushnell,Antonio Culebras,Thomas J. DeGraba,Philip B. Gorelick,John R. Guyton,Robert G. Hart,George Howard,Margaret Kelly-Hayes,J.V. (Ian) Nixon,Ralph L. Sacco +14 more
TL;DR: In this paper, the authors provide an overview of the evidence on various established and potential stroke risk factors and provide recommendations for the reduction of stroke risk, and when appropriate, formulate recommendations based on standard American Heart Association criteria.
Journal ArticleDOI
Management of Sickle Cell Disease: Summary of the 2014 Evidence-Based Report by Expert Panel Members
Barbara P. Yawn,George R. Buchanan,Araba Afenyi-Annan,Samir K. Ballas,Kathryn L. Hassell,Andra H. James,Lanetta B. Jordan,Sophie Lanzkron,Richard Lottenberg,William J. Savage,Paula Tanabe,Russell E. Ware,M. Hassan Murad,Jonathan C. Goldsmith,Jonathan C. Goldsmith,Eduardo Ortiz,Robinson Fulwood,Ann Horton,Joylene John-Sowah +18 more
TL;DR: Hydxyurea and transfusion therapy are strongly recommended for many individuals with SCD and many other recommendations are based on quality of evidence that is less than high due to the paucity of clinical trials regarding screening, management, and monitoring for individuals withSCD.
References
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Book
Applied Regression Analysis
Norman R. Draper,Harry Smith +1 more
TL;DR: In this article, the Straight Line Case is used to fit a straight line by least squares, and the Durbin-Watson Test is used for checking the straight line fit.
Journal ArticleDOI
An Introduction to Probability Theory and Its Applications.
Journal ArticleDOI
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Journal ArticleDOI
Pain in sickle cell disease. Rates and risk factors.
Orah S. Platt,Bruce D. Thorington,Donald Brambilla,Paul F. Milner,Wendell F. Rosse,Elliott Vichinsky,Thomas R. Kinney +6 more
TL;DR: The "pain rate" is a measure of clinical severity and correlates with early death in patients with sickle cell anemia over the age of 20, and even when the fetal hemoglobin level is low, one can predict that small increments in the level may have an ameliorating effect on the pain rate and may ultimately improve survival.
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