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JAMA | 2014

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 Jordan; Sophie Lanzkron; Richard Lottenberg; William J. Savage; Paula Tanabe; Russell E. Ware; M. Hassan Murad; Jonathan C. Goldsmith; Eduardo Ortiz; Robinson Fulwood; Ann Horton; Joylene John-Sowah

IMPORTANCE Sickle cell disease (SCD) is a life-threatening genetic disorder affecting nearly 100,000 individuals in the United States and is associated with many acute and chronic complications requiring immediate medical attention. Two disease-modifying therapies, hydroxyurea and long-term blood transfusions, are available but underused. OBJECTIVE To support and expand the number of health professionals able and willing to provide care for persons with SCD. EVIDENCE REVIEW Databases of MEDLINE (including in-process and other nonindexed citations), EMBASE, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, CINAHL, TOXLINE, and Scopus were searched using prespecified search terms and keywords to identify randomized clinical trials, nonrandomized intervention studies, and observational studies. Literature searches of English-language publications from 1980 with updates through April 1, 2014, addressed key questions developed by the expert panel members and methodologists. FINDINGS Strong recommendations for preventive services include daily oral prophylactic penicillin up to the age of 5 years, annual transcranial Doppler examinations from the ages of 2 to 16 years in those with sickle cell anemia, and long-term transfusion therapy to prevent stroke in those children with abnormal transcranial Doppler velocity (≥200 cm/s). Strong recommendations addressing acute complications include rapid initiation of opioids for treatment of severe pain associated with a vasoocclusive crisis, and use of incentive spirometry in patients hospitalized for a vasoocclusive crisis. Strong recommendations for chronic complications include use of analgesics and physical therapy for treatment of avascular necrosis, and use of angiotensin-converting enzyme inhibitor therapy for microalbuminuria in adults with SCD. Strong recommendations for children and adults with proliferative sickle cell retinopathy include referral to expert specialists for consideration of laser photocoagulation and for echocardiography to evaluate signs of pulmonary hypertension. Hydroxyurea therapy is strongly recommended for adults with 3 or more severe vasoocclusive crises during any 12-month period, with SCD pain or chronic anemia interfering with daily activities, or with severe or recurrent episodes of acute chest syndrome. A recommendation of moderate strength suggests offering treatment with hydroxyurea without regard to the presence of symptoms for infants, children, and adolescents. In persons with sickle cell anemia, preoperative transfusion therapy to increase hemoglobin levels to 10 g/dL is strongly recommended with a moderate strength recommendation to maintain sickle hemoglobin levels of less than 30% prior to the next transfusion during long-term transfusion therapy. A strong recommendation to assess iron overload is accompanied by a moderate strength recommendation to begin iron chelation therapy when indicated. CONCLUSIONS AND RELEVANCE Hydroxyurea and transfusion therapy are strongly recommended for many individuals with SCD. 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 with SCD.


American Journal of Public Health | 1991

Serum total cholesterol: HDL cholesterol ratios in US white and black adults by selected demographic and socioeconomic variables (HANES II).

S Linn; Robinson Fulwood; M Carroll; J G Brook; C Johnson; W Kalsbeek; Basil M. Rifkind

BACKGROUND Framingham Study findings suggest that total cholesterol (TC):High density lipoprotein cholesterol (HDL-C) ratio is a useful summary of the joint contribution of TC and HDL-C to coronary heart disease (CHD) risk. Information on the distribution of TC:HDL-C in the US population is limited to selected populations and the relationship of the ratio distribution and its correlates has received little attention. METHOD TC/HDL-C ratios were examined in a representative sample of the United States adult population ages 20 to 74 years, between February 1976 and February 1980 during NHANES II, using stratification and multivariate regression analyses. RESULTS Age-adjusted mean ratios were higher in men compared with women and were higher in Whites compared with Blacks. White men had the highest TC/HDL-C mean ratios. These relationships remained after stratification by age, education, body mass index, alcohol use, cigarette smoking, and physical activity. Using multivariate analyses, the ratios were positively related to BMI, age, and smoking; and negatively related to female sex, alcohol use, being Black, and physical activity. CONCLUSIONS Using a ratio reference point of greater than or equal to 4.5 from the Framingham study, at least an estimated 44 million persons ages 25 to 74 years in the US were found to be at higher risk of developing coronary heart disease.


Archive | 1992

The National Asthma Education Program

Suzanne S. Hurd; Robinson Fulwood

Asthma is a major health problem in the United States and throughout the world. In the U.S. it affects approximately 10 million people, 3 million of whom are under the age of 18. It accounts for 458,000 hospitalizations and more than 6.5 million physician visits annually. Approximately 2 million people with asthma suffer from limitations in activity due to their disease. Direct and health care expenditures are high, estimated to exceed


JAMA | 1989

The Prevalence of High Blood Cholesterol Levels Among Adults in the United States

Christopher T. Sempos; Robinson Fulwood; Carol M. Haines; Margaret D. Carroll; Robert F. Anda; David F. Williamson; Patrick L. Remington; James I. Cleeman

3.0 billion per year in the U.S. alone.


American Journal of Epidemiology | 1989

HIGH DENSITY LIPOPROTEIN CHOLESTEROL LEVELS AMONG US ADULTS BY SELECTED DEMOGRAPHIC AND SOCIOECONOMIC VARIABLES THE SECOND NATIONAL HEALTH AND NUTRITION EXAMINATION SURVEY 1976–1980

Shai Linn; Robinson Fulwood; Basil M. Rifkind; Margaret D. Carroll; Richard Muesing; O. Dale Williams; Clifford L. Johnson


Preventing Chronic Disease | 2009

A promotora de salud model for addressing cardiovascular disease risk factors in the US-Mexico border region.

Hector Balcazar; Matilde Alvarado; Cantu F; Pedregon; Robinson Fulwood


Chest | 1999

The National Asthma Education and Prevention Program: Partnering With Local Asthma Coalitions To Implement the Guidelines

Diana K. Schmidt; Robinson Fulwood; Claude Lenfant


Current Hypertension Reports | 2006

Role of community programs in controlling blood pressure

Robinson Fulwood; Jeanette Guyton-Krishnan; Madeleine Wallace; Ellen Sommer


Vital and Health Statistics | 1980

Serum cholesterol levels of persons 4-74 years of age by socioeconomic characteristics, United States, 1971-74

Robinson Fulwood; Sidney Abraham; Clifford L. Johnson


Preventing Chronic Disease | 2008

NHLBI Step-by-Step Approach to Adapting Cardiovascular Training and Education Curricula for Diverse Audiences

Madeleine Wallace; Robinson Fulwood; Matilde Alvarado

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Margaret D. Carroll

Centers for Disease Control and Prevention

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Ann Horton

American Institutes for Research

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Araba Afenyi-Annan

University of North Carolina at Chapel Hill

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Basil M. Rifkind

National Institutes of Health

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Christopher T. Sempos

National Institutes of Health

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Clifford L. Johnson

National Center for Health Statistics

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David F. Williamson

Centers for Disease Control and Prevention

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Eduardo Ortiz

National Institutes of Health

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