Scholars@Duke
John J. Strouse

John J. Strouse

Associate Professor of Medicine
Medicine, Hematology

Selected Publications

Comparison of Measures of Pain Intensity During Sickle Cell Disease Vaso-Occlusive Episodes.

Journal Article J Pain · December 2024 We aimed to determine the minimal clinically important difference (MCID) in pain severity and agreement between the visual analog scale (VAS) and the verbal numeric rating scale (NRS) in people with sickle cell disease (SCD) experiencing an acute vaso-occl ... Full text Link to item Cite

Black Americans With Sickle Cell Disease (SCD) Demonstrate Accelerated Epigenetic Pace of Aging Compared to Black Americans Without SCD.

Journal Article J Gerontol A Biol Sci Med Sci · November 1, 2024 BACKGROUND: Sickle cell disease (SCD) is a chronic medical condition characterized by red blood cell sickling, vaso-occlusion, hemolytic anemia, and subsequently, end-organ damage and reduced survival. Because of this significant pathophysiology and early ... Full text Link to item Cite

Hematopoietic Cell Transplant compared with Standard Care in Adolescents and Young Adults with Sickle Cell Disease.

Journal Article Blood Adv · October 29, 2024 Disease-modifying therapies are standard of care (SOC) for sickle cell disease (SCD), but hematopoietic cell transplantation (HCT) has curative potential. We compared outcomes prospectively through 2-years after biologic assignment to a Donor or No Donor ( ... Full text Link to item Cite

Hydroxyurea to prevent brain injury in children with sickle cell disease (HU Prevent)-A randomized, placebo-controlled phase II feasibility/pilot study.

Journal Article Am J Hematol · October 2024 Central nervous system (CNS) injury is common in sickle cell disease (SCD) and occurs early in life. Hydroxyurea is safe and efficacious for treatment of SCD, but high-quality evidence from randomized trials to estimate its neuroprotective effect is scant. ... Full text Link to item Cite

COVID-19 mRNA vaccination responses in individuals with sickle cell disease: an ASH RC Sickle Cell Research Network Study.

Journal Article Blood Adv · September 10, 2024 Children and adults with sickle cell disease (SCD) have increases in morbidity and mortality with COVID-19 infections. The American Society of Hematology Research Collaborative Sickle Cell Disease Research Network performed a prospective COVID-19 vaccine s ... Full text Link to item Cite

Birth Prevalence of Sickle Cell Disease and County-Level Social Vulnerability - Sickle Cell Data Collection Program, 11 States, 2016-2020.

Journal Article MMWR Morb Mortal Wkly Rep · March 28, 2024 Sickle cell disease (SCD) remains a public health priority in the United States because of its association with complex health needs, reduced life expectancy, lifelong disabilities, and high cost of care. A cross-sectional analysis was conducted to calcula ... Full text Open Access Link to item Cite

How I treat sickle cell disease in pregnancy.

Journal Article Blood · February 29, 2024 Fifty years ago, people with sickle cell disease (SCD) were discouraged from becoming pregnant, but now, most should be supported if they choose to pursue a pregnancy. They and their providers, however, should be aware of the physiological changes of pregn ... Full text Link to item Cite

Deciphering and disrupting PIEZO1-TMEM16F interplay in hereditary xerocytosis.

Journal Article Blood · January 25, 2024 Cell-surface exposure of phosphatidylserine (PS) is essential for phagocytic clearance and blood clotting. Although a calcium-activated phospholipid scramblase (CaPLSase) has long been proposed to mediate PS exposure in red blood cells (RBCs), its identity ... Full text Link to item Cite

PIEZO1-TMEM16F coupling in red blood disorders

Conference BIOPHYSICAL JOURNAL · 2024 Cite

Development of a Calculator to Determine Individualized Opioid Doses for Treatment of Vaso-Occlusive Episodes for Sickle Cell Disease in the Emergency Department

Journal Article Joint Commission Journal on Quality and Patient Safety · January 1, 2024 Sickle cell disease (SCD) is a life-limiting multisystem disease primarily affecting individuals of African and Latinx descent. Its most common complication is painful vaso-occlusive episodes (VOEs), which is also the most common reason individuals with SC ... Full text Cite

A comparison of the effect of patient-specific versus weight-based protocols to treat vaso-occlusive episodes in the emergency department.

Journal Article Acad Emerg Med · December 2023 BACKGROUND: Vaso-occlusive crises (VOCs) cause debilitating pain and are a common cause of emergency department (ED) visits, for people with sickle cell disease (SCD). Strategies for achieving optimal pain control vary widely despite evidence-based guideli ... Full text Open Access Link to item Cite

Grndad and Disease Modifying Therapy (DMT): Shifts in Dmt Are Seen at the Adolescent/Young Adult Transition in Sickle Cell Disease in a Multi-Site Prospective Registry

Conference Blood · November 2, 2023 The ability to characterize the modern person living with SCD in the US has been limited by the lack of a well-curated longitudinal registry. The Globin Research Network for Data and Discovery ( GRNDaD) registry aims to ... Full text Cite

Experiences with Delayed Diagnosis for Older Adults with SCD: “Looking Back It All Makes Sense”

Conference Blood · November 2, 2023 BackgroundImprovements in care over the last 50 years have extended the median lifespan of people living with sickle cell disease (SCD) from 14 years to as long as 61 years in cohorts fr ... Full text Cite

Personalized Exercise Intervention for Older Adults with Sickle Cell Disease: The Sicklefit Study

Conference Blood · November 2, 2023 BackgroundOlder adults with sickle cell disease (SCD) (defined as age ≥ 40 years) experience both SCD and age-related conditions such as recurrent pain episodes, avascular necrosis of th ... Full text Cite

Perception of Nutrition in Adults with Sickle Cell Disease: Concept Elicitation of Nutritional Status and the Mini Nutrition Assessment

Conference Blood · November 2, 2023 IntroductionSickle Cell Disease (SCD) is a genetic disorder that causes recurrent vaso-occlusive pain events, functional impairment, and nutritional deficiencies due to increased metabol ... Full text Cite

Experiences with Working: A Letter from Older Adults with Sickle Cell Disease

Conference Blood · November 2, 2023 IntroductionWith medical advancements over the past decades, the life expectancy for people with sickle cell disease (SCD) is longer than ever before. As they age, people with SCD acquir ... Full text Cite

COVID mRNA Vaccination Responses in Individuals with Sickle Cell Disease: An ASH Research Collaborative Clinical Trial Network Study

Conference Blood · November 2, 2023 A multi-state, long term follow-up study of individuals with sickle cell disease (SCD) demonstrated an increase in mortality and morbidity in children and adults with COVID. mRNA vaccines are recommended for all SCD pat ... Full text Cite

Identifying Geriatric Syndromes in Adults with Sickle Cell Disease Using the Sickle Cell Disease Functional Assessment (SCD-FA): The Sickle Cell Disease Functional Assessment Validation and Refinement (SILVR) Study

Conference Blood · November 2, 2023 IntroductionAs people with sickle cell disease (SCD) age, they experience accelerated functional decline. Since historically survival for SCD has been limited to middle age, geriatric sy ... Full text Cite

Successful quality improvement project to increase hydroxyurea prescriptions for children with sickle cell anaemia.

Journal Article BMJ Qual Saf · October 2023 Hydroxyurea (HU) is an effective but underused disease-modifying therapy for patients with sickle cell anaemia (SCA). EMBRACE SCD, a sickle cell disease treatment demonstration project, aimed to improve access to HU by increasing prescription (Rx) rates by ... Full text Link to item Cite

Common data model for sickle cell disease surveillance: considerations and implications.

Journal Article JAMIA Open · July 2023 OBJECTIVE: Population-level data on sickle cell disease (SCD) are sparse in the United States. The Centers for Disease Control and Prevention (CDC) is addressing the need for SCD surveillance through state-level Sickle Cell Data Collection Programs (SCDC). ... Full text Open Access Link to item Cite

Cognitive-motor dual-task interference in adults with sickle cell disease.

Journal Article Gait Posture · May 2023 BACKGROUND: Sickle cell disease (SCD) is a genetic disorder that causes physical and cognitive impairment due to hemolysis, painful vaso-occlusion episodes, joint avascular necrosis, and strokes. As individuals with SCD age and develop conditions impacting ... Full text Link to item Cite

Management of Older Adults with Sickle Cell Disease: Considerations for Current and Emerging Therapies.

Journal Article Drugs Aging · April 2023 People with sickle cell disease (SCD) are living longer than ever before, with the median survival increasing from age 14 years in 1973, beyond age 40 years in the 1990s, and as high as 61 years in recent cohorts from academic centers. Improvements in surv ... Full text Link to item Cite

Use of Structured Electronic Health Records Data Elements for the Development of Computable Phenotypes to Identify Potential Adverse Events Associated with Intravenous Immunoglobulin Infusion.

Journal Article Drug Saf · March 2023 INTRODUCTION: Detection of adverse reactions to drugs and biologic agents is an important component of regulatory approval and post-market safety evaluation. Real-world data, including insurance claims and electronic health records data, are increasingly u ... Full text Link to item Cite

Hemorrhagic Stroke in Children and Adults With Sickle Cell Anemia: The Post-STOP Cohort.

Journal Article Stroke · November 2022 BACKGROUND: Hemorrhagic stroke in young patients with sickle cell anemia remains poorly characterized. METHODS: The Post-STOP (Stroke Prevention Trial in Sickle Cell Anemia) retrospective study collected follow-up data on STOP and STOP II clinical trial co ... Full text Link to item Cite

Pediatric hematology providers' contraceptive practices for female adolescents and young adults with sickle cell disease: A national survey.

Journal Article Pediatr Blood Cancer · October 2022 BACKGROUND: Adolescent and young adult (AYA) women with sickle cell disease (SCD) have increased pregnancy-related health risks and are prescribed potentially teratogenic medications, yet limited data are available regarding pediatric SCD provider contrace ... Full text Link to item Cite

"Death is as Much Part of Life as Living": Attitudes and Experiences Preparing for Death from Older Adults with Sickle Cell Disease.

Journal Article Omega (Westport) · July 20, 2022 The life-limiting and unpredictable nature of sickle cell disease (SCD) is well-established, yet there is limited literature on end-of-life planning. The purpose of this study was to describe perspectives about preparing for death for older adults with SCD ... Full text Link to item Cite

Molecular pathogenesis and heterogeneity in type 3 VWD families in U.S. Zimmerman program.

Journal Article J Thromb Haemost · July 2022 BACKGROUND: Type 3 von Willebrand Disease (VWD) is a rare and severe form of VWD characterized by the absence of von Willebrand factor (VWF). OBJECTIVES: As part of the Zimmerman Program, we sought to explore the molecular pathogenesis, correlate bleeding ... Full text Link to item Cite

The Sickle Cell Disease Functional Assessment (SCD-FA) tool: a feasibility pilot study.

Journal Article Pilot Feasibility Stud · March 4, 2022 BACKGROUND: The life expectancy for individuals with sickle cell disease (SCD) has greatly increased over the last 50 years. Adults with SCD experience multiple complications such as cardiopulmonary disease, strokes, and avascular necrosis that lead to lim ... Full text Open Access Link to item Cite

Living Beyond Life Expectancy: Experience with Aging for Older Adults with Sickle Cell Disease

Conference Blood · November 5, 2021 AbstractBackgroundOver the last five decades, advancements in management of sickle cell disease (SCD) have led to more individuals living beyond the ... Full text Cite

Sickle Cell Screening in Children in a Resource Constrained Environment: Diagnosis and Follow up

Conference Blood · November 5, 2021 AbstractIntroduction: Sickle cell disease (SCD) is the most common hemoglobinopathy in the world and it disproportionately affects population in tropical, resource-constrained regions where there is a high p ... Full text Cite

National Survey of Pediatric Sickle Cell Providers on Their Contraceptive Practices for Female Patients

Conference Blood · November 5, 2021 AbstractIntroduction: Pregnancies of women with sickle cell disease (SCD) have increased risk of morbidity and mortality, with potential additional complications due to uncertain teratogenicity of hydroxyure ... Full text Cite

Proteomic discovery in sickle cell disease: Elevated neurogranin levels in children with sickle cell disease.

Journal Article Proteomics Clin Appl · September 2021 PURPOSE: Sickle cell disease (SCD) is an inherited hemoglobinopathy that causes stroke and silent cerebral infarct (SCI). Our aim was to identify markers of brain injury in SCD. EXPERIMENTAL DESIGN: Plasma proteomes were analyzed using a sequential separat ... Full text Link to item Cite

Pediatric Neurodevelopmental Delays in Children 0 to 5 Years of Age With Sickle Cell Disease: A Systematic Literature Review.

Journal Article J Pediatr Hematol Oncol · April 1, 2021 INTRODUCTION: Sickle cell disease (SCD) is the most common abnormal genetic blood disease that affects ∼100,000 Americans. Approximately 20% to 37% of children with sickle cell anemia have silent cerebral infarcts by the age of 14 years old. Neurocognitive ... Full text Link to item Cite

Trial design of comparing patient-specific versus weight-based protocols to treat vaso-occlusive episodes in sickle cell disease (COMPARE-VOE).

Journal Article Contemp Clin Trials · February 2021 OBJECTIVES: Painful vaso-occlusive episodes (VOE) are the most common reason for emergency department (ED) visits experienced by patients with sickle cell disease (SCD). The National Heart, Lung and Blood Institute (NHLBI) evidence-based recommendations fo ... Full text Link to item Cite

Barriers and Facilitators of Advance Care Planning for Older Adults with Sickle Cell Disease

Conference Blood · November 5, 2020 BackgroundLife expectancy for individuals with sickle cell disease (SCD) has improved significantly in the last 50 years, creating a new population of older adults with SCD; however, life expectancy of adults with S ... Full text Cite

Functional Assessment in Younger and Older Adults with Sickle Cell Disease

Conference Blood · November 5, 2020 BackgroundNearly 95% of individuals with sickle cell disease (SCD) live to become adults (age ≥ 18 years). As individuals with SCD age, they acquire both SCD and age-related complications leading to functional decli ... Full text Cite

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

Journal Article Blood Adv · August 25, 2020 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 sur ... Full text Link to item Cite

American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain.

Journal Article Blood Adv · June 23, 2020 BACKGROUND: The management of acute and chronic pain for individuals living with sickle cell disease (SCD) is a clinical challenge. This reflects the paucity of clinical SCD pain research and limited understanding of the complex biological differences betw ... Full text Open Access Link to item Cite

Comparison of US Federal and Foundation Funding of Research for Sickle Cell Disease and Cystic Fibrosis and Factors Associated With Research Productivity.

Journal Article JAMA Netw Open · March 2, 2020 IMPORTANCE: Sickle cell disease (SCD) and cystic fibrosis (CF) are severe autosomal recessive disorders associated with intermittent disease exacerbations that require hospitalizations, progressive chronic organ injury, and substantial premature mortality. ... Full text Open Access Link to item Cite

Quantification of whole-brain oxygenation extraction fraction and cerebral metabolic rate of oxygen consumption in adults with sickle cell anemia using individual T2 -based oxygenation calibrations.

Journal Article Magn Reson Med · March 2020 PURPOSE: To evaluate different T2 -oxygenation calibrations for estimating venous oxygenation in people with sickle cell anemia (SCA). METHODS: Blood T2 values were measured at 3 T in the internal jugular veins of 12 healthy volunteers and 11 SCA participa ... Full text Link to item Cite

Geriatric assessment for older adults with sickle cell disease: protocol for a prospective cohort pilot study.

Journal Article Pilot Feasibility Stud · 2020 BACKGROUND: The life expectancy for people with sickle cell disease (SCD) has improved tremendously over the last 50 years. This population experiences hemolysis and vaso-occlusion in multiple organs that lead to complications such as cardiopulmonary disea ... Full text Link to item Cite

End points for sickle cell disease clinical trials: patient-reported outcomes, pain, and the brain.

Journal Article Blood Adv · December 10, 2019 To address the global burden of sickle cell disease (SCD) and the need for novel therapies, the American Society of Hematology partnered with the US Food and Drug Administration to engage the work of 7 panels of clinicians, investigators, and patients to d ... Full text Open Access Link to item Cite

Correlates of successful transition in young adults with sickle cell disease.

Journal Article Pediatr Blood Cancer · December 2019 The transition period from pediatric care to adult care for patients with sickle cell disease (SCD) is associated with increased mortality and morbidity. Identification of risk factors for unsuccessful transition may aid in developing strategies to improve ... Full text Link to item Cite

Assessing Feasibility of a Focused Geriatric Assessment in Older Adults with Sickle Cell Disease to Address Functional Risk Factors for Morbidity and Mortality

Journal Article Blood · November 13, 2019 BackgroundDuring the last five decades the life expectancy for people living with sickle cell disease (SCD) has improved markedly, with median survival of 61 years in recent cohorts enrolle ... Full text Open Access Cite

Proteomic Discovery: Elevated Neurogranin Levels in Children with Sickle Cell Disease

Conference Blood · November 13, 2019 Sickle cell disease (SCD) is an inherited hemoglobinopathy that frequently causes neurological complications, such as stroke, silent cerebral infarct (SCI) and other forms of brain injury, including loss of cognition. SCI is defined as any ischemic ... Full text Cite

Developing a risk-based composite neurologic outcome for a trial of hydroxyurea in young children with sickle cell disease.

Journal Article Clin Trials · February 2019 BACKGROUND: Studies of interventions to prevent the many neurological complications of sickle cell disease must take into account multiple outcomes of variable severity, with limited sample size. The goals of the studies presented were to use investigator ... Full text Link to item Cite

Disparities in Foundation and Federal Support and Development of New Therapeutics for Sickle Cell Disease and Cystic Fibrosis

Conference Blood · November 29, 2018 AbstractBACKGROUND: Sickle cell disease (SCD) and cystic fibrosis (CF) are rare inherited disorders of similar severity. Disparities in funding between these two diseases have been long recognized and likely ... Full text Cite

Allergic Sensitization Is Associated with Decreased Risk of ED Visits and Hospitalizations for Pain in Sickle Cell Disease

Conference Blood · November 29, 2018 AbstractRationale: Lower airway disease is common in pediatric sickle cell disease (SCD) and is often diagnosed as asthma. This condition may be a risk factor for acute chest syndrome (ACS) and pain episodes ... Full text Cite

Evaluation of the WHO 2009 classification for diagnosis of acute dengue in a large cohort of adults and children in Sri Lanka during a dengue-1 epidemic.

Journal Article PLoS Negl Trop Dis · February 2018 BACKGROUND: Dengue is a leading cause of fever and mimics other acute febrile illnesses (AFI). In 2009, the World Health Organization (WHO) revised criteria for clinical diagnosis of dengue. METHODOLOGY/PRINCIPAL FINDINGS: The new WHO 2009 classification o ... Full text Link to item Cite

Silent cerebral infarct definitions and full-scale IQ loss in children with sickle cell anemia.

Journal Article Neurology · January 16, 2018 OBJECTIVE: To evaluate whether application of the adult definition of silent cerebral infarct (SCI) (T2-weighted hyperintensity ≥5 mm with corresponding T1-weighted hypointensity on MRI) is associated with full-scale IQ (FSIQ) loss in children with sickle ... Full text Link to item Cite

Accounting for the role of hematocrit in between-subject variations of MRI-derived baseline cerebral hemodynamic parameters and functional BOLD responses.

Journal Article Hum Brain Mapp · January 2018 Baseline hematocrit fraction (Hct) is a determinant for baseline cerebral blood flow (CBF) and between-subject variation of Hct thus causes variation in task-based BOLD fMRI signal changes. We first verified in healthy volunteers (n = 12) that Hct values c ... Full text Link to item Cite

Asthma Screening in Pediatric Sickle Cell Disease: A Clinic-Based Program Using Questionnaires and Spirometry.

Journal Article Pediatr Allergy Immunol Pulmonol · December 1, 2017 A clinician diagnosis of asthma is associated with increased morbidity and mortality in people with sickle cell disease (SCD). We hypothesized that a screening program would help identify children with asthma needing referral to pulmonary clinic. We conduc ... Full text Link to item Cite

Treatment of an acquired Factor XIII inhibitor in an adolescent with systemic lupus erythematosus and renal failure.

Journal Article Transfusion · September 2017 BACKGROUND: Factor (F)XIII deficiency is a rare inherited bleeding disorder, but can also be acquired due to the development of inhibitors. CASE REPORT: A 17-year-old female with systemic lupus erythematosus and end-stage kidney disease secondary to Class ... Full text Open Access Link to item Cite

Fast measurement of blood T1 in the human carotid artery at 3T: Accuracy, precision, and reproducibility.

Journal Article Magnetic resonance in medicine · June 2017 PurposeTo develop a fast protocol for measuring T1 values in the internal carotid artery (ICA), to validate this technique with in vitro measurements, and to evaluate its reproducibility.MethodsA modified Look-Locker sequence w ... Full text Cite

Increased prevalence of potential right-to-left shunting in children with sickle cell anaemia and stroke.

Journal Article Br J Haematol · January 2017 'Paradoxical' embolization via intracardiac or intrapulmonary right-to-left shunts (RLS) is an established cause of stroke. Hypercoagulable states and increased right heart pressure, which both occur in sickle cell anaemia (SCA), predispose to paradoxical ... Full text Link to item Cite

Cost-Effectiveness of Blood Transfusions Versus Observation for Silent Cerebral Infarcts from the Silent Cerebral Infarct Trial

Conference Blood · December 2, 2016 AbstractBACKGROUND AND OBJECTIVE: In the Silent Cerebral Infarct Trial (SIT), regular blood transfusion therapy significantly reduced the incidence of recurrent cerebral infarctions ... Full text Cite

Simultaneous Measurement of Cerebral Blood Flow and Arterial Transit Time for Sickle Cell Disease

Conference Blood · December 2, 2016 AbstractIntroductionStudies of patients with ischemic stroke and silent cerebral infarcts from sickle cell anemia (SCA) have revealed abnormalities of both large and small ve ... Full text Cite

Secondhand Smoke Is an Important Modifiable Risk Factor in Sickle Cell Disease: A Review of the Current Literature and Areas for Future Research.

Journal Article Int J Environ Res Public Health · November 12, 2016 Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy that causes significant morbidity and mortality related to chronic hemolytic anemia, vaso-occlusion, and resultant end-organ damage. Tobacco smoke exposure (TSE) through secondhand smoke ... Full text Open Access Link to item Cite

Emergence of Epidemic Dengue-1 Virus in the Southern Province of Sri Lanka.

Journal Article PLoS Negl Trop Dis · October 2016 BACKGROUND: Dengue is a frequent cause of acute febrile illness with an expanding global distribution. Since the 1960s, dengue in Sri Lanka has been documented primarily along the heavily urbanized western coast with periodic shifting of serotypes. Outbrea ... Full text Open Access Link to item Cite

Symptomatic Avascular Necrosis: An Understudied Risk Factor for Acute Care Utilization by Patients with SCD.

Journal Article South Med J · September 2016 OBJECTIVES: Sickle cell disease (SCD) is associated with high healthcare utilization rates and poor outcomes in a subset of patients, although the underlying factors that predict this phenotype are poorly understood. Prior studies suggest that comorbid ava ... Full text Link to item Cite

Management of Sickle Cell Disease in Children.

Journal Article South Med J · September 2016 Sickle cell disease (SCD) is a heterogeneous inherited disorder of hemoglobin that causes chronic hemolytic anemia, vaso-occlusion, and endothelial dysfunction. These physiologic derangements often lead to multiorgan damage in infancy and throughout childh ... Full text Link to item Cite

Personalized Reminders Increase Screening for Stroke Risk in Children with Sickle Cell Anemia.

Journal Article South Med J · September 2016 OBJECTIVES: Transcranial Doppler ultrasound (TCD) effectively identifies children with sickle cell anemia (SCA) who are at increased risk of stroke. We evaluated a low-cost quality improvement (QI) intervention to increase the proportion of children screen ... Full text Link to item Cite

Utility of the Montreal Cognitive Assessment as a Screening Test for Neurocognitive Dysfunction in Adults with Sickle Cell Disease.

Journal Article South Med J · September 2016 OBJECTIVES: Neurocognitive dysfunction is an important complication of sickle cell disease (SCD), but little is published on the utility of screening tests for cognitive impairment in people with the disease. The purpose of this study was to evaluate the M ... Full text Link to item Cite

Effect of Free Dental Services on Individuals with Sickle Cell Disease.

Journal Article South Med J · September 2016 OBJECTIVES: Poor oral health can have a negative impact on overall health. This is especially concerning for individuals with sickle cell disease (SCD), an inherited blood disorder that affects hemoglobin and can lead to an increased risk of infection and ... Full text Link to item Cite

Community Health Workers as Support for Sickle Cell Care.

Journal Article Am J Prev Med · July 2016 Community health workers are increasingly recognized as useful for improving health care and health outcomes for a variety of chronic conditions. Community health workers can provide social support, navigation of health systems and resources, and lay couns ... Full text Link to item Cite

The Association between Educational Attainment and Patterns of Emergency Department Utilization among Adults with Sickle Cell Disease.

Journal Article Int J Behav Med · June 2016 PURPOSE: Patients with low educational attainment may be at increased risk for unplanned health care utilization. This study aimed to determine what factors are related to emergency department (ED) visits in hopes of guiding treatments and early interventi ... Full text Link to item Cite

Preventive Care Delivery to Young Children With Sickle Cell Disease.

Journal Article J Pediatr Hematol Oncol · May 2016 Preventive services can reduce the morbidity of sickle cell disease (SCD) in children but are delivered unreliably. We conducted a retrospective cohort study of children aged 2 to 5 years with SCD, evaluating each child for 14 months and expecting that he/ ... Full text Link to item Cite

Secondhand smoke is associated with more frequent hospitalizations in children with sickle cell disease.

Journal Article Am J Hematol · March 2016 Tobacco smoke exposure has been associated with more frequent hospitalizations in children with sickle cell disease (SCD), but previous studies have not quantified the exposure by objective methods. We enrolled 50 children and young adults with SCD in a re ... Full text Link to item Cite

Improving Quality of Care for Sickle Cell Patients in the Pediatric Emergency Department.

Journal Article Pediatr Emerg Care · January 2016 OBJECTIVE: The aim of this study was to identify the factors associated with delays in treatment of sickle pain crisis in the pediatric emergency department with the goal of discerning whether earlier pain management is correlated with better clinical outc ... Full text Link to item Cite

Lower Airway Obstruction Is Associated With Free Hemoglobin In Sickle Cell Disease

Conference AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE · January 1, 2016 Link to item Cite

Sickle cell disease.

Chapter · 2016 Sickle cell disease (SCD) is an inherited hemoglobinopathy caused by a mutation in the sixth amino acid of the β-globin gene (HBB). It is the most common serious genetic diseases in childhood, affecting approximately 1 in 2500 births and 100 000 individual ... Full text Link to item Cite

Primary Care Providers' Comfort Levels in Caring for Patients with Sickle Cell Disease.

Journal Article South Med J · September 2015 OBJECTIVE: The purpose of this study was to determine the comfort levels of primary care providers in caring for individuals with sickle cell disease (SCD) and determine factors that improved or lessened provider comfort. METHODS: We surveyed providers at ... Full text Link to item Cite

Quality improvement process in a sickle cell infusion center.

Journal Article Am J Med · May 2015 BACKGROUND: The American Pain Society recommends that individuals experiencing sickle cell crisis receive parenteral pain medication within 30 minutes of assessment. We examined factors affecting achievement of this standard at the Johns Hopkins Sickle Cel ... Full text Link to item Cite

Crucial role for the VWF A1 domain in binding to type IV collagen.

Journal Article Blood · April 2, 2015 Von Willebrand factor (VWF) contains binding sites for platelets and for vascular collagens to facilitate clot formation at sites of injury. Although previous work has shown that VWF can bind type IV collagen (collagen 4), little characterization of this i ... Full text Link to item Cite

Health-related quality of life in children with sickle cell anemia: impact of blood transfusion therapy.

Journal Article Am J Hematol · February 2015 The completion of the Multicenter Silent Infarct Transfusion Trial demonstrated that children with pre-existing silent cerebral infarct and sickle cell anemia (SCA) who received regular blood transfusion therapy had a 58% relative risk reduction of infarct ... Full text Link to item Cite

Transcranial Doppler screening of Medicaid-insured children with sickle cell disease.

Journal Article J Pediatr · January 2015 Transcranial Doppler screening reduces the risk of stroke in children with sickle cell disease. We tested the effect of informational letters sent to parents and doctors of Medicaid-insured children on improving screening efficiency. The letters did not im ... Full text Link to item Cite

Screening for Neurocognitive Dysfunction in an Adult Population with Sickle Cell Disease

Conference Blood · December 6, 2014 AbstractIntroduction: Studies have described neuroimaging abnormalities and neurocognitive dysfunction in adults living with sickle cell anemia and no previous history of neurological impairment. At the John ... Full text Cite

Perceived discrimination, patient trust, and adherence to medical recommendations among persons with sickle cell disease.

Journal Article J Gen Intern Med · December 2014 BACKGROUND: Adults with sickle cell disease (SCD) report experiencing discriminatory behavior from some healthcare providers. The impact of discrimination on health outcomes in SCD, including adherence to physician recommendations, is not known. OBJECTIVE: ... Full text Link to item Cite

Reproducibility of detecting silent cerebral infarcts in pediatric sickle cell anemia.

Journal Article J Child Neurol · December 2014 Detecting silent cerebral infarcts on magnetic resonance images (MRIs) in children with sickle cell anemia is challenging, yet reproducibility of readings has not been examined in this population. We evaluated consensus rating, inter-, and intra-grader agr ... Full text Link to item Cite

Congenital platelet disorder and type I von Willebrand disease presenting as prolonged bleeding after cleft lip and palate repair.

Journal Article Cleft Palate Craniofac J · November 2014 Cleft lip and palate can be associated with coagulopathy. Here, we report the first known case of congenital platelet disorder and von Willebrand disease presenting as prolonged bleeding after cleft lip and palate repair. After identifying the underlying p ... Full text Link to item Cite

Perceived discrimination in health care is associated with a greater burden of pain in sickle cell disease.

Journal Article J Pain Symptom Manage · November 2014 CONTEXT: Perceived discriminatory experiences in society have been associated with a higher burden of pain among some minority patient populations. OBJECTIVES: To describe the extent to which patients with sickle cell disease (SCD) perceive discrimination ... Full text Link to item Cite

Silent cerebral infarction, income, and grade retention among students with sickle cell anemia.

Journal Article Am J Hematol · October 2014 Children with sickle cell anemia have a higher-than-expected prevalence of poor educational attainment. We test two key hypotheses about educational attainment among students with sickle cell anemia, as measured by grade retention and use of special educat ... Full text Link to item Cite

Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia.

Journal Article N Engl J Med · August 21, 2014 BACKGROUND: Silent cerebral infarcts are the most common neurologic injury in children with sickle cell anemia and are associated with the recurrence of an infarct (stroke or silent cerebral infarct). We tested the hypothesis that the incidence of the recu ... Full text Link to item Cite

An unequal burden: poor patient-provider communication and sickle cell disease.

Journal Article Patient Educ Couns · August 2014 OBJECTIVE: To assess disparities in the quality of healthcare provider communication experienced by African-American adults with and without sickle cell disease (SCD) in the U.S. METHODS: Poor provider communication was assessed by the Provider Communicati ... Full text Link to item Cite

Randomized controlled trial of sildenafil for preventing recurrent ischemic priapism in sickle cell disease.

Journal Article Am J Med · July 2014 BACKGROUND: Successful preventive therapy for ischemic priapism, a disorder of penile erection with major physical and psychologic consequences, is limited. We conducted a randomized, double-blind, placebo-controlled clinical trial to assess the efficacy a ... Full text Link to item Cite

Attitudes toward clinical trials among patients with sickle cell disease.

Journal Article Clin Trials · June 2014 Background A substantial number of planned clinical trials for sickle cell disease (SCD) have terminated early due to insufficient patient enrollment. Purpose To describe attitudes toward clinical trials among a sample of adults with SCD and identify patie ... Full text Link to item Cite

Respiratory syncytial virus and seasonal influenza cause similar illnesses in children with sickle cell disease.

Journal Article Pediatr Blood Cancer · May 2014 BACKGROUND: Respiratory syncytial virus (RSV) is a cause of acute chest syndrome (ACS) in sickle cell disease (SCD), but its clinical course and acute complications have not been well characterized. We compared RSV to seasonal influenza infections in child ... Full text Link to item Cite

New insights provided by a comparison of impaired deformability with erythrocyte oxidative stress for sickle cell disease.

Journal Article Blood Cells Mol Dis · April 2014 Sickle cell disease (SCD) is associated with increase in oxidative stress and irreversible membrane changes that originates from the instability and polymerization of deoxygenated hemoglobin S (HbS). The relationship between erythrocyte membrane changes as ... Full text Link to item Cite

Computerized physician order entry improves compliance with a manual exchange transfusion protocol in the pediatric intensive care unit.

Journal Article J Pediatr Hematol Oncol · March 2014 AIM: To evaluate the use of a computerized physician order entry (CPOE) protocol on manual red blood cell (RBC) exchange transfusion in critically ill children with sickle cell disease. METHODS: We conducted a retrospective study of children with sickle ce ... Full text Link to item Cite

Hospital-associated venous thromboembolism in children: incidence and clinical characteristics.

Journal Article J Pediatr · February 2014 OBJECTIVE: To determine incidence and clinical characteristics of hospital-associated venous thromboembolism (VTE) in pediatric patients. STUDY DESIGN: A retrospective analysis of patients with hospital-associated VTE at the Johns Hopkins Hospital from 199 ... Full text Link to item Cite

Parent education and biologic factors influence on cognition in sickle cell anemia.

Journal Article Am J Hematol · February 2014 Children with sickle cell anemia have a high prevalence of silent cerebral infarcts (SCIs) that are associated with decreased full-scale intelligence quotient (FSIQ). While the educational attainment of parents is a known strong predictor of the cognitive ... Full text Link to item Cite

VARIATION IN THE TREATMENT OF STROKE IN SICKLE CELL DISEASE: A SURVEY OF PROVIDERS

Journal Article AMERICAN JOURNAL OF HEMATOLOGY · December 1, 2013 Link to item Cite

IMPROVING QUALITY OF CARE FOR SICKLE CELL PATIENTS IN THE PEDIATRIC EMERGENCY DEPARTMENT

Journal Article AMERICAN JOURNAL OF HEMATOLOGY · December 1, 2013 Link to item Cite

Primary Care Providers Comfort Levels in Caring for Patients with Sickle Cell Disease

Journal Article AMERICAN JOURNAL OF HEMATOLOGY · December 1, 2013 Link to item Cite

Perceived Discrimination In Health Care Is Associated With Daily Chronic Pain In Sickle Cell Disease

Conference Blood · November 15, 2013 AbstractIntroductionPerceived discrimination due to race or ethnicity has been associated with a greater burden of pain among minority ... Full text Cite

A Risk-Prediction Model For Identifying Venous Thromboembolism In Hospitalized Pediatric Patients: A Single Institution Retrospective Case-Control Analysis

Conference Blood · November 15, 2013 AbstractIntroductionVenous thromboembolism (VTE) is increasing in children, especially in the tertiary care setting. Hospital-associat ... Full text Cite

High rates of recurrent biliary tract obstruction in children with sickle cell disease.

Journal Article Pediatr Blood Cancer · April 2013 BACKGROUND: Individuals with sickle cell disease (SCD) have an increased risk of cholelithiasis from bilirubin stones. Symptomatic biliary tract disease (BTD) includes acute and chronic cholecystitis, obstruction of the common bile duct (CBD), cholangitis, ... Full text Link to item Cite

A double-blind, randomized, multicenter phase 2 study of prasugrel versus placebo in adult patients with sickle cell disease.

Journal Article J Hematol Oncol · February 17, 2013 BACKGROUND: Platelet activation has been implicated in the pathogenesis of sickle cell disease (SCD) suggesting antiplatelet agents may be therapeutic. To evaluate the safety of prasugrel, a thienopyridine antiplatelet agent, in adult patients with SCD, we ... Full text Link to item Cite

Chikungunya as a cause of acute febrile illness in southern Sri Lanka.

Journal Article PLoS One · 2013 BACKGROUND: Chikungunya virus (CHIKV) re-emerged in Sri Lanka in late 2006 after a 40-year hiatus. We sought to identify and characterize acute chikungunya infection (CHIK) in patients presenting with acute undifferentiated febrile illness in unstudied rur ... Full text Open Access Link to item Cite

Anticoagulation monitoring during pediatric extracorporeal membrane oxygenation.

Journal Article ASAIO J · 2013 The best method of monitoring anticoagulation during extracorporeal membrane oxygenation (ECMO) is unknown. We conducted a prospective observational study in a tertiary pediatric intensive care unit. Antifactor Xa, antithrombin (AT), and factor VIII activi ... Full text Link to item Cite

Acute silent cerebral ischemic events in children with sickle cell anemia.

Journal Article JAMA Neurol · January 2013 BACKGROUND: Irregular, sporadic episodes of ischemic brain injury are known to occur in sickle cell anemia (SCA), resulting in overt stroke and silent cerebral infarction. Ongoing ischemia in other organs is common in SCA but has never been documented in t ... Full text Link to item Cite

Patent foramen ovale in patients with sickle cell disease and stroke: case presentations and review of the literature.

Journal Article Case Rep Hematol · 2013 Although individuals with sickle cell disease (SCD) are at increased risk for stroke, the underlying pathophysiology is incompletely understood. Intracardiac shunting via a patent foramen ovale (PFO) is associated with cryptogenic stroke in individuals wit ... Full text Link to item Cite

Describing Adherence to Recommended Preventative Care Behaviors Among Adults with Sickle Cell Disease

Conference Blood · November 16, 2012 AbstractAbstract 2058Background:Healthcare professionals caring for patients with sickle cell disease ... Full text Cite

Ambulatory care connections of Medicaid-insured children with sickle cell disease.

Journal Article Pediatr Blood Cancer · November 2012 BACKGROUND: Sickle cell disease (SCD) requires coordinated ambulatory care from generalists and hematologists. We examined when children with SCD establish ambulatory care connections, whether these connections are maintained, and how these connections are ... Full text Link to item Cite

Hydroxyurea for the treatment of sickle cell disease: efficacy, barriers, toxicity, and management in children.

Journal Article Pediatr Blood Cancer · August 2012 Hydroxyurea is the only approved medication in the United States for the treatment of sickle cell anemia (HbSS) and is widely used in children despite an indication limited to adults. We review the evidence of efficacy and safety in children with reference ... Full text Link to item Cite

NEONATAL AORTIC THROMBOSIS: A CASE SERIES

Conference PEDIATRIC BLOOD & CANCER · July 1, 2012 Link to item Cite

Comparison of the use and results of sentinel lymph node biopsy in children and young adults with melanoma.

Journal Article Cancer · May 15, 2012 BACKGROUND: Data on sentinel lymph node (SLN) biopsy in children with melanoma are limited. In this study, the authors compared the factors associated with SLN biopsy use and metastases in pediatric and young adult patients with melanoma. METHODS: The 2008 ... Full text Link to item Cite

Unsuspected rickettsioses among patients with acute febrile illness, Sri Lanka, 2007.

Journal Article Emerg Infect Dis · May 2012 We studied rickettsioses in southern Sri Lanka. Of 883 febrile patients with paired serum samples, 156 (17.7%) had acute rickettsioses; rickettsioses were unsuspected at presentation. Additionally, 342 (38.7%) had exposure to spotted fever and/or typhus gr ... Full text Link to item Cite

Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure.

Journal Article Blood · April 19, 2012 The most common form of neurologic injury in sickle cell anemia (SCA) is silent cerebral infarction (SCI). In the Silent Cerebral Infarct Multi-Center Clinical Trial, we sought to identify risk factors associated with SCI. In this cross-sectional study, we ... Full text Link to item Cite

Unsuspected dengue and acute febrile illness in rural and semi-urban southern Sri Lanka.

Journal Article Emerg Infect Dis · February 2012 Dengue virus (DENV), a globally emerging cause of undifferentiated fever, has been documented in the heavily urbanized western coast of Sri Lanka since the 1960s. New areas of Sri Lanka are now being affected, and the reported number and severity of cases ... Full text Link to item Cite

Unsuspected rickettsioses among patients with acute febrile Illness, Sri Lanka, 2007

Journal Article Emerging Infectious Diseases · 2012 We studied rickettsioses in southern Sri Lanka. Of 883 febrile patients with paired serum samples, 156 (17.7%) had acute rickettsioses; rickettsioses were unsuspected at presentation. Additionally, 342 (38.7%) had exposure to spotted fever and/or typhus gr ... Full text Cite

The epidemiology, evaluation and treatment of stroke in adults with sickle cell disease.

Journal Article Expert Rev Hematol · December 2011 Stroke is a frequent and severe complication in adults with sickle cell disease. Ischemic stroke often causes physical and cognitive disability, while hemorrhagic stroke has a high mortality rate. As more children survive, the number of strokes in adults i ... Full text Link to item Cite

Glial fibrillary acidic protein as a brain injury biomarker in children undergoing extracorporeal membrane oxygenation.

Journal Article Pediatr Crit Care Med · September 2011 OBJECTIVE: To determine whether, in children, plasma glial fibrillary acidic protein is associated with brain injury during extracorporeal membrane oxygenation and with mortality. DESIGN: Prospective, observational study. SETTING: Pediatric intensive care ... Full text Link to item Cite

Leptospirosis as frequent cause of acute febrile illness in southern Sri Lanka.

Journal Article Emerg Infect Dis · September 2011 To determine the proportion of fevers caused by leptospirosis, we obtained serum specimens and epidemiologic and clinical data from patients in Galle, Sri Lanka, March-October 2007. Immunoglobulin M ELISA was performed on paired serum specimens to diagnose ... Full text Link to item Cite

Subtotal splenic embolization is a safe and effective treatment for isolated splenic vascular tumors associated with consumptive coagulopathy.

Journal Article J Pediatr Hematol Oncol · July 2011 Consumptive coagulopathy is a known complication of large vascular tumors. We describe 2 episodes of consumptive coagulopathy in young children, which were secondary to isolated splenic vascular tumors. One child was successfully treated by subtotal emboli ... Full text Link to item Cite

Fast measurement of blood T1 in the human jugular vein at 3 Tesla.

Journal Article Magn Reson Med · May 2011 Current T(1) values for blood at 3T largely came from in vitro studies on animal blood or freshly drawn human blood. Measurement of blood T(1) in vivo could provide more specific information, e.g., for individuals with abnormal blood composition. Here, blo ... Full text Link to item Cite

Urgency of emergency department visits by children with sickle cell disease: a comparison of 3 chronic conditions.

Journal Article Acad Pediatr · 2011 OBJECTIVE: Children with sickle cell disease (SCD) often receive care in the emergency department (ED), but the urgency of these frequent visits is not well understood. This study examined ED use by children with SCD by comparing the urgency of ED visits a ... Full text Link to item Cite

Severe pandemic H1N1 and seasonal influenza in children and young adults with sickle cell disease.

Journal Article Blood · November 4, 2010 Influenza causes excess morbidity in sickle cell disease (SCD). H1N1 pandemic influenza has been severe in children. To compare H1N1 with seasonal influenza in SCD (patients younger than 22), we reviewed medical records (1993-2009). We identified 123 cases ... Full text Link to item Cite

Incidental findings on brain magnetic resonance imaging of children with sickle cell disease.

Journal Article Pediatrics · July 2010 OBJECTIVE: We describe the prevalence and range of incidental intracranial abnormalities identified through MRI of the brain in a large group of children screened for a clinical trial. METHODS: We included 953 children between 5 and 14 years of age who wer ... Full text Link to item Cite

Design of the silent cerebral infarct transfusion (SIT) trial.

Journal Article Pediatr Hematol Oncol · March 2010 BACKGROUND: Silent cerebral infarct (SCI) is the most common cause of serious neurological disease in sickle cell anemia (SCA), affecting approximately 22% of children. The goal of this trial is to determine whether blood transfusion therapy will reduce fu ... Full text Link to item Cite

Burden of influenza-related hospitalizations among children with sickle cell disease.

Journal Article Pediatrics · February 2010 OBJECTIVE: Children with sickle cell disease (SCD) are considered to be at high risk for complications from influenza infection despite minimal published data that characterize the burden of influenza in this population. Our objectives were to (1) estimate ... Full text Link to item Cite

Safety and Efficacy of a Manual Isovolumetric Double Volume Exchange Transfusion Protocol in Children

Conference AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE · January 1, 2010 Link to item Cite

DENGUE AMONG PATIENTS WITH UNDIFFERENTIATED FEVER IN SOUTHERN SRI LANKA

Conference AMERICAN JOURNAL OF TROPICAL MEDICINE AND HYGIENE · November 1, 2009 Link to item Cite

A systematic review of barriers and interventions to improve appropriate use of therapies for sickle cell disease.

Journal Article J Natl Med Assoc · October 2009 Clinical experts have expressed concern about underutilization of sickle cell disease (SCD) therapies, including hydroxyurea, prophylactic antibiotics, iron chelation, bone marrow transplantation, pain management during vaso-occlusive crisis, and receipt o ... Full text Link to item Cite

The excess burden of stroke in hospitalized adults with sickle cell disease.

Journal Article Am J Hematol · September 2009 This report compares the relative rates and risk factors associated with stroke in adults versus children with sickle cell disease (SCD) in the United States over the last decade. We identified incident strokes in patients with SCD using ICD-9 codes for ac ... Full text Link to item Cite

Delayed diagnosis of iliac vein thrombus in a sexually-active adolescent with Klippel-Trénaunay syndrome.

Journal Article J Pediatr Adolesc Gynecol · June 2009 BACKGROUND: Although iliac vein thrombus is uncommon in adolescents, it can present with pelvic inflammatory disease (PID) symptoms. CASE: A 19-year-old sexually active female with Klippel-Trénaunay syndrome (KTS) presented with fever, abdominal and lower ... Full text Link to item Cite

Hydroxyurea for sickle cell disease: a systematic review for efficacy and toxicity in children.

Journal Article Pediatrics · December 2008 CONTEXT: Hydroxyurea is the only approved medication for the treatment of sickle cell disease in adults; there are no approved drugs for children. OBJECTIVE: Our goal was to synthesize the published literature on the efficacy, effectiveness, and toxicity o ... Full text Link to item Cite

Systematic review: Hydroxyurea for the treatment of adults with sickle cell disease.

Journal Article Ann Intern Med · June 17, 2008 BACKGROUND: Hydroxyurea is the only approved drug for treatment of sickle cell disease. OBJECTIVE: To synthesize the published literature on the efficacy, effectiveness, and toxicity of hydroxyurea when used in adults with sickle cell disease. DATA SOURCES ... Full text Link to item Cite

Lymphadenopathy as the primary manifestation of malignant transformation in two patients with severe congenital neutropenia.

Journal Article Pediatr Blood Cancer · May 2008 We present the cases of two patients with severe congenital neutropenia (SCN) who both developed generalized adenopathy. Although both had recent histories that placed infection high on the differential of causes for the adenopathy, biopsies demonstrated a ... Full text Link to item Cite

Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease.

Journal Article Pediatr Blood Cancer · May 2008 BACKGROUND: Acute chest syndrome (ACS) is a frequent cause of hospitalization and mortality in children with sickle cell disease. Transfusion is often required to prevent respiratory failure and treatment with dexamethasone may reduce the length of admissi ... Full text Link to item Cite

Mutations in TMPRSS6 cause iron-refractory iron deficiency anemia (IRIDA).

Journal Article Nat Genet · May 2008 Iron deficiency is usually attributed to chronic blood loss or inadequate dietary intake. Here, we show that iron deficiency anemia refractory to oral iron therapy can be caused by germline mutations in TMPRSS6, which encodes a type II transmembrane serine ... Full text Link to item Cite

Hydroxyurea for the treatment of sickle cell disease.

Journal Article Evid Rep Technol Assess (Full Rep) · March 2008 OBJECTIVES: To synthesize the published literature on the efficacy, effectiveness, and toxicity of hydroxyurea (HU) when used for treatment of sickle cell disease (SCD); and to review the evidence regarding barriers to its use. DATA SOURCES: Articles cited ... Link to item Cite

Predictors of In-Hospital Mortality and Charges in Sickle Cell Disease: Results from the California Discharge Databases 1998–2005.

Conference Blood · November 16, 2007 AbstractMost studies of survival in sickle cell disease (SCD) include only patients followed at referral centers. We used the public set of the California Patient Discharge Databases (1998–2005) to compare i ... Full text Cite

Predictors of Acute Intracranial Pathology Identified by Computerized Tomography in Children with Sickle Cell Disease.

Journal Article Blood · November 16, 2006 AbstractHeadache and other symptoms and signs of possible CNS disease are common in children with sickle cell disease (SCD). Computerized tomography (CT) is a rapid and widely available method to image the s ... Full text Cite

Primary hemorrhagic stroke in children with sickle cell disease is associated with recent transfusion and use of corticosteroids.

Journal Article Pediatrics · November 2006 OBJECTIVES: Primary hemorrhagic stroke is an uncommon complication of sickle cell disease, with reported mortality rates of 24% to 65%. Most reported cases are in adults; little is known about its occurrence in children. Proposed risk factors include previ ... Full text Link to item Cite

Inverse correlation between cerebral blood flow measured by continuous arterial spin-labeling (CASL) MRI and neurocognitive function in children with sickle cell anemia (SCA).

Journal Article Blood · July 1, 2006 Overt stroke, clinically "silent" cerebral infarct, and neurocognitive impairment are frequent complications of sickle cell anemia (SCA). Current imaging techniques have limited sensitivity and specificity to identify children at risk for neurocognitive im ... Full text Link to item Cite

Corticosteroids and Increased Risk of Readmission after Acute Chest Syndrome.

Conference Blood · November 16, 2005 AbstractAcute chest syndrome (ACS) is a cause of frequent hospitalization and death in patients with sickle cell disease (SCD). Therapies commonly used include antibiotics, inhaled B-agonists, oxygen, high-d ... Full text Cite

Pediatric melanoma: risk factor and survival analysis of the surveillance, epidemiology and end results database.

Journal Article J Clin Oncol · July 20, 2005 PURPOSE: To evaluate risk factors for the development of and factors influencing survival in pediatric melanoma. PATIENTS AND METHODS: We evaluated 1,255 children (age < 20 years) and 2,673 young adults (age 20 to 24 years) with melanoma in the 2001 Nation ... Full text Link to item Cite

Significant responses to platinum-based chemotherapy in renal medullary carcinoma.

Journal Article Pediatr Blood Cancer · April 2005 Most patients with renal medullary carcinoma (RMC) have advanced disease at presentation and rarely respond to radiation or chemotherapy. We describe two adolescents with metastatic disease who had significant responses to cisplatin or carboplatin in combi ... Full text Link to item Cite

Risk Factors for Intracranial Hemorrhage in Children with Sickle Cell Anemia.

Conference Blood · November 16, 2004 Background: Intracranial hemorrhage (ICH) is an uncommon, but devastating, complication of sickle cell disease (SCD) with mortality from 30 to 65%. Most reported cases are in adults; little is known about children. Proposed risk factors include pre ... Full text Cite

Effect of maternal anemia at high altitude on infant hematocrit and oxygenation.

Journal Article Am J Trop Med Hyg · April 2004 Hematocrit levels were determined in 36 mothers living at high altitudes (3,750 meters) and their infant cord bloods to determine the effect of maternal anemia on the infant. The arterial oxygen saturation (SaO(2)) and respiratory rate of the infants were ... Link to item Cite

Ecology and management of mahogany (Swietenia macrophylla King) in the Chimanes Forest, Beni, Bolivia

Conference Botanical Journal of the Linnean Society · January 1, 1996 Mahogany (Swietenia macrophylla King) regeneration in areas of erosion on high terraces and in forest killed by flooding and deposition of alluvial sediments in the Chimanes Forest, Bolivia. These hydrological disturbances are patchy, and only one of five ... Full text Cite