A5023851992- Hemoglobinopathies and Related Disorders
- Iron Metabolism and Disorders
- Blood groups and transfusion
- Prenatal Screening and Diagnostics
- Bone and Joint Diseases
- Food Security and Health in Diverse Populations
- Pharmacological Effects and Toxicity Studies
- Hemoglobin structure and function
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Erythrocyte Function and Pathophysiology
- Hematological disorders and diagnostics
- Heme Oxygenase-1 and Carbon Monoxide
- Epigenetics and DNA Methylation
- Emergency and Acute Care Studies
- Pregnancy and preeclampsia studies
- Neonatal Health and Biochemistry
- Hematopoietic Stem Cell Transplantation
- Folate and B Vitamins Research
- Neurological Complications and Syndromes
- Palliative Care and End-of-Life Issues
- Metabolism and Genetic Disorders
- Homelessness and Social Issues
- Epilepsy research and treatment
- Blood donation and transfusion practices
- Adolescent and Pediatric Healthcare
University of Illinois Chicago
2015-2024
Pediatric Oncology Group
2022
University of Illinois Urbana-Champaign
2012-2022
Comer Children's Hospital
2011-2019
University of Illinois Hospital & Health Sciences System
2015-2017
National Institutes of Health
2004-2017
National Heart Lung and Blood Institute
2007-2017
Children's National
2009-2012
University of Illinois System
2012
National Institutes of Health Clinical Center
2006-2011
Oxidative stress contributes to the complex pathophysiology of sickle cell disease. Oral therapy with pharmaceutical-grade l-glutamine (USAN, glutamine) has been shown increase proportion reduced form nicotinamide adenine dinucleotides in erythrocytes, which probably reduces oxidative and could result fewer episodes cell–related pain.
Section:ChooseTop of pageAbstract <<ContentsOverviewIntroductionMethodsHow to Use These Guidelin...Definition PH in SCDDiagnosis SCDEstimating Mortality Risk...Treatment Patients wit...Future DirectionsReferencesCITING ARTICLES
The neurotoxic effects of cyclosporine therapy are well known but poorly understood. Imaging studies typically show subcortical edema predominantly affecting the posterior regions brain. We sought to determine causes for these findings by comparing radiographic data with various clinical parameters.In a 3-year period, 16 patients neurologic attributed were examined CT, MR imaging, or both. In most cases, imaging was performed both at onset syndrome and after it had resolved. evaluated...
A multicenter investigation of allogeneic bone marrow transplantation for children with sickle cell disease was conducted that included 27 European and North American transplant centers. Fifty-nine patients who ranged in age from 3.3 to 15.9 years (median, 10.1 years) received HLA-identical sibling allografts between September 1991 April 2000. Fifty-five survive, 50 survive free disease, a median follow-up 42.2 months (range, 11.8 115 months) after transplantation. Of the successful...
The authors evaluated education attainment and neuropsychological deficits in children with sickle cell disease (SCD) silent cerebral infarcts. Children infarcts had twice the rate of school difficulties as without Eighty percent infarct cases clinically significant cognitive deficits, whereas 35% academic skills. show high rates poor educational attainment, frontal lobe injury. Poor performance SCD is one indicator
Stroke occurs in 7–8% of children with Sickle Cell Disease (Hb SS) and is a major cause morbidity. Rates recurrence have been reduced from 46–90% to less than 10% through chronic blood transfusions. Prevention first stroke, however, would be preferable because even one stroke can irreversible brain injury. Transcranial Doppler (TCD) ultrasound detect arterial flow rates associated subsequent risk. By combining TCD screening potentially effective treatment, may prevented. The Trial Anemia...
Studies have shown that ovarian failure is a common side-effect of chemotherapy treatment; however, continuation regular menses post-treatment does not necessarily imply the ovaries escaped damage. This animal study measures directly primordial follicle (PMF) loss following exposure to and evaluates reproductive outcome significant destruction PMF population. Inbred Balb/c mice aged 5–6 weeks were administered different doses an alkylating agent, cyclophosphamide, total number remaining in...
This study assessed reproductive performance, fetal viability and teratogenicity in female mice exposed to cyclophosphamide across a timeline corresponding different stages of follicle maturation. Pregnancies were established Balb/c 1–4 weeks after administration non-sterilizing dose (75 mg/kg). Each mating group represented stage follicular growth at the time exposure. The number corpora lutea, pregnancies resorptions determined. Surviving fetuses evaluated for gross malformations. Results...
We conducted a prospective, multicenter investigation of human-leukocyte antigen (HLA) identical sibling bone marrow transplantation (BMT) in children with severe sickle cell disease (SCD) between 1991 and 2000. To determine if were protected from complications SCD after successful BMT, we extended our initial study BMT for to conduct assessments the central nervous system (CNS) pulmonary function 2 or more years transplantation. In addition, impact on gonadal was studied. After patients...
Allogeneic hematopoietic stem cell transplantation (HSCT) is rarely performed in adult patients with sickle disease (SCD). We utilized the chemotherapy-free, alemtuzumab/total body irradiation 300 cGy regimen sirolimus as post-transplantation immunosuppression 13 high-risk SCD between November 2011 and June 2014. Patients received matched related donor (MRD) granulocyte colony–stimulating factor–mobilized peripheral blood cells, including 2 cases that were ABO incompatible. Quality-of-life...
Background Sickle cell disease (SCD), a congenital hemolytic anemia that exacts terrible global morbidity and mortality, is driven by polymerization of mutated sickle hemoglobin (HbS) in red blood cells (RBCs). Fetal (HbF) interferes with this polymerization, but HbF epigenetically silenced from infancy onward DNA methyltransferase 1 (DNMT1). Methods findings To pharmacologically re-induce DNMT1 inhibition, first-in-human clinical trial (NCT01685515) combined 2 small molecules—decitabine to...
The cause of death in people affected by sickle cell disease (SCD) is often challenging to define as prior studies have used retrospective or administrative data for analysis. We a prospective longitudinal registry assess mortality and clinical co-morbidities among subjects enrolled the Sickle Cell Disease Implementation Consortium (SCDIC) registry. At enrollment, we collected following data: patient-reported demographics, SCD phenotype, baseline laboratory values, comorbidities, current...
<h3>Background</h3> A substantial minority of neurologically normal children with sickle cell disease have lesions consistent cerebral infarction as seen on magnetic resonance imaging (MRI). <h3>Objectives</h3> To determine if transfusion therapy affects the rate at which silent infarcts develop and to evaluate contribution MRI brain stroke prediction by transcranial Doppler (TCD) ultrasonography. <h3>Study Design</h3> Children elevated TCD ultrasonographic velocity were randomized receive...
Community health workers are increasingly recognized as useful for improving care and outcomes a variety of chronic conditions. can provide social support, navigation systems resources, lay counseling. Social cultural alignment community with the population they serve is an important aspect worker intervention. Although interventions have been shown to improve patient-centered in underserved communities, these not evaluated sickle cell disease. Evidence from other disease areas suggests that...
Sickle cell disease (SCD) is an inherited hemoglobinopathy that predominantly affects African Americans in the United States. The associated with complications leading to high healthcare utilization rates, including emergency department (ED) visits and hospitalizations. Optimal SCD care requires a multidisciplinary approach involving specialists ensure preventive care, minimize prevent unnecessary ED However, most individuals receive sub-optimal or are unaffiliated (have not seen...
To examine neurocognitive functioning in children classified with overt cerebral vascular accidents (CVAs), silent infarcts, or without central nervous system (CNS) pathology on magnetic resonance imaging.Participants were 63 and adolescents sickle cell disease (SCD).Children CVAs infarcts differed from their peers CNS measures of attention executive functioning.We consider these deficits the result high frequency frontal lobe incurred by SCD. Recommendations include use tests designed to...