A5028994533- Hemoglobinopathies and Related Disorders
- Iron Metabolism and Disorders
- Blood groups and transfusion
- Folate and B Vitamins Research
- Bone and Joint Diseases
- Neonatal Health and Biochemistry
- Blood donation and transfusion practices
- Prenatal Screening and Diagnostics
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Pharmacological Effects and Toxicity Studies
- Childhood Cancer Survivors' Quality of Life
- Erythrocyte Function and Pathophysiology
- Liver Disease Diagnosis and Treatment
- Protease and Inhibitor Mechanisms
- Child Nutrition and Water Access
- Renal and related cancers
- Hepatitis B Virus Studies
- Chronic Kidney Disease and Diabetes
- Erythropoietin and Anemia Treatment
- Tumors and Oncological Cases
- Platelet Disorders and Treatments
- Blood properties and coagulation
- Hematological disorders and diagnostics
- Adolescent and Pediatric Healthcare
- Acute Lymphoblastic Leukemia research
University of Miami
2016-2025
Jackson Memorial Hospital
2006-2024
University of Miami Health System
2022
L'Hôpital Universitaire Justinien
2018-2019
Pediatrics and Genetics
2019
Children's Hospital of Los Angeles
1990-2015
University of Miami Hospital
2013
Memorial Medical Center
2007-2012
Loma Linda University
1997-2000
Loma Linda University Medical Center
1997
The up-regulation of P-selectin in endothelial cells and platelets contributes to the cell–cell interactions that are involved pathogenesis vaso-occlusion sickle cell–related pain crises. safety efficacy crizanlizumab, an antibody against adhesion molecule P-selectin, were evaluated patients with cell disease.
Background Little is known about the national outcome of children and adults with sickle cell disease (SCD) given contemporary care. Procedure We investigated number deaths, standardized crude age-adjusted mortality rates, causes death among individuals SCD across United States during 1999–2009 according to certificates by using a publicly available website (http://wonder.cdc.gov/). Data were compared 1979–1998. Results When 1979–1998, significantly decreased 61% in infants <1 year age, 67%...
We evaluated 85 patients with serologic evidence <i>of Borrelia burgdorferi</i> infection. Manifestations included encephalopathy (41), neuropathy (27), meningitis (2), multiple sclerosis (MS) (6), and psychiatric disorders (3). performed lumbar punctures in 53, brain MRI 33, evoked potentials (EPs) 33. Only an MS-like illness had abnormal EPs, elevated IgG index, oligoclonal bands the cerebrospinal fluid. Twelve of 18 encephalopathy, meningitis, or focal CNS disease intrathecal synthesis...
Journal Article Inhibition of Collagenolytic Activity and Metastasis Tumor Cells by a Recombinant Human Tissue Inhibitor Metalloproteinases Get access Ofelia A. Alvarez, Alvarez Division Hematology-Oncology, Department Pediatrics, Children's Hospital Los Angeles the University Southern California School MedicineLos Angeles, CA Search for other works this author on: Oxford Academic PubMed Google Scholar David F. Carmichael, Carmichael Synergen, Inc.Boulder, CO Yves DeClerck * Correspondence...
Abstract Background Children with sickle cell anemia (SCA) often develop hyposthenuria and renal hyperfiltration at an early age, possibly contributing to the glomerular injury insufficiency commonly seen later in life. The Phase III randomized double‐blinded Clinical Trial of Hydroxyurea Infants SCA (BABY HUG) tested hypothesis that hydroxyurea can prevent kidney dysfunction by reducing hyperfiltration. Procedure 193 infants (mean age 13.8 months) received 20 mg/kg/day or placebo for 24...
Abstract The cell adhesion molecule P‐selectin plays a key role in the pathogenesis of vaso‐occlusive crisis (VOC) patients with sickle disease (SCD). In double‐blind, placebo‐controlled phase 2 SUSTAIN study, crizanlizumab (humanized, anti‐P‐selectin monoclonal antibody) 5 mg/kg significantly lowered rate VOC SCD by 45% vs placebo. SUSTAIN, were randomized to 2.5 mg/kg, or placebo intravenously 14 times over 52 weeks. primary endpoint was annual This post hoc descriptive analysis evaluated...
Importance Transitioning from pediatric to adult health care is crucial for the continuity of young adults with sickle cell disease. Among 5 hematology practices participating in Florida Pediatric Hematology Learning and Action Network, 3 lacked transition programs before this quality improvement project. Objective To evaluate implementation improve individuals transitioning Design, Setting, Participants This multicenter observational study was conducted at centers Florida. All patients...
Although hemoglobin SC (HbSC) disease is usually considered less severe than sickle cell anemia (SCA), which includes HbSS and HbS/β 0 ‐thalassemia genotypes, many patients with HbSC experience complications, including vaso‐occlusive pain, acute chest syndrome, avascular necrosis, retinopathy, poor quality of life. Fully 20 years after the clinical laboratory efficacy hydroxyurea was proven in adult SCA patients, safety utility treatment for remain unclear. Recent NHLBI evidence‐based...
Background Children with cancer experience pain related to the disease process, treatment, and associated procedures. For children leukemia, experienced after diagnosis has received scant attention. Objective To examine experience, management strategies, outcomes during first year of acute leukemia. Methods A longitudinal descriptive approach was used collect data at seven points from 95 English- Spanish-speaking children, ages 4 17 years, receiving care in one three southern California...
Abstract Background Stroke occurs in 5–10% of children with sickle cell anemia (SCA) and has a high (>50%) risk recurrence without therapy. Chronic monthly erythrocyte transfusions effectively prevent recurrent stroke, but their long‐term use is limited by serious side effects, including iron overload. An alternative to transfusion for secondary stroke prevention SCA needed, especially one that also improves the management Methods With Transfusions Changing Hydroxyurea (SWiTCH) an...
Summary Transcranial Doppler ( TCD ) With Transfusions Changing to Hydroxyurea TW i TCH trial is a randomized, open‐label comparison of hydroxycarbamide (also termed hydroxyurea) versus continued chronic transfusion therapy for primary stroke prevention in patients with sickle cell anaemia SCA and abnormal . Severity location iron overload an important secondary outcome measure. We report the baseline findings abdominal organ burden 121 participants. At enrollment, were young (9·8 ± 2·9...
Background Sickle cell disease (SCD) is a genetic that impacts patients' quality of life, healthcare costs, and life expectancy. Elevated sickle hemoglobin (HbS), which readily polymerizes, causes red blood sickling, leading to chronic hemolytic anemia complications often requiring hospitalization transfusions. In 2019, voxelotor, inhibits HbS polymerization, was approved for SCD treatment.Objectives This study uses real-world evidence assess voxelotor's effectiveness in patients typical...
The purpose of this study is to report the incidence pancreatitis in patients treated with pegaspargase our hospital during a 2-year period.We identified episodes related intramuscular administration 2,500 IU/m(2) for treatment childhood hematological malignancies period (May 1996-April 1998). Patients were evaluated clinically and by sequential serum amylase lipase determinations radiographic examinations. For comparison, who only received native Escherichia coli L-asparaginase examined...
Abstract Background Microalbuminuria (MA) is an early indicator for glomerulopathy in sickle cell disease (SCD). Procedure We reviewed the medical records of asymptomatic patients ages 4–20 with hemoglobinopathies, who were screened MA order to find out its prevalence and risk factors. Results Nineteen 120 (15.8%) had detected by spot urine (mean albumin absolute value 6.95 ± 0.56 mg/dl) abnormal creatinine ratios (79.8 0.62 mg/g creatinine). Twenty four‐hour collections confirmed 57% cases...
To compare the non-neurological events in children with sickle cell anemia (SCA) and previous stroke enrolled SWiTCH. The NHLBI-sponsored Phase III multicenter randomized clinical trial transfusions changing to hydroxyurea (SWiTCH) (ClinicalTrials.gov NCT00122980) compared continuation of chronic blood transfusion/iron chelation switching hydroxyurea/phlebotomy for secondary prevention management iron overload. All were included analysis (intention treat). Fisher's Exact test was used...
Although effective agents are available to prevent painful vaso-occlusive episodes of sickle cell disease (SCD), there no disease-modifying therapies for ongoing episodes; treatment remains supportive. A previous phase 3 trial poloxamer 188 reported shortened duration in SCD, particularly children and participants treated with hydroxyurea.To reassess the efficacy episodes.Phase 3, randomized, double-blind, placebo-controlled, multicenter, international conducted from May 2013 February 2016...
Abstract Background Albuminuria with normal serum creatinine occurs frequently in patients sickle cell disease (SCD), but the rate of progression to more advanced chronic renal is unknown. The purpose this study was investigate children and young adults SCD albuminuria over time. Procedure Urine albumin/creatinine (A/C) ratios were obtained serially. Serum cystatin C levels determined a subgroup 20 patients. Results Of 38 who had (30 microalbuminuria 8 proteinuria), 10.5% progressive during...