PET imaging of upregulated VLA-4 in vaso-occlusive crisis using 64Cu-CB-TE1A1P-PEG4-LLP2A in a mouse model of sickle cell disease

Conclusion: Increased 64Cu-LLP2A uptake post LPS challenge in sickle (M21) mice, but not in control mice, suggests possible localization of the tracer in areas of vaso-occlusion. These results suggest a potential application of 64Cu-LLP2A in imaging vaso-occlusive events. Research support: This research was supported by a University of Pittsburgh Vascular Medicine Institute P3HVB grant. UPMC Hillman Cancer Center shared resources (In Vivo Imaging Facility) were supported in part by P30CA047904.
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Preclinical Probes for Cardiovascular, Endocrine & amp; Other I Source Type: research

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With the increase in availability of high depth whole genome sequencing (WGS) data of individuals with sickle cell disease (SCD), easy access to the raw sequencing data remains an issue due to technical and regulatory challenges. A compliant system that can provide facile data access would accelerate scientific discovery of genetic variants associated with clinical phenotypes. Cloud storage and computing provide an ultimate solution to this data access, which we have shown through the St. Jude Cloud (https://stjude.cloud) where over 5000 whole genome sequences for pediatric cancer patients are being shared in collaboration...
Source: Blood - Category: Hematology Authors: Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Sickle Cell Disease-Genomic, Gene Regulation, and Pain Mechanisms Source Type: research
Backgroundβ-globin gene transfer has the potential for substantial clinical benefit in patients with sickle cell disease (SCD). LentiGlobin Drug Product (DP) contains autologous CD34+ hematopoietic stem cells (HSCs) transduced with the BB305 lentiviral vector (LVV), encoding β-globin with an anti-sickling substitution (T87Q). The safety and efficacy of LentiGlobin gene therapy is being evaluated in the ongoing Phase 1 HGB-206 study (NCT02140554). Results in the initial 7 patients treated with LentiGlobin DP from steady state bone marrow harvested (BMH) HSCs using original DP manufacturing process (Group A) demons...
Source: Blood - Category: Hematology Authors: Tags: 801. Gene Therapy and Transfer: Gene Therapy for Blood Cell Disorders Source Type: research
Although sickle cell disease (SCD) is a monogenic disorder, the severity and specific organ dysfunction and failure are strongly influenced by genetic modifiers. Rapid identification of all modifiers in patients and well-phenotyped cohorts will better define the impact of relevant variants on clinical status, inform disease biology, and identify new therapeutic strategies. We created the Sickle Genome Project (SGP), a whole genome sequencing (WGS) strategy, to define genomic variation and modifiers of SCD. We performed WGS on 871 African American SCD patients from St. Jude Children's Research Hospital who participated in t...
Source: Blood - Category: Hematology Authors: Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster III Source Type: research
ConclusionHospitalization rates are rising among most age-groups of adults with SCD. The reasons for this finding are unclear but the rising rates may reflect the fragmentation of care for SCD in adults as well as age-related increases in pain-related comorbidities and SCD complications as SCD patients live longer. However, there has been no associated increase in-hospital SCD mortality, supporting extant data which suggest that the rate of opioid-related deaths in SCD is low, and the use of opioids for pain control may be considered relatively safe in the SCD population.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - Category: Hematology Authors: Tags: 901. Health Services Research-Non-Malignant Conditions: Sickle Cell Disease Source Type: research
Sickle cell disease is a genetic blood disorder with significant morbidity and mortality. This disease is characterized by vaso-occlusive pain crisis and end-organ damage, ultimately contributing to poor quality of life and reduced life expectancy. While many advances have been made in the management of sickle cell disease, there remains room for improvement in the delivery of care for these patients.The Lifespan Academic Medical Center is the largest health care organization in the state of Rhode Island. In January of 2018, a large cohort of patients was referred to Lifespan's Cancer Institute for treatment of sickle cell...
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical Source Type: research
This study was approved by the IRB at UHCMC. Within a 24-hour period, RBC adhesion to LN was quantitated by microscopy after passage of unprocessed whole blood through a LN-coated microfluidic adhesion assay, the SCD biochip [1]. Samples were analyzed for hemoglobin (Hb) phenotype by high-performance liquid chromatography (HPLC) in the clinical lab. Correlative clinical data, including, baseline lab values, and medical history, were obtained from the patients' medical records and used to characterize our results. Data from people with multiple samples were used as median values.Results:Blood samples from 19 unselected pati...
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research
Background: Vaso-occlusive crisis (VOC) is the hallmark complication of sickle cell disease (SCD). The majority of SCD-related healthcare costs in the United States, estimated at $2.4 billion annually, are attributed to frequent healthcare utilization due to recurrent VOC (1-3). Risk factors such as the prescription of nonsteroidal anti-inflammatory drugs (NSAIDs) only without opioids, older age, and steroid treatment have been identified to be associated with readmissions in pediatric SCD patients (4, 5), but limited data exist about potential predictors for readmission in adults (6). The impact of inpatient opioid utiliz...
Source: Blood - Category: Hematology Authors: Tags: 901. Health Services Research-Non-Malignant Conditions: Poster III Source Type: research
Pediatric Blood&Cancer, EarlyView.
Source: Pediatric Blood and Cancer - Category: Cancer & Oncology Authors: Source Type: research
Pediatric Blood&Cancer, EarlyView.
Source: Pediatric Blood and Cancer - Category: Cancer & Oncology Authors: Source Type: research
Pediatric Blood&Cancer, EarlyView.
Source: Pediatric Blood and Cancer - Category: Cancer & Oncology Authors: Source Type: research
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