Brief Summary
'Sickle-shaped' anemia was first clinically described in the US in 1910, and the mutated heritable sickle hemoglobin molecule was identified in 1949. The pathophysiology of SCD is a consequence of abnormal polymerization of sickle hemoglobin (HbS) and its effects on red cell membrane properties, shape, and density, and subsequent critical changes in inflammatory cell and endothelial cell function. Our goal is to understand the impact of CMA abnormalities in SCD, by interrogating a number of recognized interactions in a range of clinical phenotypes.
To date, correlative studies in SCD, by us and others, have range between clinical reports, based on tests, interventions, and chart review of individuals or groups of individuals and, at the other extreme, identification of functional gene polymorphisms based on population studies. The investigators wish to augment these studies through a systematic examination of cellular membrane properties and activation status. Of hematologic disorders, SCD may be unusually susceptible to such an examination.
To date, correlative studies in SCD, by us and others, have range between clinical reports, based on tests, interventions, and chart review of individuals or groups of individuals and, at the other extreme, identification of functional gene polymorphisms based on population studies. The investigators wish to augment these studies through a systematic examination of cellular membrane properties and activation status. Of hematologic disorders, SCD may be unusually susceptible to such an examination.
Brief Title
Sickle Cell Disease Biofluid Chip Technology (SCD BioChip)
Detailed Description
Novel biofluidic chip technology can investigate surface characteristics that are typically measured with conventional techniques, such as fluorescent activated cell sorting (FACS), immunohistochemistry, or microscopic imaging methods. In FACS, cells of interest are isolated, extensively processed, incubated with a fluorescent-labeled antibody and sorted by optical recognition. In the proposed SCD biofluidic chip (SCD biochip), the interrogating antibody coats the microchannel surface and captures the cell population(s) of interest, without processing, incubation, or in vitro manipulation. The SCD biochip can also quantitate cellular adherence to experimental biological surfaces that are comprised of subcellular components. The SCD biochip is technically simple and experimentally flexible, whereby the population of interest is retained on the chip and quantitated in situ. The microchip system allows retrieval of viable isolated cells for potential downstream processing, analysis, and in vitro culture.
Categories
Central Contacts
Central Contact Role
Contact
Central Contact Phone
(216) 368-6447
Central Contact Email
umut@case.edu
Completion Date
Completion Date Type
Estimated
Conditions
Sickle Cell Disease
Eligibility Criteria
Inclusion Criteria
* Male or female ≥12 years of age at the time of consent (enrollment).
* Documentation Sickle Cell Disease, including HbSS or compound heterozygus HbSC- or HbSβ- thalassemia diagnosis as evidenced by one or more clinical features.
* Written informed consent (and assent when applicable) obtained from subject or subject's legal representative and ability for subject to comply with the requirements of the study.
Exclusion Criteria
* Presence of a condition or abnormality that in the opinion of the Investigator would compromise the safety of the patient or the quality of the data.
* Male or female ≥12 years of age at the time of consent (enrollment).
* Documentation Sickle Cell Disease, including HbSS or compound heterozygus HbSC- or HbSβ- thalassemia diagnosis as evidenced by one or more clinical features.
* Written informed consent (and assent when applicable) obtained from subject or subject's legal representative and ability for subject to comply with the requirements of the study.
Exclusion Criteria
* Presence of a condition or abnormality that in the opinion of the Investigator would compromise the safety of the patient or the quality of the data.
Inclusion Criteria
Inclusion Criteria
* Male or female ≥12 years of age at the time of consent (enrollment).
* Documentation Sickle Cell Disease, including HbSS or compound heterozygus HbSC- or HbSβ- thalassemia diagnosis as evidenced by one or more clinical features.
* Written informed consent (and assent when applicable) obtained from subject or subject's legal representative and ability for subject to comply with the requirements of the study.
* Male or female ≥12 years of age at the time of consent (enrollment).
* Documentation Sickle Cell Disease, including HbSS or compound heterozygus HbSC- or HbSβ- thalassemia diagnosis as evidenced by one or more clinical features.
* Written informed consent (and assent when applicable) obtained from subject or subject's legal representative and ability for subject to comply with the requirements of the study.
Gender
All
Gender Based
false
Keywords
Sickle Cell Disease
Biofluidic Chip Technology
Healthy Volunteers
No
Last Update Post Date
Last Update Post Date Type
Actual
Last Update Submit Date
Minimum Age
12 Years
NCT Id
NCT02824471
Org Class
Other
Org Full Name
University Hospitals Cleveland Medical Center
Org Study Id
05-14-07C
Overall Status
Recruiting
Primary Completion Date
Primary Completion Date Type
Estimated
Official Title
Sickle Cell Disease (SCD) Biochip': Towards a Simple and Reliable Way to Monitor Sickle Cell Disease
Primary Outcomes
Outcome Description
Red and white cell adhesion to biomolecules (e.g. laminin, fibronectin, and selectins) will be measured on a microfluidic device, the SCD Biochip. Adhesion will be measured under normal oxygen and low oxygen conditions. Adhesions will be assessed relative to clinical findings, such as hematology parameters, and evidence for vaso-occlusion, pain, inflammation, and vasculopathy in patients with sickle cell disease.endothelial and hematopoietic precursor cells based on membrane properties and adhesion.
Outcome Measure
Develop an SCD Biochip with which to examine key cellular properties and interactions, including RBC and WBC cellular, adhesive, and inflammatory properties, and circulating endothelial and hematopoietic precursor cell characteristics.
Outcome Time Frame
2 years
Secondary Outcomes
Outcome Description
The investigators will examine and validate clinical correlations with these cellular/membrane properties in larger populations of SCD, across a range of phenotypes, using our simple rapid flexible SCD Biochip platform.
Outcome Time Frame
2 years
Outcome Measure
Correlate SCD Biochip function in heterogeneous SCD populations, including HbSS and HbSC at a range of ages, and in those with acute and chronic complications and compared with normal controls.
Start Date
Start Date Type
Actual
Status Verified Date
First Post Date
First Post Date Type
Estimated
First Submit Date
First Submit QC Date
Study Population
Children (≥12 years old) and adults with SCD, including HbSS or compound heterozygus HbSC- or HbSβ- thalassemia will be offered participation. Control samples (HbAA) may be obtained from healthy volunteers.
Std Ages
Child
Adult
Older Adult
Locked Fields
Render the field
Maximum Age Number (converted to Years and rounded down)
999
Minimum Age Number (converted to Years and rounded down)
12
Investigators
Investigator Type
Principal Investigator
Investigator Name
Deepa Manwani
Investigator Email
dmanwani@montefiore.org
Investigator Phone
718-741-2342