ID

45561

Beskrivning

Principal Investigator: Richard A. Gibbs, PhD, Baylor College of Medicine, Houston, TX, USA MeSH: Anemia, Sickle Cell https://www.ncbi.nlm.nih.gov/projects/gap/cgi-bin/study.cgi?study_id=phs000691 Sickle cell disease (SCD) is a severe debilitating hematological disorder associated with a high degree of morbidity and mortality. There are approximately 200,000 babies born with sickle cell disease each year, with the disease predominately affecting individuals in Africa. The overall global burden of the disease is tremendous, with more than 100,000 patients currently in the US and further millions worldwide. The governing bodies of the World Health Organization have recently adopted a resolution to strengthen the response to sickle disease in all affected countries and there is a definite need for high quality sickle cell disease research that has the potential to improve the treatment and prognosis of patients with this devastating disease. The clinical manifestations of SCD arise from a complex pathophysiology that includes hemolysis, acute vaso-occlusion, endothelial dysfunction, inflammation, and chronic organ damage. While the individual clinical course of this disease is highly variable, many of the associated complications demonstrate some degree of heritability. Intensive research into identifying genetic modifiers that can affect the pathophysiology of SCD has been limited to date and there is an urgent need to improve of our knowledge the molecular mechanisms underlying the clinical complications of SCD. The Sickle cell CIP project is investigating complication of stroke and pharmacogenomics of hydroxyurea response in patients with sickle cell anemia. The major benefit of hydroxyurea comes from its ability to induce fetal hemoglobin (HbF) and higher HbF levels are associated with reduced morbidity and mortality in SCA patients. We will perform whole exome and whole genome sequencing of SCA patients in order to identify genome variants associated with incidences of stroke and HbF response to hydroxyurea.

Länk

dbGaP-study=phs000691

Nyckelord

  1. 2023-01-13 2023-01-13 - Chiara Middel
Rättsinnehavare

Richard A. Gibbs, PhD, Baylor College of Medicine, Houston, TX, USA

Uppladdad den

13 januari 2023

DOI

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Licens

Creative Commons BY 4.0

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dbGaP phs000691 CIP: Differential Response to Hydroxyurea and Incidence of Stroke in Sickle Cell Disease

Eligibility Criteria

Inclusion and exclusion criteria
Beskrivning

Inclusion and exclusion criteria

Alias
UMLS CUI [1,1]
C1512693
UMLS CUI [1,2]
C0680251
Inclusion Criteria: Patients with sickle cell anemia - Age between 3 years and 22 years inclusive - History of hydroxyurea use with evidence of drug related myelosupression, such as an absolute neutrophil count (ANC) of less than 4000/UL. Or patients with sickle cell anemia and a history of overt clinical stroke.
Beskrivning

Elig.phs000691.v2.p1.1

Datatyp

boolean

Alias
UMLS CUI [1,1]
C1512693
UMLS CUI [1,2]
C0002895
UMLS CUI [1,3]
C0001779
UMLS CUI [2,1]
C0262926
UMLS CUI [2,2]
C1524063
UMLS CUI [2,3]
C0020402
UMLS CUI [2,4]
C3887511
UMLS CUI [2,5]
C0458082
UMLS CUI [2,6]
C0854467
UMLS CUI [2,7]
C0948762
UMLS CUI [3,1]
C0002895
UMLS CUI [3,2]
C0262926
UMLS CUI [3,3]
C5400991
Exclusion Criteria: Any subject who does not meet the inclusion criteria.
Beskrivning

Elig.phs000691.v2.p1.2

Datatyp

boolean

Alias
UMLS CUI [1,1]
C0680251
UMLS CUI [1,2]
C5236167

Similar models

Eligibility Criteria

Name
Typ
Description | Question | Decode (Coded Value)
Datatyp
Alias
Item Group
Inclusion and exclusion criteria
C1512693 (UMLS CUI [1,1])
C0680251 (UMLS CUI [1,2])
Elig.phs000691.v2.p1.1
Item
Inclusion Criteria: Patients with sickle cell anemia - Age between 3 years and 22 years inclusive - History of hydroxyurea use with evidence of drug related myelosupression, such as an absolute neutrophil count (ANC) of less than 4000/UL. Or patients with sickle cell anemia and a history of overt clinical stroke.
boolean
C1512693 (UMLS CUI [1,1])
C0002895 (UMLS CUI [1,2])
C0001779 (UMLS CUI [1,3])
C0262926 (UMLS CUI [2,1])
C1524063 (UMLS CUI [2,2])
C0020402 (UMLS CUI [2,3])
C3887511 (UMLS CUI [2,4])
C0458082 (UMLS CUI [2,5])
C0854467 (UMLS CUI [2,6])
C0948762 (UMLS CUI [2,7])
C0002895 (UMLS CUI [3,1])
C0262926 (UMLS CUI [3,2])
C5400991 (UMLS CUI [3,3])
Elig.phs000691.v2.p1.2
Item
Exclusion Criteria: Any subject who does not meet the inclusion criteria.
boolean
C0680251 (UMLS CUI [1,1])
C5236167 (UMLS CUI [1,2])

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