Cooperative Study of Sickle Cell Disease (CSSCD) - Catalog
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Name
Cooperative Study of Sickle Cell Disease (CSSCD)
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Accession Number
HLB00380408a
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Acronym
CSSCD
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Related studies
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BSI Study IDs
CSS
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Is public use dataset
False
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Keywords
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Ingestion StatusReleased
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Has Study Datasets
True
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Has Specimens
True
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Specimen ID TypeCoded
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Study Website
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The Framingham Heart Study Group requires that the requestor must obtain full or expedited IRB/Ethics Committee review and approval to obtain these data. Waivers or a determination that the research is exempt from ethical regulations do not suffice.
False
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Clinical Trial URLs
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Study typeEpidemiology Study
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Collection TypeOpen BioLINCC Study
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Cohort typeBoth
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Interventions
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Study Open Date (Data)
2009-10-01
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Study Open Date (Specimens)
2011-03-02
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Date materials available
2008-10-13
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Last updated
None
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Study period
1977-1995
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Study Contacts
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NHLBI Division
DBDR
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ClassificationBlood Disease
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HIV study classificationnon-HIV
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COVID study classificationnon-COVID
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Pre-Website # of Specimens Shipped
6148
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# of Returned Specimens
0
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Primary Publication URLs
N/A
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Commercial use data restrictionsNo
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Data restrictions based on area of researchNo
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Commercial use specimen restrictionsNo
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Non-genetic use specimen restrictions based on area of useNo
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Genetic use of specimens allowed?Yes
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Genetic use area of research restrictionsNo
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Specific Consent Restrictions
None.
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ConditionsAnemia, Sickle Cell
Blood Disease
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Objectives
The Cooperative Study of Sickle Cell Disease was initiated in 1977 to determine the natural history of sickle cell disease (SCD) from birth to death in order to identify those factors contributing to the morbidity and mortality of the disease. Specific objectives include: 1) to study the effect of sickle cell disease on growth and development from birth through adolescence 2) to study the conditions or events that may be related to the onset of painful crises 3) to obtain data on the nature, duration, and outcome of major complications of SCD 4) determine the nature, prevalence, and age- related incidence of organ damage due to SCD, and 5) study the role of SCD and its interaction with selected health events.
Phases 2 and 3 of the study involved followup of the infant cohort. A total of 709 infants (age less than 6 months) were enrolled during Phase 1 of the Cooperative Study of Sickle Cell Disease (CSSCD), and Phases 2 and 3 of the CSSCD was designed to follow these children for an additional 10 years. The study objectives included: 1) define prospectively the natural history of sickle cell disease; 2) determine the relationships between cognitive and academic functioning and brain status as determined by MRI; 3) determine the cognitive or behavioral markers of silent infarct; 4) determine the relationship of family functioning on the Family Environment Scale (FES) to brain status, cognitive functioning, and social and demographic factors; 5) continue studies that will enhance the state of knowledge on the influence of sickle cell disease on the psychosocial adjustment of children and adolescents.
Phase 2A of the study sought to examine the progression of organ damage in the heart, lung, kidney and liver in adult cohort patients (born before 1/1/56) enrolled in phase 1 of the study between 3/79 and 5/81. A total of 620 patients from 11 centers were eligible for phase 2A.
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Background
Sickle cell disease is a single-gene defect that results in sickle-shaped red blood cells. Although the manifestations of sickle cell disease have been described, variations in the severity and number of manifestations, as well as interactions with other health events, leads to significant gaps in the understanding of the natural history of the disorder. For example, impairments to renal, cardiac, and pulmonary organ function are known to occur in sickle cell patients; however, the descriptions of these outcomes was generally retrospective in nature and occurred when organ damage was severe. In addition, only limited data existed on the social, economic, educational, vocational, and psychological adjustment of patients and families, and as with any chronic disease, impediments to sickle cell patients achieving their educational and vocational goals needs to be elucidated.
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Participants
CSSCD was a multicenter, prospective study on the natural history of sickle cell disease and participant enrollment into Phase 1 of the CSSCD began in 1978. Participant entry ended in 1981 for all patients greater than six months of age; however, infants continued to be enrolled until 1988. Both mild and hospital-based sickle cell patients were recruited. A total of 4,085 participants, ranging in age from newborns to adults, were enrolled in Phase 1 from 23 centers across the US. Data collection for phase 1 of the CSSCD ended in 1988.
For Phases 2 and 3, 450 were enrolled in the Phase 2 followup along with 17 children that were between 6 and 10 months of age at the time of Phase 1 enrollment. Phase 2 followup concluded in 1994 and 378 patients continued to be followed in the Phase 3 continuation. Data collection ended in 1998.
A total of 359 adult phase 1 participants were enrolled in phase 2A of the study between September of 1989 and July 1991. Exit visits began approximately 2 years later and were concluded in September of 1993.
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Design
In phase 1, four protocols were developed according to participant age: Newborn to six months, pediatric (6 months to 10 years of age), adolescent (age 10-19 years) and adult (age 20+). Protocols were designed to collect similar data at similar times. Participants underwent a baseline exam for assessment of demographics, prior medical history, lab assessments, and clinical data. Post baseline data included routine follow-up examinations, measures of organ damage, and collection of acute and chronic complications.
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Conclusions
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Disease classification
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Publications
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Mat typesDNA
Serum
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Network
The study population available in BioLINCC study data may be lower than total study enrollment due to Informed Consent restrictions and other factors.
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Subjects
Newborn with control: 76
Newborn without control: 557
Pediatric <2: 292
Pediatric >=2: 928
Adolescent: 1038
Adult: 1194
Last Modified: May 24, 2024, 11:42 a.m. -
Age
Newborn with Control
Newborn without Control
Pediatric < 2
Pediatric >= 2
Adolescent
Adult
Total Subjects
0
76
557
171
804
1
121
9
130
2-5
476
476
6-10
443
116
559
11-15
549
549
16-20
373
82
455
21-25
376
376
26-30
319
319
31-35
181
181
36-40
96
96
41-45
54
54
46-50
33
33
51-55
20
20
>55
33
33
Last Modified: Aug. 21, 2025, 10:27 a.m. -
Sex
Newborn with Control
Newborn without Control
Pediatric < 2
Pediatric >= 2
Adolescent
Adult
Total Subjects
Female
36
267
137
440
517
691
2088
Male
40
290
155
488
521
503
1997
Last Modified: Aug. 21, 2025, 10:07 a.m. -
Race
Total Subjects
Missing w/Reason
13
Missing w/o Reason
1
Black
3974
Other
97
Last Modified: Aug. 21, 2025, 11:40 a.m.
Please note that biospecimen availability is subject to review by the NHLBI, BioLINCC, and the NHLBI Biorepository. Certain biospecimens may not be made available for your request. Section 3.0 of the BioLINCC Handbook describes the components of the review process.
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Material Types
Last Modified: Nov. 30, 2015, 1:27 p.m. -
General Freeze/Thaw Status
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Visits (Vials)
21 August 2025
Serum
DNA
Total Vials
Visit 1
904
.
904
Visit 2
412
.
412
Visit 3
31
.
31
Unknown
157
782
939
Last Modified: Aug. 21, 2025, 9:59 a.m. -
Visits (Subjects)
21 August 2025
Serum
Total number of subjects
Average volume (mL) per subject
Visit 1
309
2.97
Visit 2
153
2.68
Visit 3
15
1.59
Unknown
58
2.56
DNA
Total number of subjects
Average Mass (ug) per subject
Average vials per subject
Unknown
114
196.00
6.86
Last Modified: Aug. 21, 2025, 10 a.m.