Cooperative Study of The Clinical Course of Sickle Cell Disease

NCT00005277 | Completed

• 1 other identifier: 1500
• Study Type: observational
• Target: N/A
• Locations: 0 countries
• Sites: N/A

Brief Summary

To determine the natural history of sickle cell disease from birth to death in order to identify those factors contributing to the morbidity and mortality of the disease.

Conditions

Anemia, Sickle Cell; Blood Disease

Eligibility Criteria

• Age: Up to 25 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64)

Black males and females with sickle cell disease

Contact the study team to discuss eligibility requirements. They can help determine if this study is right for you.

Sponsors & Collaborators

• National Heart, Lung, and Blood Institute (NHLBI): lead

Related Publications (38)

• Milner PF, Kraus AP, Sebes JI, Sleeper LA, Dukes KA, Embury SH, Bellevue R, Koshy M, Moohr JW, Smith J. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991 Nov 21;325(21):1476-81. doi: 10.1056/NEJM199111213252104.

  PMID: 1944426 BACKGROUND

• Platt OS, Thorington BD, Brambilla DJ, Milner PF, Rosse WF, Vichinsky E, Kinney TR. Pain in sickle cell disease. Rates and risk factors. N Engl J Med. 1991 Jul 4;325(1):11-6. doi: 10.1056/NEJM199107043250103.

  PMID: 1710777 BACKGROUND

• Vichinsky EP, Earles A, Johnson RA, Hoag MS, Williams A, Lubin B. Alloimmunization in sickle cell anemia and transfusion of racially unmatched blood. N Engl J Med. 1990 Jun 7;322(23):1617-21. doi: 10.1056/NEJM199006073222301.

  PMID: 2342522 BACKGROUND

• Rosse WF, Gallagher D, Kinney TR, Castro O, Dosik H, Moohr J, Wang W, Levy PS. Transfusion and alloimmunization in sickle cell disease. The Cooperative Study of Sickle Cell Disease. Blood. 1990 Oct 1;76(7):1431-7.

  PMID: 2207318 BACKGROUND

• Gaston M, Rosse WF. The cooperative study of sickle cell disease: review of study design and objectives. Am J Pediatr Hematol Oncol. 1982 Summer;4(2):197-201.

  PMID: 7114401 BACKGROUND

• Steinberg MH, Rosenstock W, Coleman MB, Adams JG, Platica O, Cedeno M, Rieder RF, Wilson JT, Milner P, West S. Effects of thalassemia and microcytosis on the hematologic and vasoocclusive severity of sickle cell anemia. Blood. 1984 Jun;63(6):1353-60.

  PMID: 6722353 BACKGROUND

• Platt OS, Rosenstock W, Espeland MA. Influence of sickle hemoglobinopathies on growth and development. N Engl J Med. 1984 Jul 5;311(1):7-12. doi: 10.1056/NEJM198407053110102.

  PMID: 6727978 BACKGROUND

• Farber MD, Koshy M, Kinney TR. Cooperative Study of Sickle Cell Disease: Demographic and socioeconomic characteristics of patients and families with sickle cell disease. J Chronic Dis. 1985;38(6):495-505. doi: 10.1016/0021-9681(85)90033-5.

  PMID: 4008590 BACKGROUND

• Embury SH, Gholson MA, Gillette P, Rieder RF. The leftward deletion alpha-thal-2 haplotype in a black subject with hemoglobin SS. Blood. 1985 Mar;65(3):769-71.

  PMID: 3971048 BACKGROUND

• Pearson HA, Gallagher D, Chilcote R, Sullivan E, Wilimas J, Espeland M, Ritchey AK. Developmental pattern of splenic dysfunction in sickle cell disorders. Pediatrics. 1985 Sep;76(3):392-7.

  PMID: 2412200 BACKGROUND

• Zarkowsky HS, Gallagher D, Gill FM, Wang WC, Falletta JM, Lande WM, Levy PS, Verter JI, Wethers D. Bacteremia in sickle hemoglobinopathies. J Pediatr. 1986 Oct;109(4):579-85. doi: 10.1016/s0022-3476(86)80216-5.

  PMID: 3531449 BACKGROUND

• Gaston MH, Verter JI, Woods G, Pegelow C, Kelleher J, Presbury G, Zarkowsky H, Vichinsky E, Iyer R, Lobel JS, et al. Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial. N Engl J Med. 1986 Jun 19;314(25):1593-9. doi: 10.1056/NEJM198606193142501.

  PMID: 3086721 BACKGROUND

• Espeland M. Estimation of growth curves from longitudinal data collected at irregular time intervals. Comput Biomed Res. 1986 Dec;19(6):575-87. doi: 10.1016/0010-4809(86)90031-5.

  PMID: 3791979 BACKGROUND

• Gaston M, Smith J, Gallagher D, Flournoy-Gill Z, West S, Bellevue R, Farber M, Grover R, Koshy M, Ritchey AK, et al. Recruitment in the Cooperative Study of Sickle Cell Disease (CSSCD). Control Clin Trials. 1987 Dec;8(4 Suppl):131S-140S. doi: 10.1016/0197-2456(87)90016-x.

  PMID: 3440386 BACKGROUND

• Steinberg MH, West MS, Gallagher D, Mentzer W. Effects of glucose-6-phosphate dehydrogenase deficiency upon sickle cell anemia. Blood. 1988 Mar;71(3):748-52.

  PMID: 3345344 BACKGROUND

• Koshy M, Entsuah R, Koranda A, Kraus AP, Johnson R, Bellvue R, Flournoy-Gill Z, Levy P. Leg ulcers in patients with sickle cell disease. Blood. 1989 Sep;74(4):1403-8.

  PMID: 2475188 BACKGROUND

• Lee PC. Consistent collimator overlaps in field matching with computer-controlled x-ray collimators. Med Dosim. 1997 Spring;22(1):59-61. doi: 10.1016/s0958-3947(96)00149-5.

  PMID: 9136110 BACKGROUND

• Vichinsky EP, Styles LA, Colangelo LH, Wright EC, Castro O, Nickerson B. Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease. Blood. 1997 Mar 1;89(5):1787-92.

  PMID: 9057664 BACKGROUND

• Kinney TR, Sleeper LA, Wang WC, Zimmerman RA, Pegelow CH, Ohene-Frempong K, Wethers DL, Bello JA, Vichinsky EP, Moser FG, Gallagher DM, DeBaun MR, Platt OS, Miller ST. Silent cerebral infarcts in sickle cell anemia: a risk factor analysis. The Cooperative Study of Sickle Cell Disease. Pediatrics. 1999 Mar;103(3):640-5. doi: 10.1542/peds.103.3.640.

  PMID: 10049969 BACKGROUND

• Armstrong FD, Thompson RJ Jr, Wang W, Zimmerman R, Pegelow CH, Miller S, Moser F, Bello J, Hurtig A, Vass K. Cognitive functioning and brain magnetic resonance imaging in children with sickle Cell disease. Neuropsychology Committee of the Cooperative Study of Sickle Cell Disease. Pediatrics. 1996 Jun;97(6 Pt 1):864-70.

  PMID: 8657528 BACKGROUND

• Moser FG, Miller ST, Bello JA, Pegelow CH, Zimmerman RA, Wang WC, Ohene-Frempong K, Schwartz A, Vichinsky EP, Gallagher D, Kinney TR. The spectrum of brain MR abnormalities in sickle-cell disease: a report from the Cooperative Study of Sickle Cell Disease. AJNR Am J Neuroradiol. 1996 May;17(5):965-72.

  PMID: 8733975 BACKGROUND

• Koshy M, Weiner SJ, Miller ST, Sleeper LA, Vichinsky E, Brown AK, Khakoo Y, Kinney TR. Surgery and anesthesia in sickle cell disease. Cooperative Study of Sickle Cell Diseases. Blood. 1995 Nov 15;86(10):3676-84.

  PMID: 7579333 BACKGROUND

• Smith JA, Espeland M, Bellevue R, Bonds D, Brown AK, Koshy M. Pregnancy in sickle cell disease: experience of the Cooperative Study of Sickle Cell Disease. Obstet Gynecol. 1996 Feb;87(2):199-204. doi: 10.1016/0029-7844(95)00367-3.

  PMID: 8559523 BACKGROUND

• Covitz W, Espeland M, Gallagher D, Hellenbrand W, Leff S, Talner N. The heart in sickle cell anemia. The Cooperative Study of Sickle Cell Disease (CSSCD). Chest. 1995 Nov;108(5):1214-9. doi: 10.1378/chest.108.5.1214.

  PMID: 7587419 BACKGROUND

• Gill FM, Sleeper LA, Weiner SJ, Brown AK, Bellevue R, Grover R, Pegelow CH, Vichinsky E. Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease. Blood. 1995 Jul 15;86(2):776-83.

  PMID: 7606007 BACKGROUND

• Brown AK, Sleeper LA, Miller ST, Pegelow CH, Gill FM, Waclawiw MA. Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease. Arch Pediatr Adolesc Med. 1994 Aug;148(8):796-804. doi: 10.1001/archpedi.1994.02170080026005.

  PMID: 7519102 BACKGROUND

• Castro O, Brambilla DJ, Thorington B, Reindorf CA, Scott RB, Gillette P, Vera JC, Levy PS. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. 1994 Jul 15;84(2):643-9.

  PMID: 7517723 BACKGROUND

• Wang WC, Grover R, Gallagher D, Espeland M, Fandal A. Developmental screening in young children with sickle cell disease. Results of a cooperative study. Am J Pediatr Hematol Oncol. 1993 Feb;15(1):87-91. doi: 10.1097/00043426-199302000-00011.

  PMID: 7680549 BACKGROUND

• West MS, Wethers D, Smith J, Steinberg M. Laboratory profile of sickle cell disease: a cross-sectional analysis. The Cooperative Study of Sickle Cell Disease. J Clin Epidemiol. 1992 Aug;45(8):893-909. doi: 10.1016/0895-4356(92)90073-v.

  PMID: 1624972 BACKGROUND

• Wang WC, Gallagher DM, Pegelow CH, Wright EC, Vichinsky EP, Abboud MR, Moser FG, Adams RJ. Multicenter comparison of magnetic resonance imaging and transcranial Doppler ultrasonography in the evaluation of the central nervous system in children with sickle cell disease. J Pediatr Hematol Oncol. 2000 Jul-Aug;22(4):335-9. doi: 10.1097/00043426-200007000-00010.

  PMID: 10959904 BACKGROUND

• Burlew K, Telfair J, Colangelo L, Wright EC. Factors that influence adolescent adaptation to sickle cell disease. J Pediatr Psychol. 2000 Jul-Aug;25(5):287-99. doi: 10.1093/jpepsy/25.5.287.

  PMID: 10880059 BACKGROUND

• Thompson RJ Jr, Armstrong FD, Kronenberger WG, Scott D, McCabe MA, Smith B, Radcliffe J, Colangelo L, Gallagher D, Islam S, Wright E. Family functioning, neurocognitive functioning, and behavior problems in children with sickle cell disease. J Pediatr Psychol. 1999 Dec;24(6):491-8. doi: 10.1093/jpepsy/24.6.491.

  PMID: 10608100 BACKGROUND

• Miller ST, Macklin EA, Pegelow CH, Kinney TR, Sleeper LA, Bello JA, DeWitt LD, Gallagher DM, Guarini L, Moser FG, Ohene-Frempong K, Sanchez N, Vichinsky EP, Wang WC, Wethers DL, Younkin DP, Zimmerman RA, DeBaun MR; Cooperative Study of Sickle Cell Disease. Silent infarction as a risk factor for overt stroke in children with sickle cell anemia: a report from the Cooperative Study of Sickle Cell Disease. J Pediatr. 2001 Sep;139(3):385-90. doi: 10.1067/mpd.2001.117580.

  PMID: 11562618 BACKGROUND

• Wang W, Enos L, Gallagher D, Thompson R, Guarini L, Vichinsky E, Wright E, Zimmerman R, Armstrong FD; Cooperative Study of Sickle Cell Disease. Neuropsychologic performance in school-aged children with sickle cell disease: a report from the Cooperative Study of Sickle Cell Disease. J Pediatr. 2001 Sep;139(3):391-7. doi: 10.1067/mpd.2001.116935.

  PMID: 11562619 BACKGROUND

• Thompson RJ Jr, Armstrong FD, Link CL, Pegelow CH, Moser F, Wang WC. A prospective study of the relationship over time of behavior problems, intellectual functioning, and family functioning in children with sickle cell disease: a report from the Cooperative Study of Sickle Cell Disease. J Pediatr Psychol. 2003 Jan-Feb;28(1):59-65. doi: 10.1093/jpepsy/28.1.59.

  PMID: 12490632 BACKGROUND

• Pegelow CH, Macklin EA, Moser FG, Wang WC, Bello JA, Miller ST, Vichinsky EP, DeBaun MR, Guarini L, Zimmerman RA, Younkin DP, Gallagher DM, Kinney TR. Longitudinal changes in brain magnetic resonance imaging findings in children with sickle cell disease. Blood. 2002 Apr 15;99(8):3014-8. doi: 10.1182/blood.v99.8.3014.

  PMID: 11929794 BACKGROUND

• Manci EA, Culberson DE, Yang YM, Gardner TM, Powell R, Haynes J Jr, Shah AK, Mankad VN; Investigators of the Cooperative Study of Sickle Cell Disease. Causes of death in sickle cell disease: an autopsy study. Br J Haematol. 2003 Oct;123(2):359-65. doi: 10.1046/j.1365-2141.2003.04594.x.

  PMID: 14531921 BACKGROUND

• Hsu LL, Miller ST, Wright E, Kutlar A, McKie V, Wang W, Pegelow CH, Driscoll C, Hurlet A, Woods G, Elsas L, Embury S, Adams RJ; Stroke Prevention Trial (STOP) and the Cooperative Study of Sickle Cell Disease (CSSCD). Alpha Thalassemia is associated with decreased risk of abnormal transcranial Doppler ultrasonography in children with sickle cell anemia. J Pediatr Hematol Oncol. 2003 Aug;25(8):622-8. doi: 10.1097/00043426-200308000-00007.

  PMID: 12902915 BACKGROUND

MeSH Terms

Conditions

Anemia, Sickle Cell; Hematologic Diseases

Condition Hierarchy (Ancestors)

Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Anemia; Hemic and Lymphatic Diseases; Hemoglobinopathies; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Paul Levy

  University of Illinois at Chicago

Study Design

• Study Type: observational
• Sponsor Type: NIH

Study Record Dates

• First Submitted: May 25, 2000
• First Posted: May 26, 2000
• Study Start: September 1, 1977
• Study Completion: December 1, 2000
• Last Updated: June 24, 2005

Record last verified: 2004-08