The purpose of this acquisition is to establish a centralized laboratory to characterize and catalogue autopsy and surgical specimens and correlate pathological findings with clinical data to determine more precisely the causes and risk factors related to mortality in patients with sickle cell disease. All autopsy and surgical specimens from the present Cooperative Study of Sickle Cell Disease (CSSCD) and other studies supported by the Sickle Cell Disease Branch such as: Comprehensive Sickle Cell Center collaborative studies (MRI, Transfusion) etc.; Hydroxyurea Collaborative Study; and the Prophylactic Penicillin Collaborative Study will be analyzed by the Centralized Pathology Unit. The Centralized Pathology Unit will have responsibility to finalize, reproduce, and distribute, to all clinical centers, standardized protocols and forms for the collection of autopsy and surgical samples. Other responsibilities include: 1. To assure compliance of all the clinical centers to the standardized protocols developed by the involved Contractor. 2. Receive and process all surgical and autopsy samples. 3. Perform biochemical and enzyme assays on tissue samples, when appropriate. 4. Provide routine and specialized staining of tissue, as required. 5 Examine tissue specimens using routine or electron microscopy. Tissues requiring special characterization shall be identified by the contractor prior to surgery or autopsy. 6. Conduct characterization, cataloging and interpretation of all samples in relationship to pathology, vascular changes and morphology. 7. Establishment of a registry of slides, of all surgical and autopsy specimens for: CSSCD participants, collaborative, and other studies supported by the Sickle Cell disease Branch. 8. To establish the cause of death, for the previous and new deaths in the CSSCD, collaborative and other studies supported by the sickle Cell Disease Branch. The completion of these objectives will greatly impact on achieving the ultimate goal which is to accurately access the causes and risks factors that contribute to mortality in patients with sickle cell disease.

Agency
National Institute of Health (NIH)
Institute
Division of Blood Diseases And Resources (NHLBI)
Type
Research and Development Contracts (N01)
Project #
N01HB007086-005
Application #
2312536
Study Section
Project Start
1990-09-30
Project End
1995-09-29
Budget Start
1994-06-03
Budget End
1995-09-29
Support Year
Fiscal Year
1994
Total Cost
Indirect Cost
Name
University of South Alabama
Department
Pathology
Type
Schools of Medicine
DUNS #
City
Mobile
State
AL
Country
United States
Zip Code
36688
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Manci, Elizabeth A; Culberson, Donald E; Gardner, Julia M et al. (2004) Perivascular fibrosis in the bone marrow in sickle cell disease. Arch Pathol Lab Med 128:634-9
Manci, Elizabeth A; Culberson, Donald E; Yang, Yih-Ming et al. (2003) Causes of death in sickle cell disease: an autopsy study. Br J Haematol 123:359-65
Kean, Leslie S; Manci, Elizabeth A; Perry, Jennifer et al. (2003) Chimerism and cure: hematologic and pathologic correction of murine sickle cell disease. Blood 102:4582-93
Mvumbi, L; Manci, E A; Ulmer, R D et al. (1998) Decreased placental and umbilical cord glycogen levels associated with meconium-stained amniotic fluid. Placenta 19:295-9
Ballas, S K; Pindzola, A; Chang, C D et al. (1998) Postmortem diagnosis of hemoglobin SC disease complicated by fat embolism. Ann Clin Lab Sci 28:144-9
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Tuohy, A M; McKie, V; Manci, E A et al. (1997) Internal carotid artery occlusion in a child with sickle cell disease: case report and immunohistochemical study. J Pediatr Hematol Oncol 19:455-8