Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

Author:

DeBaun Michael R.1,Sarnaik Sharada A.2,Rodeghier Mark J.3,Minniti Caterina P.4,Howard Thomas H.5,Iyer Rathi V.6,Inusa Baba7,Telfer Paul T.8,Kirby-Allen Melanie9,Quinn Charles T.10,Bernaudin Françoise11,Airewele Gladstone12,Woods Gerald M.13,Panepinto Julie Ann14,Fuh Beng15,Kwiatkowski Janet K.16,King Allison A.17,Rhodes Melissa M.18,Thompson Alexis A.19,Heiny Mark E.20,Redding-Lallinger Rupa C.21,Kirkham Fenella J.22,Sabio Hernan23,Gonzalez Corina E.24,Saccente Suzanne L.25,Kalinyak Karen A.10,Strouse John J.26,Fixler Jason M.27,Gordon Mae O.28,Miller J. Phillip29,Noetzel Michael J.30,Ichord Rebecca N.31,Casella James F.26

Affiliation:

1. Department of Pediatrics, Division of Hematology/Oncology, Vanderbilt University, Nashville, TN;

2. Department of Pediatrics, Division of Hematology/Oncology, Wayne State University, Detroit, MI;

3. Rodeghier Consultants, Chicago, IL;

4. National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD;

5. Department of Pediatrics, Division of Hematology/Oncology, University of Alabama at Birmingham, Birmingham, AL;

6. Department of Pediatrics, Division of Hematology/Oncology, University of Mississippi Medical Center, Jackson, MS;

7. Department of Paediatrics, Evelina Children's Hospital, St Thomas' Hospital National Health Service Trust, London, United Kingdom;

8. Department of Pediatric Hematology, Royal London Hospital, London, United Kingdom;

9. Hospital for Sick Children, University of Toronto, Toronto, ON;

10. Department of Pediatrics, Hematology/Oncology, Cincinnati Children's Hospital Medical Center and University of Cincinnati, Cincinnati, OH;

11. Department Pediatrie, Hopital Intercommunal de Creteil, Creteil, France;

12. Department of Pediatrics, Hematology/Oncology, Baylor College of Medicine, Houston, TX;

13. Department of Pediatrics, Hematology/Oncology, University of Missouri–Kansas City, Kansas City, MO;

14. Department of Pediatrics, Hematology/Oncology, Medical College of Wisconsin, Milwaukee, WI;

15. Department of Pediatrics, Hematology/Oncology, Brody School of Medicine, Greenville, NC;

16. Department of Pediatrics, Hematology/Oncology, University of Pennsylvania, Children's Hospital of Philadelphia, Philadelphia, PA;

17. Department of Pediatrics, Hematology/Oncology, Washington University School of Medicine, St Louis, MO;

18. Department of Pediatrics, Hematology/Oncology, The Ohio State University, Columbus, OH;

19. Department of Pediatrics, Hematology/Oncology, Northwestern University, Chicago, IL;

20. Department of Pediatrics, Hematology/Oncology, Indiana University–Purdue University Indiana, Indianapolis, IN;

21. Department of Pediatrics, Hematology/Oncology, University of North Carolina at Chapel Hill, Chapel Hill, NC;

22. Neurosciences Unit, Institute of Child Health (University College London), London, United Kingdom;

23. Department of Pediatrics, Hematology/Oncology, Wake Forest University Health Sciences, Winston-Salem, NC;

24. Department of Pediatrics, Hematology/Oncology, Georgetown University Hospital, Washington, DC;

25. Department of Pediatrics, Hematology/Oncology, University of Arkansas Medical Sciences, Little Rock, AR;

26. Department of Pediatrics, Hematology/Oncology, Johns Hopkins University School of Medicine, Baltimore, MD;

27. Department of Pediatrics, Hematology/Oncology, Sinai Hospital, Baltimore, MD;

28. Department of Ophthalmology and Visual Sciences, Biostatistics, Washington University School of Medicine, St Louis, MO;

29. Division of Biostatistics, Washington University School of Medicine, St Louis, MO;

30. Departments of Neurology and Pediatrics, Washington University School of Medicine, St Louis, MO; and

31. Department of Pediatrics, Neurology, University of Pennsylvania, Children's Hospital of Philadelphia, Philadelphia, PA

Abstract

AbstractThe most common form of neurologic injury in sickle cell anemia (SCA) is silent cerebral infarction (SCI). In the Silent Cerebral Infarct Multi-Center Clinical Trial, we sought to identify risk factors associated with SCI. In this cross-sectional study, we evaluated the clinical history and baseline laboratory values and performed magnetic resonance imaging of the brain in participants with SCA (HbSS or HbSβ° thalassemia) between the ages of 5 and 15 years with no history of overt stroke or seizures. Neuroradiology and neurology committees adjudicated the presence of SCI. SCIs were diagnosed in 30.8% (251 of 814) participants who completed all evaluations and had valid data on all prespecified demographic and clinical covariates. The mean age of the participants was 9.1 years, with 413 males (50.7%). In a multivariable logistic regression analysis, lower baseline hemoglobin concentration (P < .001), higher baseline systolic blood pressure (P = .018), and male sex (P = .030) were statistically significantly associated with an increased risk of an SCI. Hemoglobin concentration and systolic blood pressure are risk factors for SCI in children with SCA and may be therapeutic targets for decreasing the risk of SCI. This study is registered at www.clinicaltrials.gov as #NCT00072761.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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