This Article Full Text (PDF) References Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Bernaudin, F. Articles by Brugières, P. Search for Related Content PubMed PubMed Citation Articles by Bernaudin, F. Articles by Brugières, P. Social Bookmarking                   What's this?

Multicenter Prospective Study of Children With Sickle Cell Disease: Radiographic and Psychometric Correlation

Department of Pediatrics, francoise.bemaudin{at}chicreteil.fr

S. Verlhac, MD

Department of Radiology Créteil

F. Fréard, CP

Department of Pediatrics

F. Roudot-Thoraval, MD

Department of Statistics Créteil

M. Benkerrou, MD

Department of Hemato-Pediatrics Debré, Paris

I. Thuret, MD

Department of Hemato-Pediatrics, Marseille

R. Mardini, MD

Department of Hemato-Pediatrics, Debrousse, Lyon

J.P. Vannier, MD, PhD

Department of Hemato-Pediatrics Rouen, France

E. Ploix, CP

Department of Hemato-Pediatrics Debré, Paris

M. Romero, CP

Department of Pediatrics

C. Cassé-Perrot, CP

Department of Hemato-Pediatrics, Marseille

M. Helly, CP

Department of Hemato-Pediatrics, Debrousse, Lyon

E. Gillard, CP

Department of Hemato-Pediatrics Rouen, France

G. Sebag, MD

Department of Radiology Robert Debré, Paris

H. Kchouk, MD

Department of Radiology Robert Debré, Paris

J.P. Pracros, MD

Department of Radiology Debrousse, Lyon

B. Finck, MD

Department of Radiology Debrousse, Lyon

J.N. Dacher, MD, PhD

Department of Radiology Rouen, France.

V. Ickowicz, MD

Department of Radiology Rouen, France.

C. Raybaud, MD

Department of Neuroradiology Marseille

M. Poncet, MD

Department of Neuroradiology Marseille

E. Lesprit, MD

Department of Pediatrics

P.H. Reinert, MD

Department of Pediatrics

P. Brugières, MD

Department of Neuroradiology Créteil

After obtaining familial informed consent, between January 1996 and July 1997, 173 children (5 to 15 years old) with sickle cell disease were enrolled in a prospective multicenter study using blood screening, transcranial Doppler ultrasonography (n = 143), cerebral magnetic resonance imaging (n = 144), and neuropsychologic performance evaluation (n = 156) (Wechsler Intelligence tests WISC-III, WIPPSI-R), which were also performed in 76 sibling controls (5 to 15 years old). Among the 173 patients with sickle cell disease (155 homozygous for hemoglobin SS, 8 sickle cell ß⁰ thalassemia, 3 sickle cell ß ⁺ thalassemia, 7 sickle cell hemoglobin C disease SC), 12 (6.9%) had a history of overt stroke, and the incidence of abnormal transcranial Doppler ultrasonography (defined as mean middle cerebral artery velocity > 200 cm/sec or absent) was 8.4% in the overall study population and 9.6% in patients with homozygous sickle cell anemia. The silent stroke rate was 15%. Significantly impaired cognitive functioning was observed in sickle cell disease patients with a history of stroke (Performance IQ and Full Scale IQ), but also in patients with silent strokes (Similarities, Vocabulary, and Verbal Comprehension). However, infarcts on magnetic resonance imaging were not the only factors of cognitive deficit: Verbal IQ, Performance IQ, and Full Scale IQ were strongly impaired in patients with severe chronic anemia (hematocrit 20%) and in those with thrombocytosis (platelets > 500 x 10⁹/L). Multivariate logistic regression analysis showed that abnormal magnetic resonance imaging (odds ratio [OR] = 2.76) (P = .047), hematocrit 20% (OR = 5.85) (P = .005), and platelets > 500 x 10⁹/L (OR = 3.99) ( P = .004) were independent factors of cognitive deficiency (Full Scale IQ < 75) in sickle cell disease patients. The unfavorable effect of low hematocrit has already been suggested, but this is the first report concerning an effect of thrombocytosis and showing that silent stroke alone is not a factor of cognitive deficit when not associated with low hematocrit or thrombocytosis. The effect of hydroxyurea, which is known to increase hematocrit and decrease platelet count, on cognitive functioning of sickle cell patients should be evaluated prospectively. (J Child Neurol 2000;15:333-343).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

				F. Bernaudin, G. Socie, M. Kuentz, S. Chevret, M. Duval, Y. Bertrand, J.-P. Vannier, K. Yakouben, I. Thuret, P. Bordigoni, et al. Long-term results of related myeloablative stem-cell transplantation to cure sickle cell disease Blood, October 1, 2007; 110(7): 2749 - 2756. [Abstract] [Full Text] [PDF]

				C. M. Hill, A. M. Hogan, N. Onugha, D. Harrison, S. Cooper, V. J. McGrigor, A. Datta, and F. J. Kirkham Increased Cerebral Blood Flow Velocity in Children With Mild Sleep-Disordered Breathing: A Possible Association With Abnormal Neuropsychological Function Pediatrics, October 1, 2006; 118(4): e1100 - e1108. [Abstract] [Full Text] [PDF]

				J. J. Strouse, C. S. Cox, E. R. Melhem, H. Lu, M. A. Kraut, A. Razumovsky, K. Yohay, P. C. van Zijl, and J. F. Casella Inverse correlation between cerebral blood flow measured by continuous arterial spin-labeling (CASL) MRI and neurocognitive function in children with sickle cell anemia (SCA) Blood, July 1, 2006; 108(1): 379 - 381. [Abstract] [Full Text] [PDF]

				M. C. Kral, R. T. Brown, J. K. Cure, N. Besenski, S. M. Jackson, and M. R. Abboud Radiographic Predictors of Neurocognitive Functioning in Pediatric Sickle Cell Disease J Child Neurol, January 1, 2006; 21(1): 37 - 44. [Abstract] [PDF]

				O. S. Platt Prevention and Management of Stroke in Sickle Cell Anemia Hematology, January 1, 2006; 2006(1): 54 - 57. [Abstract] [Full Text] [PDF]

				R. G. Steen, C. Fineberg-Buchner, G. Hankins, L. Weiss, A. Prifitera, and R. K. Mulhern Cognitive Deficits in Children With Sickle Cell Disease J Child Neurol, February 1, 2005; 20(2): 102 - 107. [Abstract] [PDF]

				R. Grueneich, M. D. Ris, W. Ball, K. A. Kalinyak, R. Noll, K. Vannatta, and R. Wells Relationship of Structural Magnetic Resonance Imaging, Magnetic Resonance Perfusion, and Other Disease Factors to Neuropsychological Outcome in Sickle Cell Disease J. Pediatr. Psychol., March 1, 2004; 29(2): 83 - 92. [Abstract] [Full Text] [PDF]

				G. R. Buchanan, M. R. DeBaun, C. T. Quinn, and M. H. Steinberg Sickle Cell Disease Hematology, January 1, 2004; 2004(1): 35 - 47. [Abstract] [Full Text] [PDF]

				R. G. Steen, M. A. Miles, K. J. Helton, S. Strawn, W. Wang, X. Xiong, and R. K. Mulhern Cognitive Impairment in Children with Hemoglobin SS Sickle Cell Disease: Relationship to MR Imaging Findings and Hematocrit AJNR Am. J. Neuroradiol., March 1, 2003; 24(3): 382 - 389. [Abstract] [Full Text] [PDF]

				J. Schatz, R. L. Finke, J. M. Kellett, and J. H. Kramer Cognitive Functioning in Children With Sickle Cell Disease: A Meta-Analysis J. Pediatr. Psychol., December 1, 2002; 27(8): 739 - 748. [Abstract] [Full Text] [PDF]

				J. Schatz, D. A. White, A. Moinuddin, M. Armstrong, and M. R. DeBaun Lesion Burden and Cognitive Morbidity in Children With Sickle Cell Disease J Child Neurol, December 1, 2002; 17(12): 890 - 894. [Abstract] [PDF]

				C. H. Pegelow, E. A. Macklin, F. G. Moser, W. C. Wang, J. A. Bello, S. T. Miller, E. P. Vichinsky, M. R. DeBaun, L. Guarini, R. A. Zimmerman, et al. Longitudinal changes in brain magnetic resonance imaging findings in children with sickle cell disease Blood, April 15, 2002; 99(8): 3014 - 3018. [Abstract] [Full Text] [PDF]

				A. Eldor and E. A. Rachmilewitz The hypercoagulable state in thalassemia Blood, January 1, 2002; 99(1): 36 - 43. [Abstract] [Full Text] [PDF]

				R. J. Adams, K. Ohene-Frempong, and W. Wang Sickle Cell and the Brain Hematology, January 1, 2001; 2001(1): 31 - 46. [Abstract] [Full Text] [PDF]