Abstract W MP111: Prevalence and Risk Factors of Silent Cerebral Infarcts in Children with Hemoglobin SC Disease
Background: Hemoglobin (Hb) SC disease comprises 22% of all sickle hemoglobinopathies and is the second most common form of sickle cell disease. Silent cerebral infarcts (SCI) are common in Hb SS disease and are associated with male sex, higher systolic blood pressure (SBP), and lower baseline Hb concentration among children with Hb SS. However, SCI prevalence and risk factors are less well characterized in Hb SC disease. We tested the hypothesis that risk factors for SCI in Hb SC and Hb SS are similar.
Methods: Retrospective chart review of children with Hb SC seen at St. Louis Children’s Hospital (SLCH) Sickle Cell Clinic 2004-2012 who had brain magnetic resonance imaging (MRI) available. At SLCH, all children with sickle cell disease undergo screening brain MRI after their 6th birthday; additional MRIs are obtained for concerning symptoms. Cerebral infarctions were identified as T2- or FLAIR-weighted hyperintensities identified in at least 2 planes or decreased diffusion; silent infarcts were diagnosed when the patient had no neurological symptoms that correlated with the infarct lesions. Hb concentrations and SBP were obtained from clinic well visits within a year before and after MRI. Human Studies Committee approval and waiver of consent were granted for this study.
Results: Thirteen of 96 patients with Hb SC disease had SCI on MRI. The prevalence of SCI was 13.5%. Twelve (92%) subjects had subcortical lesions; 11 (85%) had SCI in the frontal lobe; and 10 (77%) subjects had bilateral infarctions. Seven (50%) of subjects with SCI were male. The mean age at identification of SCI was 11.9 years (range, 6.2-19.3 years). Ten children with SCIs had repeat MRI (range 0.1-6.4 years) following the SCI diagnosis. No child had progression or additional SCI on repeat imaging. Sex, SBP, and Hb concentration were not significantly different between children with and without SCIs (p=0.4).
Conclusion: Our cohort of children with Hb SC had a SCI prevalence of 13.5%. The majority of children had bilateral subcortical lesions. SCIs had frontal lobe predominance. Unlike children with Hb SS, gender, elevated SBP or lower baseline Hb were not risk factors for SCIs in this cohort.
Author Disclosures: K. Guilliams: None. J. Sun: None. M. Hulbert: None.
- © 2014 by American Heart Association, Inc.