A Study of Silent
A Study of Silent Cerebral Infarcts in Pediatric Patients with Sickle Cell Disease by Magnetic Resonance Imaging
Eglal Hussein S. S. Algohary1; Muneer Al Bagishi2; Qasem Al Alwan3; Ashwak Safi El-Din M.4; Waleed H. AlbuAli1 and Fulton S.D’souza1
1Pediatric Department, College of Medicine, King Feisal University; 2 PediatricDepartment, Maternity and Children Hospital- Al Ahsa; 3 Radiology Department; King Fahd Hospital- Al Ahsa; 4Department of FAMCO Medicine, college of Medicine KingAbdel Aziez University. firstname.lastname@example.org
Abstract: BACKGROUND: Sickle cell disease (SCD) is a common hereditary disease in Saudi Arabia, with a high prevalence in the Eastern and Southern regions. Among the genitive basis of the disease, the homozygous form (Hb-SS) is the most seriousone. Silent cerebral infarcts (SCI) are the most common form of neurologic injury in those children and are defined as an abnormal magnetic resonance image (MRI) of the brain with increased signal intensity in multiple T2-weighted images accompanied by undetectable neurocognitive deficits on examination. OBJECTIVES:The present study is designed to detect the relative frequency of SCI using MRI among children with SCD as general and especially homozygous form (Hb-SS). Associated cognitive impairment is evaluated on basis of occurrence SCI in one hand and in relation to other risk factors associated with this chronic disease without SCI finding in the other hand. METHODOLOGY: A hospital based cross-sectional study was conducted on a total of 90 patients with SCD of both sexes, who had been followed since birth. They were consecutively seen and chosen neither have previous seizure or other neurological abnormality nor having history of cerebrovascular accident.From these patients, 59 were submitted to undergo MRI scanning and to evaluate their IQ level. Relationsbetween MRI finding, IQ levels and laboratory results are conducted. RESULTS:Age distribution of the studied SCD patients was ranged from 3 to 12 years and 8 months; with a mean equals 8.08±2.58 yrs. Male to female ratio was 3 to 2. SCI findings were detected in two patients; homozygous Hb-SS[A female and malepatients, their ages 9.17 and 7.75 years old respectively, haveMRI of the brain with increased signal intensity in multiple T2-weighted images] out of 53 homozygous Hb-SS (the relative frequency 3.77%), and out of total 59sickling patients subjected to MRI study ( the relative frequency 3.39%). Statistical analysis revealed strong significant correlations of IQ values decline in homozygous Hb-SS patients having SCI findings with all studied parameters(P< 0.01). Moreover, IQ values significantly inversely correlated with both age (p=0.01) and percentage of Hb.S (p=0.04); where it significantly directly correlated with both total Hb. Concentration (p=0.05) and percentage of Hb.F(p=0.013) in sickling patients without evidence of SCI. In spite ofIQ values in patients with HbSβ0-thalassemia showed the lowest subnormal values, they did not reach to statistical level. Percentage of Hb.A2, serum ferritin level, total leukocyte count, and platelets count are forming also risk factors that should be considered in the follow up of patients with SCD. CONCLUSION:SCD-SS is associated with an increased risk of silent infarction in diseased patients in a percentage of 3.77% in Al Ahsa, eastern area of KSA. A decline in the IQ values of those patients was significantly related to their age, Hemoglobin concentration, and percentageof Hb.S, Hb.F, and Hb.A2. Patients with HbSβ0-thalassemia are emerging to prone to risk ofsilent infarction& hazard of decline IQ as patient with homozygous Hb.SS.RECOMMENDATIONS:Regional newborn screening programs for SCD using hematological genetic phenotypic analysis should be considered in eastern area of KSA. Meticulous follow up of SCD-SS children, specially preteen age, and routine brain MRI scan will yield better prognosis and preserve their cognitive achievements. A Strategy for preventive therapy must be designed.
[Eglal Hussein S. S. Algohary; Muneer Al Bagishi; Qasem Al Alwan; Ashwak Safi El-Din M; Waleed H. AlbuAli and Fulton S.D’souza. A Study of Silent Cerebral Infarcts in Pediatric Patients with Sickle Cell Disease by Magnetic Resonance Imaging. J Am Sci 2012;8(10):629-640]. (ISSN: 1545-1003).http://www.jofamericanscience.org. 86
Key words: Sickle cell disease; Silent cerebral infarcts;Magnetic resonance image;Cognitive impairment; IQ; Pediatrics Full Text 86