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      Conventional and advanced brain MR imaging in patients with sickle cell anemia

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          Abstract

          Background:

          Sickle cell disease (SCD) is an autosomal recessive hemolytic disorder; its cerebrovascular complications include silent cerebral ischemia, infarct, and brain atrophy. Conventional magnetic resonance imaging (MRI) often underestimates the extent of injury. Diffusion tensor imaging (DTI) can demonstrate and quantify microstructural brain changes in SCD cases having normal routine MRI.

          Objective:

          To identify various neurological abnormalities in asymptomatic sickle cell patients using routine MRI and to evaluate the microstructure of various regions of the brain using DTI.

          Materials and Methods:

          A prospective, randomized case–control study was conducted over a period of 2 years. A total of 58 cases of SCD and 56 age- and sex-matched controls were included. Routine MRI and DTI were performed in both the groups following a standard protocol. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were calculated in certain pre-defined regions. Primary data were analyzed using MS excel version 17. Analysis of variance test was performed and statistical significance was set at P < 0.05.

          Results:

          Thirty regions of interest with 60 variables were included in the final analysis. Patients with SCD showed statistically significant reduced FA values, increased ADC values, or both, clustered in several brain areas, including pons, cerebral peduncle, corpus callosum, frontal, temporal, parietal white matter, centrum semiovale, periventricular areas, basal ganglia, and left thalamus ( P < 0.05).

          Conclusion:

          DTI is a promising method for characterizing microstructural changes, when conventional MRI is normal.

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          Most cited references10

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          Longitudinal changes in brain magnetic resonance imaging findings in children with sickle cell disease.

          Children with sickle cell anemia (HbSS) are at high risk for neurologically overt cerebral infarcts associated with stroke and neurologically silent cerebral infarcts correlated with neuropsychometric deficit. We used complete magnetic resonance imaging (MRI) histories from 266 HbSS children, aged 6 through 19 years, who were enrolled in the Cooperative Study of Sickle Cell Disease (CSSCD) to examine silent infarct prevalence, localization, recurrence, and progression. We report a baseline prevalence of 21.8%, marginally higher than previously reported due to improved imaging technologies. Although we observed no overall sex difference in prevalence, most lesions in girls occurred before age 6, whereas boys remained at risk until age 10. Silent infarcts were significantly smaller and less likely to be found in the frontal or parietal cortex than were infarcts associated with stroke. Children with silent infarct had an increased incidence of new stroke (1.03/100 patient-years) and new or more extensive silent infarct (7.06/100 patient-years) relative to stroke incidence among all children in our cohort (0.54/100 patient-years). Both events were substantially less frequent than the risk of stroke recurrence among children not provided chronic transfusion therapy. Although chronic transfusion is known to decrease occurrence of new silent infarcts and strokes in children with elevated cerebral arterial blood flow velocity, further study is required to determine its risk-benefit ratio in children with silent infarct and normal velocities. Until safe and effective preventive strategies against infarct recurrence are discovered, MRI studies are best reserved for children with neurologic symptoms, neuropsychometric deficits, or elevated cerebral artery velocities.
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            Pathophysiology of sickle cell disease is mirrored by the red blood cell metabolome.

            Emerging metabolomic tools can now be used to establish metabolic signatures of specialized circulating hematopoietic cells in physiologic or pathologic conditions and in human hematologic diseases. To determine metabolomes of normal and sickle cell erythrocytes, we used an extraction method of erythrocytes metabolites coupled with a liquid chromatography-mass spectrometry-based metabolite profiling method. Comparison of these 2 metabolomes identified major changes in metabolites produced by (1) endogenous glycolysis characterized by accumulation of many glycolytic intermediates; (2) endogenous glutathione and ascorbate metabolisms characterized by accumulation of ascorbate metabolism intermediates, such as diketogulonic acid and decreased levels of both glutathione and glutathione disulfide; (3) membrane turnover, such as carnitine, or membrane transport characteristics, such as amino acids; and (4) exogenous arginine and NO metabolisms, such as spermine, spermidine, or citrulline. Finally, metabolomic analysis of young and old normal red blood cells indicates metabolites whose levels are directly related to sickle cell disease. These results show the relevance of metabolic profiling for the follow-up of sickle cell patients or other red blood cell diseases and pinpoint the importance of metabolomics to further depict the pathophysiology of human hematologic diseases.
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              The spectrum of brain MR abnormalities in sickle-cell disease: a report from the Cooperative Study of Sickle Cell Disease.

              To define the spectrum of abnormalities in sickle-cell disease, including infarction, atrophy, and hemorrhage, that are identified by brain MR imaging. All MR studies included T1, T2, and intermediate pulse sequences. Images were interpreted without knowledge of the clinical history or neurologic examination findings. Brain MR imaging was performed in 312 children with sickle-cell disease. Seventy patients (22%) had infarction/ischemia and/or atrophy, infarction/ischemia was noted in 39 children (13%) who had no history of a stroke (the "silent" group). The prevalence rates for silent lesions were 17% for sickle-cell anemia and 3% for hemoglobin sickle-cell disease. For patients with sickle-cell anemia and a history of cerebrovascular accident, infarction/ischemia lesions typically involved both cortex and deep white matter, while silent lesions usually were confined to deep white matter. Within the age range studied, the prevalence of infarction/ischemia did not increase significantly with age, although older patients with lesions had more lesions than did younger patients with lesions. Brain MR imaging showed infarction/ischemia in the absence of a recognized cerebrovascular accident in 13% of patients. The prevalence of these lesions did not increase significantly between the ages of 6 and 14 years, suggesting that lesions are present by age 6. However, the increase in the average number of lesions per patient with age may indicate progressive brain injury.
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                Author and article information

                Journal
                Indian J Radiol Imaging
                Indian J Radiol Imaging
                IJRI
                The Indian Journal of Radiology & Imaging
                Medknow Publications & Media Pvt Ltd (India )
                0971-3026
                1998-3808
                Jul-Sep 2018
                : 28
                : 3
                : 305-311
                Affiliations
                [1]Department of Radiodiagnosis, J.L.N. Hospital and Research Centre, Bhilai, Chhattisgarh, India
                [1 ]Department of Hematology, J.L.N. Hospital and Research Centre, Bhilai, Chhattisgarh, India
                [2 ]Department of Gastroenterology, J.L.N. Hospital and Research Centre, Bhilai, Chhattisgarh, India
                Author notes
                Correspondence: Dr. Pratibha Issar, Department of Radiodiagnosis, J.L.N. Hospital and R.C, Bhilai - 490 009, Chhattisgarh, India. E-mail: mareesh_23@ 123456yahoo.co.in
                Article
                IJRI-28-305
                10.4103/ijri.IJRI_166_17
                6176679
                30319206
                38129972-79eb-4774-98f1-0c76cd1864ad
                Copyright: © 2018 Indian Journal of Radiology and Imaging

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                Categories
                Neuroradiology & Head and Neck Imaging

                Radiology & Imaging
                apparent diffusion coefficient,diffusion tensor imaging,fractional anisotropy,sickle cell disease,sickle cell anemia,sickle cell trait

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