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      A Traditional Review of Sickle Cell Disease and the Associated Onset of Dementia: Hematological and Neurocognitive Crossroads

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          Abstract

          Sickle cell trait and disease are potential risk factors for dementia and cognitive dysfunction in African Americans, as are genetic variants. This illness affects around 300 million people globally. Due to its ability to defend against severe malaria, it represents an evolutionary survival advantage. It has been shown that sickle cell disease and trait are independent risk factors for the prevalence and incidence of albuminuria and chronic renal disease. Sickle cell anemia impairs cognitive performance in people with minimal or mild manifestations of the genetic blood disorder, owing mostly to its cerebrovascular implications. Similarly, various cerebral minor vascular disorders, such as silent cerebral infarcts, have been linked to the sickle cell trait, which is associated with impaired cognitive ability. It has been found that patients with sickle cell disease have a significantly decreased subcortical and cortical brain volume. Adults and children with sickle cell disease have been documented to have attention-related issues, particularly reduced sustained attention. 

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          Global epidemiology of haemoglobin disorders and derived service indicators.

          To demonstrate a method for using genetic epidemiological data to assess the needs for equitable and cost-effective services for the treatment and prevention of haemoglobin disorders. We obtained data on demographics and prevalence of gene variants responsible for haemoglobin disorders from online databases, reference resources, and published articles. A global epidemiological database for haemoglobin disorders by country was established, including five practical service indicators to express the needs for care (indicator 1) and prevention (indicators 2-5). Haemoglobin disorders present a significant health problem in 71% of 229 countries, and these 71% of countries include 89% of all births worldwide. Over 330,000 affected infants are born annually (83% sickle cell disorders, 17% thalassaemias). Haemoglobin disorders account for about 3.4% of deaths in children less than 5 years of age. Globally, around 7% of pregnant women carry b or a zero thalassaemia, or haemoglobin S, C, D Punjab or E, and over 1% of couples are at risk. Carriers and at-risk couples should be informed of their risk and the options for reducing it. Screening for haemoglobin disorders should form part of basic health services in most countries.
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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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              Sickle Cell Disease: New Opportunities and Challenges in Africa

              Sickle cell disease (SCD) is one of the most common genetic causes of illness and death in the world. This is a review of SCD in Africa, which bears the highest burden of disease. The first section provides an introduction to the molecular basis of SCD and the pathophysiological mechanism of selected clinical events. The second section discusses the epidemiology of the disease (prevalence, morbidity, and mortality), at global level and within Africa. The third section discusses the laboratory diagnosis and management of SCD, emphasizing strategies that been have proven to be effective in areas with limited resources. Throughout the review, specific activities that require evidence to guide healthcare in Africa, as well as strategic areas for further research, will be highlighted.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                19 October 2021
                October 2021
                : 13
                : 10
                : e18906
                Affiliations
                [1 ] Family Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
                [2 ] Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
                [3 ] Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND
                [4 ] General Surgery, Research, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
                [5 ] Internal Medicine, Family Medicine, Neurology, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
                [6 ] Diagnostic Radiology, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
                [7 ] Psychiatry, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
                Author notes
                Article
                10.7759/cureus.18906
                8530004
                34703679
                f0a04c6d-56b7-45d3-9360-1f37e1ae6213
                Copyright © 2021, Ojinnaka et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 11 July 2021
                : 19 October 2021
                Categories
                Family/General Practice
                Neurology
                Hematology

                sickle cell disease,dementia,cognitive dysfunction,sickle trait,sickle cell trait

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