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      In Patients with Minor Beta-Thalassemia, Cognitive Performance Is Related to Length of Education, But Not to Minor Beta-Thalassemia or Hemoglobin Levels

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          Abstract

          Objective: Thalassemia is one of the most frequent monogenic disorders, leading to impairment in the maturation and survival of red blood cells. The question examined here is whether, and if so, to what extent, people with beta-thalassemia might also be impaired in their cognitive functioning. Previous results in adults with beta-thalassemia showed cognitive impairment when compared to healthy controls. However, length of education was never taken into consideration as a possible confounder. Accordingly, the aim of the present study was to assess people with minor beta-thalassemia and compare them to healthy controls, while controlling for length of education.

          Method : A total of 25 adults (mean age: 29.36 years; 56% females) with beta-thalassemia and 25 healthy controls (mean age: 27.84 years; 72% females) took part in this cross-sectional study. They underwent cognitive testing (executive functions, attention, working memory), and their haemoglobin levels were assessed.

          Results: Cognitive performance did not significantly differ between patients with minor beta-thalassemia and healthy controls. Irrespective of group, higher cognitive performance was strongly associated with time spent in education. No gender differences were observed.

          Conclusion: Compared to healthy controls, cognitive performance was not impaired among patients with minor beta-thalassemia when length of education was introduced as a further co-variate. In both patients with minor beta-thalassemia and healthy controls, higher cognitive performance was associated with time spent for education. Health professionals should inform patients with minor beta-thalassemia that cognitive performance is related to the length of education and not to the health status of minor beta-thalassemia per se.

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          Most cited references16

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          Thalassaemia.

          Inherited haemoglobin disorders, including thalassaemia and sickle-cell disease, are the most common monogenic diseases worldwide. Several clinical forms of α-thalassaemia and β-thalassaemia, including the co-inheritance of β-thalassaemia with haemoglobin E resulting in haemoglobin E/β-thalassaemia, have been described. The disease hallmarks include imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic haemolytic anaemia, compensatory haemopoietic expansion, hypercoagulability, and increased intestinal iron absorption. The complications of iron overload, arising from transfusions that represent the basis of disease management in most patients with severe thalassaemia, might further complicate the clinical phenotype. These pathophysiological mechanisms lead to an array of clinical manifestations involving numerous organ systems. Conventional management primarily relies on transfusion and iron-chelation therapy, as well as splenectomy in specific cases. An increased understanding of the molecular and pathogenic factors that govern the disease process have suggested routes for the development of new therapeutic approaches that address the underlying chain imbalance, ineffective erythropoiesis, and iron dysregulation, with several agents being evaluated in preclinical models and clinical trials.
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            New trend in the epidemiology of thalassaemia

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              The 50 and 100-item short forms of the Paced Auditory Serial Addition Task (PASAT): demographically corrected norms and comparisons with the full PASAT in normal and clinical samples.

              While the standard 200-item version of the Paced Auditory Serial Addition Task (PASAT) is a sensitive neuropsychological instrument, it can be quite aversive to some patients due to its length and progressively increasing difficulty. We present demographically-corrected norms for 50 and 100-item short-form versions in a sample of 560 neurologically normal adults. Age, education, and ethnicity (but not gender) were found to be significant predictors of performance. In a clinical sample of 786 HIV-infected adults, diagnostic accuracy of the 50, 100, and 200-item versions was essentially equivalent (using clinical ratings of a comprehensive neuropsychological battery as the gold standard, overall classification rates of the three PASAT versions were 71%, 74%, and 73%, respectively), with better specificity (89-92%) than sensitivity (46-53%). The 50-item version showed moderate ceiling effects, but the 100-item test did not. In a mixed clinical sample of 40 subjects, the 50-item version was administered more than twice as fast as the 200-item version, and was tolerated better (discomfort rating of 4.0 vs. 5.9 on a 10-point scale, p < .05). We conclude that in many cases the PASAT-50 and PASAT-100 provide equivalent diagnostic accuracy with a significant reduction in administration time and patient discomfort.
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                Author and article information

                Journal
                Iran J Psychiatry
                Iran J Psychiatry
                IJPS
                Iranian Journal of Psychiatry
                Psychiatry & Psychology Research Center, Tehran University of Medical Sciences (Tehran, Iran )
                1735-4587
                2008-2215
                January 2019
                : 14
                : 1
                : 47-53
                Affiliations
                [1 ]Research Center for Behavioral Disorders and Substance Abuse, Hamadan University of Medial Sciences, Hamadan, Iran.
                [2 ]Department of Pathology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran.
                [3 ]Department of Community Medicine, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran.
                [4 ]University of Basel, Psychiatric Clinics, Center for Affective, Stress and Sleep Disorders, Basel, Switzerland.
                [5 ]University of Basel, Department of Sport, Exercise and Health, Division of Sport Sciences and Psychosocial Health, Basel, Switzerland.
                [6 ]Kermanshah University of Medical Sciences, Sleep Disorders Research Center and Substance Abuse Prevention Research Center, Kermanshah, Iran.
                Author notes
                [* ]Corresponding Author: Address: Research Center for Behavioral Disorders and Substances Abuse, Hamadan University of Medical Sciences, Hamadan, Iran. Postal Code: 6516848741. Tel: 98-8138271066, Fax: 98-8138271066, Email: Ahmadpanah@ 123456umsha.ac.ir
                Article
                IJPS-14-47
                6505049
                31114617
                701a036e-eaa4-4f0f-a3c2-d872d86f40b7
                Copyright © Psychiatry & Psychology Research Center, Tehran University of Medical Sciences

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License, ( http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 19 May 2018
                : 1 September 2018
                : 10 September 2018
                Categories
                Original Article

                Clinical Psychology & Psychiatry
                beta-thalassemia minor,cognitive performance,long-term memory,length of education,working memory

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