Cognitive deficits in beta-thalassemia major

Monastero, Roberto; Monastero, G.; Ciaccio, C; Padovani, Alessandro; Camarda, Rosolino

doi:10.1034/j.1600-0404.2000.102003162.x

Cited by 68 publications

(93 citation statements)

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“…Our findings support the results of other studies [2][3][4][5][6][7][8][9][10][11][12][13][14] on the quality of life of TM patients and those with other chronic diseases, including Crohn disease, ulcerative colitis, cystic fibrosis, congenital heart disease, asthma, epilepsy, sweet diabetes and cancer [23--26]. These studies found that patients with these diseases show a significant decrease in life satisfaction and mental health or a significant increase in probable psychiatric disorders, including somatisation, interpersonal sensitivity, depression, anxiety and psychosis.…”

Section: Discussionsupporting

confidence: 83%

“…These treatments can also have an impact on the families of patients, especially on siblings [2][3][4][5][6][7][8][9][10]. Some investigations have found that 80% of people diagnosed with TM have at least 1 psychiatric disorder [2], the most frequent being disembodiment [3,4], anxiety [5,6], depression [7], somatoform disorders [8] and anger [9,10]. This disease can also have a deleterious impact on the social activities and education of patients [11,12].…”

Section: Introductionmentioning

confidence: 99%

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Quality of life of Iranian β-thalassaemia major patients living on the southern coast of the Caspian Sea

Khani

Majdi²,

Marzabadi³

et al. 2012

East Mediterr Health J

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In the present study, the aim was to explore the quality of life of thalassaemia major (TM) patients according to age, sex, school performance, severity and complications of the disease. A cross-sectional descriptive study was conducted. Quality of life was evaluated by 4 questionnaires; demographic characteristics, the short form 36 health survey (SF-36), Persian version of symptoms checklist-90-revised (SCL-90-R) and life satisfaction index (LSI). 687 (41.08%) of the eligible patients with TM completed the questionnaires. With the SF-36 questionnaire, 329 (47.9%) participants had an excellent level of physical functioning. With the SCL-90-R questionnaire, 446 (64.9%) participants had a global severity index of > 0.7, considered psychiatric patients, 141 (20.5%) patients had GSI between 0.4 and 0.7, considered suspected psychiatric patients and 100 (14.6%) participants had GSI of < 0.4, considered non-psychiatric patients. The mean score of LSI questionnaire was 20.50 (SD 5.95), range 2-37. Beta-TM patients are at risk of psychiatric symptoms and need appropriate psychiatric counselling. Qualité de vie de patients iraniens atteints de β-thalassémie majeure vivant sur la côte sud de la mer Caspienne RÉSUMÉ La présente étude vise à examiner la qualité de vie de patients atteints de thalassémie majeure en fonction de l'âge, du sexe, des résultats scolaires, et de la gravité et des complications de la maladie. Une étude descriptive transversale a été menée. Dans cette étude, la qualité de vie a été évaluée au moyen de quatre outils : les caractéristiques démographiques, le questionnaire d'évaluation de la santé SF-36 en version abrégée, la version en persan de l'échelle de symptômes SCL-90-R et l'indice de satisfaction de vie. Parmi les patients éligibles souffrant de thalassémie majeure, 687 d'entre eux (41,08 %) ont rempli les questionnaires. Selon les résultats du questionnaire SF-36, 329 participants (47,9 %) avaient un excellent niveau de capacité physique fonctionnelle. En tout, 446 participants (64,9 %) ont obtenu un indice de sévérité global de plus de 0,7 (patients psychiatriques) au questionnaire SCL-90-R ; 141 participants (20,5 %) présentaient un indice de sévérité global allant de 0,4 à 0,7 (patients psychiatriques présumés) ; et 100 participants (14,6 %) avaient des résultats inférieurs à 0,4 (patients non psychiatriques). Le score moyen au questionnaire d'indice de satisfaction de vie était de 20,50 (± 5,95, extrêmes 2-37). Les patients souffrant de β-thalassémie majeure risquent de présenter des symptômes psychiatriques et ont donc besoin d'un accompagnement dans ce domaine.

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Section: Discussionsupporting

confidence: 83%

Section: Introductionmentioning

confidence: 99%

Quality of life of Iranian β-thalassaemia major patients living on the southern coast of the Caspian Sea

Khani

Majdi²,

Marzabadi³

et al. 2012

East Mediterr Health J

View full text Add to dashboard Cite

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“…In a study on 46 patients with b-thalassemia major, it was reported that the neuropsychological tests were significantly impaired especially in patients with signs of hemosiderosis [26]. In this study, a significant relationship was shown between the timing of institution and duration of transfusion and chelation therapies and neurological findings, while there was no correlation between desferrioxamine doses, hemoglobin, and ferritin levels.…”

Section: Discussionmentioning

confidence: 47%

Cognitive dysfunction in β‐thalassemia minor

et al. 2006

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Turkey b-thalassemia minor is a common, hereditary, and mostly symptomless disease. Previous studies have shown that lower hemoglobin values are associated with poorer cognitive functions. We aimed to evaluate the cognitive function in patients with b-thalassemia minor. Thirty-two male subjects with b-thalassemia minor and 32 sex-, age-, and education statusmatched healthy subjects were enrolled in the study. Blood tests and P300 potentials were carried out. P300 potential latency in all patients was significantly longer than those in the control group (337.63 ± 34.89 msec and 310.66 ± 14.30 msec, respectively; t 4.046, P < 0.001). The amplitudes of P300 in patient group were significantly lower than those in the control group (5.19 ± 3.59 lV and 9.81 ± 3.33 lV, respectively; t 5.349, P < 0.001). In this study, we have found that P300 potentials are adversely affected for cognitive functions in patients with b-thalassemia minor. Am. J. Hematol. 82:203-207, 2007. V V C 2006 Wiley-Liss, Inc.

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“…In addition, factors associated with chronic illness leading to regular school absence, physical and social restrictions, abnormal mental state, rather than the disease per se, could also play a potential role in the development of cognitive dysfunction in patients with thalassemia. 2,3 It would have been interesting to evaluate the effect of the various chelation agents on iron deposition. Although our preliminary results showed no significant differences, this retrospective study was limited by the confounding variable of different treatment times and the small sample size.…”

Section: Discussionmentioning

confidence: 99%

MR Quantitative Susceptibility Imaging for the Evaluation of Iron Loading in the Brains of Patients with -Thalassemia Major

Qiu

Chan

Chu

et al. 2014

American Journal of Neuroradiology

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Cognitive deficits in beta-thalassemia major

Abstract: Our findings suggest that beta-th is associated with neuropsychological impairment involving multiple cognitive domains and argue for a potential role of hemosiderosis on cognitive functioning.

Cited by 68 publications

References 0 publications

Quality of life of Iranian β-thalassaemia major patients living on the southern coast of the Caspian Sea

Quality of life of Iranian β-thalassaemia major patients living on the southern coast of the Caspian Sea

Cognitive dysfunction in β‐thalassemia minor

MR Quantitative Susceptibility Imaging for the Evaluation of Iron Loading in the Brains of Patients with -Thalassemia Major

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