Growing up with the families of B-thalassaemia major using an accelerated longitudinal design

Khairkar, Praveen; Malhotra, Sonal; Marwaha, R. K.

doi:10.1016/s0924-9338(11)72089-2

Cited by 14 publications

(19 citation statements)

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“…The authors concluded that the intellectual development of β‐TM children is within the expected range. This is in line with Khairkar et al ., who reported normal IQ in β‐TM children, but that study did not include a healthy control group. In contrast, Duman et al .…”

Section: Introductionsupporting

confidence: 88%

Cognitive functions in adults with β‐thalassemia major: before and after blood transfusion and comparison with healthy controls

Raz

Koren

Dan

et al. 2016

Annals of the New York Academy of Sciences

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While β-thalassemia major (β-TM)-related physiological complications have been well established, less is known about implications for neuropsychological and cognitive function. The few existing studies have focused almost exclusively on children. We evaluated cognitive function in adult β-TM patients compared to healthy controls (study 1) and in β-TM patients before and after blood transfusion (study 2). Performance intelligence quotient (IQ) was evaluated with four subtests from the Wechsler Adult Intelligence Scale (WAIS-III). Attention functions were evaluated using the online continuous performance test (OCPT). The results of study 1 revealed poorer performance of β-TM patients on three of the four intelligence subtests, with significantly lower total performance IQ scores compared with controls. The percentage of participants with abnormal performance IQ (<85) was almost five times higher in the β-TM group (58%) than in the control group (12%). In study 2, significant differences were found in OCPT performance as a function of blood transfusion. Before transfusion, patients had higher rates of omission and commission errors, slower response times (RTs), and lower RT consistency than after transfusion. As β-TM patients' life expectancy is increasing, assessment and treatment of neurocognitive functions should become an integral part of appropriate follow-up to improve patients' quality of life.

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Section: Introductionsupporting

confidence: 88%

Cognitive functions in adults with β‐thalassemia major: before and after blood transfusion and comparison with healthy controls

Raz

Koren

Dan

et al. 2016

Annals of the New York Academy of Sciences

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“…The sample size ranged widely, from 12 (Orsini et al , ) to 294 (Karimi et al , ) (median: 30 patients) The cognitive evaluation was also heterogeneous: Wechsler Intelligence Scale for Children (WISC) and WAIS (or their subtests) were applied in 10/15 studies. Similarly, data presentation was strikingly heterogeneous: mean IQ values were mostly preferred (presented as total, verbal and/or performance IQ values, 8/15 studies, or simply as total IQ, 4/15 studies) but sample description often adopted variable IQ classes (<90, <80 and <70 (Logothetis et al , ; Raafat et al , ); <85 and <70 (Economou et al , ); <88, <76 and <64 (Karimi et al , ); <85 (Teli et al , ; Raz et al , ); <90 but greater than 70 (Khairkar et al , ); borderline or average (Elalfy et al , ).…”

Section: Resultsmentioning

confidence: 99%

Brain functional impairment in beta‐thalassaemia: the cognitive profile in Italian neurologically asymptomatic adult patients in comparison to the reported literature

et al. 2019

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Summary Cognitive involvement in beta‐thalassaemia is strikingly controversial and poorly studied in adulthood. This multicentre prospective study investigated 74 adult neurologically‐asymptomatic beta‐thalassaemia patients (mean‐age 34·5 ± 10·3 years; 53 transfusion‐dependent [TDT], 21 non‐transfusion dependent [NTDT]) and 45 healthy volunteers (mean‐age 33·9 ± 10·7 years). Participants underwent testing with Wechsler Adult Intelligence Scale‐Fourth Edition (WAIS‐IV), Brief Psychiatric Rating Scale (BPRS) and multiparametric brain 3T‐magnetic resonance imaging (MRI) for parenchymal, vascular and iron content evaluation. Patients had lower Full‐Scale Intelligence Quotient (FSIQ) than controls (75·5 ± 17·9 vs. 97·4 ± 18·1, P < 0·0001) even after correction for education level. Compared to TDT, NTDT showed a trend of higher FSIQ (P = 0·08) but a similar cognitive profile at WAIS‐subtests. FSIQ correlated with total and indirect bilirubin (P < 0·0001 and P = 0·002, respectively); no correlation was found with splenectomy, intracranial MRI/magnetic resonance‐angiography findings, brain tissue iron content or other disease‐related clinical/laboratory/treatment data. FSIQ did not correlate with BPRS scores, although the latter were higher among patients (28·74 ± 3·1 vs. 27·29 ± 4·8, P = 0·01) mainly because of increased depression and anxiety levels. Occupation rate was higher among controls (84·4% vs. 64·9%, P = 0·004) and correlated with higher FSIQ (P = 0·001) and education level (P = 0·001). In conclusion, Italian adult beta‐thalassaemia patients seem to present a characteristic cognitive profile impairment and an increased rate of psychological disorders with possible profound long‐term socio‐economic consequences.

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“…16 percent of our patients showed excessive sleep problems, while the Indian study revealed only 1 percent of the parents had such problems [10]. …”

Section: Discussionmentioning

confidence: 99%

Psychosocial problems of Pakistani parents of Thalassemic children: a cross sectional study done in Bahawalpur, Pakistan

Aziz

Sadaf

Kanwal

2012

BioPsychoSocial Med

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BackgroundThalassemia is a blood disorder passed down through families (inherited) in which the body makes an abnormal form of hemoglobin. This disorder results in excessive destruction of red blood cells, and there is no effective treatment. Patients require lifelong blood transfusion, usually started within 6 to 12 months of birth of patient, which on other hand has its own complications. It is a chronic disease that manifests so early in life that it leads to psychological and social problems for parents . We focused on parents to assess the impact of their child’s disease.ObjectiveTo determine the psychosocial problems of parents of thalassemic children.MethodsThis cross sectional study was conducted among the parents of thalassemic children attending THALASSEMIA CENTRE, BAHAVAL VICTORIA HOSPITAL (BVH), BAHAWALPUR, PAKISTAN during the year 2011. A self designed questionnaire was used that contained questions regarding psychological and social aspects. Patient Health Questionnaire-9 (PHQ-9) was used to assess the depression of parents of thalassemic children.ResultsOf the 100 parents interviewed, the majority were mothers (71%) , with a mean age of 32 ± 8.07 years for both sexes. 29 percent of the parents had moderate to severe depression, 16 percent had sleep disturbances. 56 percent were downgraded by relatives. There was a significant relationship between respondent education and depression (p < 0.05).ConclusionA substantial number of parents have psychosocial problems due to the disease of their child. Parent counseling is needed on regular basis.

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Growing up with the families of B-thalassaemia major using an accelerated longitudinal design

Cited by 14 publications

References 0 publications

Cognitive functions in adults with β‐thalassemia major: before and after blood transfusion and comparison with healthy controls

Cognitive functions in adults with β‐thalassemia major: before and after blood transfusion and comparison with healthy controls

Brain functional impairment in beta‐thalassaemia: the cognitive profile in Italian neurologically asymptomatic adult patients in comparison to the reported literature

Psychosocial problems of Pakistani parents of Thalassemic children: a cross sectional study done in Bahawalpur, Pakistan

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