Cognitive profile of children with sickle cell anemia compared to healthy controls

Castro, Isabel Pimenta Spínola; Viana, Marcos Borato

doi:10.1016/j.jped.2018.04.012

Cited by 16 publications

(9 citation statements)

References 24 publications

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“…[33] The low cognitive in sicklers showed adverse effect on the socioeconomically status of patients with SCA. [34]…”

Section: Discussionmentioning

confidence: 99%

The impact of sickle cell anemia on the quality of life of sicklers at school age

Salih

2019

J Family Med Prim Care

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Background:Sickle cell anemia (SCA) is prevalent in Sudan in general, and in particular in the western part of the country among Misseria tribe. School absence, depressive symptoms, embarrassment, social disturbances, and emotional disturbance present negative impact for sicklers. Therefore, an urgent call for improving the quality of life of sicklers is needed.Objectives:To examine the impact of SCA on the quality of life of school-age children affected by the disease from the perspective of three dimensions: psychological, social, and schooling.Materials and Methods:This is a prospective, cross-sectional, observational study carried out over 2 weeks’ period in April 2011 in Western Sudan, Gineana District. This study included 107 patients within the age group of 7–15 years with confirmed diagnosis of SCA in a steady state. A sickler with SCA who had hemoglobin genotype “SS” on hemoglobin electrophoresis without crises over the past 4 weeks was considered to be in steady state. Questionnaires designed by expert covering social and psychological aspects of the disease were filled by the authors.Results:Of 107 patients with confirmed diagnosis of SCA in steady state, 54.2% of them were male. About 17.8%, 29.6%, 23.4%, 48.6%, 16.8%, and 45.8% of the patients suffered from school absence, teasing, embarrassment due to bedwetting, embarrassment due to jaundice, failure to contribute to school activities such as sport, and depressive symptoms, respectively. Around 8.4% of the patients repeated classes. Divorce and anxiety among sicklers’ families were found in 6.5% and 8.4%, respectively. Twenty-one (19.6%) families had less interest in social activities in the community.Conclusion:Sickle cell disease has many social and psychological problems which need to be addressed. Enuresis, depressive symptoms, school absentees, and deterioration in school performance were the common problems encountered.

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“…[33] The low cognitive in sicklers showed adverse effect on the socioeconomically status of patients with SCA. [34]…”

Section: Discussionmentioning

confidence: 99%

The impact of sickle cell anemia on the quality of life of sicklers at school age

Salih

2019

J Family Med Prim Care

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“…Sickle cell disease (SCD) is a genetic disorder characterised by recurrent acute vaso-occlusive pain and progressive endorgan dysfunction, including the brain (Ware et al, 2017;Kato et al, 2018). Patients with SCD are at increased risk for neurocognitive deficits when compared to healthy controls matched for age, race and/or socioeconomic factors (Schatz et al, 2002;Steen et al, 2005;Castro & Viana, 2019). This gap appears to widen with age, particularly for patients with the HbSS genotype (Knight et al, 1995), recurrent vaso-occlusive pain (Glass et al, 2013), silent cerebral infarct (SCI) (Wang et al, 2001) or severe anaemia and thrombocytosis (Bernaudin et al, 2000).…”

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confidence: 99%

Association between hydroxycarbamide exposure and neurocognitive function in adolescents with sickle cell disease

et al. 2020

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Summary Patients with sickle cell disease (SCD) are at increased risk for neurocognitive impairments. While disease‐modifying treatment, such as hydroxycarbamide (hydroxyurea), may decrease this risk, it has not been systematically investigated in children with SCD. We screened neurocognitive functioning in 103 adolescents with SCD (16–17 years, 50% female) and compared outcomes between patients with a history of exposure to hydroxycarbamide (n = 12 HbSC/HbSβ+ thalassaemia; n = 52 HbSS/HbSβ0 thalassaemia) and those never treated with hydroxycarbamide (n = 31 HbSC/HbSβ+ thalassaemia; n = 8 HbSS/HbSβ0 thalassaemia). Demographic distributions were similar between the groups. After adjusting for socioeconomic status, the hydroxycarbamide group had significantly higher scores on nonverbal IQ (HbSC/HbSβ thalassaemia: P = 0·036, effect size [d] = 0·65), reaction speed (HbSS/HbSβ0 thalassaemia: P = 0·002, d = 1·70), sustained attention (HbSS/HbSβ0 thalassaemia: P = 0·014, d = 1·30), working memory (HbSC/HbSβ+ thalassaemia: P = 0·034, d = 0·71) and verbal memory (HbSC/HbSβ+ thalassaemia: P = 0·038, d = 0·84) when compared to those who did not receive hydroxycarbamide. In patients with HbSS/HbSβ0 thalassaemia, longer treatment duration with hydroxycarbamide was associated with better verbal memory (P = 0·009) and reading (P = 0·002). Markers of hydroxycarbamide effect, including higher fetal haemoglobin (HbF), higher mean corpuscular volume (MCV) and lower white blood cell count (WBC), were associated with better verbal fluency (HbF: P = 0·014, MCV: P = 0·006, WBC: P = 0·047) and reading (MCV: P = 0·021, WBC: P = 0·037). Cognitive impairment may be mitigated by exposure to hydroxycarbamide in adolescents with SCD.

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“…(MeSH) es la enfermedad hematológica hereditaria más común con una distribución heterogénea, siendo frecuentemente encontrada en la población con ascendencia africana (12,13). El genotipo predominante es el homocigoto (HbSS) (14).…”

Section: Epidemiologíaunclassified

Anemia falciforme y la resistencia a la malaria. Revisión narrativa

Isaza

Herrera-Almanza

Hernández-Martínez

et al. 2020

Rev. Fac. Cienc. Salud Univ. Cauca

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La enfermedad de células falciformes (SCD, por sus siglas en inglés) es una hemoglobinopatía autosómica recesiva, producida por la mutación del gen de la cadena β-globina que genera la sustitución de valina por el ácido glutámico en la posición del sexto aminoácido de la hemoglobina. Existen diversos mecanismos moleculares y celulares por los cuales se explica su fisiopatología y gracias a su actual entendimiento, se encuentran en investigación clínica y preclínica nuevos agentes farmacológicos. La hemoglobina falciforme (HbS), que es la consecuencia de dicha mutación, se relaciona como una forma de protección contra la malaria en los heterocigotos; sin embargo, el estado homocigoto (HbSS) puede estar asociado con una mayor susceptibilidad a la malaria. Dicha relación de protección en heterocigotos se evidencia en niveles más bajos de parasitemia y de aparición más tardía, lo que se ha tratado de explicar por diversas teorías. Por tanto, este artículo tiene como objetivo revisar la relación entre la SCD y la protección que genera en infección por malaria, además de exponer la fisiopatología y el tratamiento de la SCD para lograr un entendimiento más amplio de la enfermedad.

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Cognitive profile of children with sickle cell anemia compared to healthy controls

Abstract: The cognitive impairment of children with sickle cell anemia is severe and manifests even when the disease effect is adjusted to the socioeconomic status. In the authors' view, such impairment requires an early preventive approach in order to avoid this cognitive damage.

Cited by 16 publications

References 24 publications

The impact of sickle cell anemia on the quality of life of sicklers at school age

The impact of sickle cell anemia on the quality of life of sicklers at school age

Association between hydroxycarbamide exposure and neurocognitive function in adolescents with sickle cell disease

Anemia falciforme y la resistencia a la malaria. Revisión narrativa

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