Neurologic complications of sickle cell disease

Venkataraman, Akila; Adams, Robert J.

doi:10.1016/b978-0-7020-4087-0.00068-1

Cited by 17 publications

(14 citation statements)

References 99 publications

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“…Sickle cell patients develop splenic dysfunction early (4-6 months of age) in the course of their disease [49]. This raises the possibility that HU therapy might be able to exert a significant spleen dysfunction [42], hypoxemia [52], pulmonary hypertension [53], glomerular hyperfiltration [54], neurocognitive delay [53], silent brain infarcts [41], elevated transcranial Doppler (TCD) velocities, and primary stroke [55,56]. Furthermore, a Belgian multicenter study showed a mean hospital stay of 5.3 days in the HU-treated group and 15.2 days in the placebo group [57].…”

Section: Attenuation Of Organ Dysfunctionmentioning

confidence: 99%

Retrospection of the effect of hydroxyurea treatment in patients with sickle cell disease

Verma

Lakkakula

2018

Acta Haematologica Polonica

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Sickle cell anemia (SCA) is one of the inherited hemoglobin disorders with substantial morbidity and early mortality. Hydroxyurea is the US Food and Drug Administration (FDA)-approved medication that has emerged as the primary disease-modifying therapy for SCA. Our purpose is to summarize the available evidence regarding the pharmacology, clinical efficacy, and safety of hydroxyurea therapy for the treatment of SCA. The electronic databases PubMed and Embase were searched from their starting dates to May 31, 2016. Databases were searched using the following terms: sickle cell, hydroxyurea, nitric oxide, dosing, therapeutic, and safety monitoring. Hydroxyurea therapy may cause severe myelosuppression when used in patients with SCA. SCA patients are initially treated with hydroxyurea at 10 or 20 mg/kg, and then the dose- is escalated to mild myelosuppression using a standardized regimen. Routine blood monitoring should be performed while the patient receives hydroxyurea treatment. Hydroxyurea can increase fetal hemoglobin (HbF) level and ameliorate some of the vascular symptoms in patients with SCA. Hydroxyurea therapy may help to avoid frequent hospitalizations, especially in patients with vaso-occlusive crisis. Taken together, available evidence suggests that hydroxyurea represents an inexpensive and effective treatment option that should be offered to patients with SCA.

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Section: Attenuation Of Organ Dysfunctionmentioning

confidence: 99%

Retrospection of the effect of hydroxyurea treatment in patients with sickle cell disease

Verma

Lakkakula

2018

Acta Haematologica Polonica

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“…Notwithstanding the abundance of epidemiological evidence, the mechanism of onset of cognitive and neurobehavioral dysfunction in SCD, and the underlying cellular and pathobiological mechanisms are largely unknown. [15][16][17][18] The presence of cognitive and neurobehavioral dysfunction in individuals with SCD in the absence of overt cerebral injury 2,14 suggests the involvement of cellular mechanisms that are not detectable on clinical imaging. The mechanisms of cognitive and neurobehavioral dysfunction in SCD in the absence of overt cerebral injury is largely unknown.…”

Section: Introductionmentioning

confidence: 99%

Age and neuroinflammation are important components of the mechanism of cognitive and neurobehavioral deficits in sickle cell disease

Hardy

Rached

Jones

et al. 2020

Preprint

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Objectives. Cognitive and neurobehavioral abnormalities are the most common and complex complications of sickle cell disease (SCD). Known risk factors influencing abnormalities are stroke and silent cerebral infarcts, but a majority of cases do not have overt cerebral injury and the underlying mechanism is not well understood. This study aims to determine whether sickle cell mice could recapitulate features of cognitive and neurobehavioral impairment observed in sickle cell patients as well as to determine the underlying cellular mechanism of these SCD complications.Methods. Using a longitudinal cross-sectional study design, we evaluated cognition and neurobehavioral deficits as an outcome. Six as well as 13 months old male Townes humanized sickle cell (SS) and matched control (AA) mice were tested. The combination of novel object recognition and fear conditioning tests was employed to measure anxiety/depression, learning and memory. Immunohistochemistry was performed to quantify bone marrow-derived microglia (CD45 + ) and activated microglia (Iba1 + ) in the dentate and peri-dentate gyrus to determine if these factors were potential pathogenic mechanisms associated with cognitive and neurobehavioral abnormalities. We evaluated neurogenesis by measuring 5'Bromodeoxyuridine (BrdU) and doublecortin (DCX) and phenotyped proliferating cells via quantification of glial fibrillary acid protein (GFAP + ), neuronal nuclei (NeuN + ), CD45 + and Iba1 + . In addition, Golgi-Cox staining was used to assess neuroplasticity via measurement of dendritic spine density and morphology, as well as dendrite arbors.Results. Compared to matched AA, 13 months old SS mice showed significant evidence of anxiety/depression by the shorter distance traveled as well as thigmotaxis. Additionally, SS mice were significantly less likely to recognize the novel object as well as have a reduced preference for the novel object. There were no significant differences between 6 months old SS and AA.But the difference reappeared after the same mice were aged to 13 months. Aged mice exhibited more anxiety/depression behaviors and thigmotaxis and were less likely to recognize or show a lower percent preference for the novel object compared to aged control (AA) mice.Immunohistochemistry analysis shows that sickle cell (SS) mice had significantly more CD45 + and Iba1 + activated microglia cells in the dentate and peri-dentate gyrus area compared to AA mice. SS mice also had a significantly lower dendritic spine density compared with controls.Treatment of aged SS mice with minocycline resulted in significant improvement of cognitive and neurobehavioral function compared to matched vehicle-treated SS mice. Also immunohistochemical and histological analysis showed that treated SS mice had significantly fewer CD45 + cells and activated microglia in the dentate and peri-dentate gyrus area.Furthermore, there was significant improvement in dendritic spine and dendrite arbor density as well as spine maturation in treated sickle cell mice compared with vehicle-trea...

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“…Alterações em habilidades sensório-motoras foram relacionadas ao histórico de AVE (RUFFIEUX et al, 2013). Os AVEs silenciosos também são a causa de alterações neurocognitivas e fator de risco para AVEs subsequentes (SCHATZ; MCCLELLAN, 2006;VENKATARAMAN;ADAMS, 2014). A lesão isquêmica "silenciosa" ou "infarto silencioso" pode causar várias deficiências neurocognitivas e afetar os lobos frontais causando déficits de atenção, dificuldades ou defasagens nas habilidades executivas e na memória de curto e longo prazo (ANGULO, 2007).…”

Section: Doença Falciforme E Aves: Manifestações Clínicasunclassified

“…HOGAN et al, 2006;BRASIL, 2009), com diferenças nas manifestações clínicas (GUEGUEN et al, 2014). Diferente do portador homozigoto para a HbSS, os indivíduos com o tipo HbSC geralmente apresentam condições clínicas benignas (HOGAN et al, 2006;WANG, 2007;VENKATARAMAN;ADAMS, 2014). Este dado deve ser analisado com cautela, pois apesar da expectativa de condições clínicas benignas, a sintomatologia é variável e estes indivíduos são crianças de risco para alterações do desenvolvimento, além das sequelas clínicas da doença.…”

Section: Doença Falciforme E Aves: Manifestações Clínicasunclassified

“…Suas complicações neurológicas podem ser causadas por AVE, ataques isquêmicos transitórios ou infartos cerebrais silenciosos (SCHATZ; MCCLELLAN, 2006;TARAZI et al, 2007;BROUSSEAU;PANEPINTO, 2009;REES;GLADWIN, 2010;VENKATARAMAN;ADAMS, 2014). A consequência do efeito cumulativo destas complicações caracteriza-se por alterações no desempenho cognitivo, linguístico, pessoalsocial e motor fino, levando a diminuição do rendimento acadêmico ou abandono escolar, que repercutirão na possibilidade destes indivíduos obterem melhor inserção no mercado de trabalho, além do resultante sofrimento psicoafetivo (DAVIS et al, 1997;SCHATZ, 2003;BERKELHAMMER et al, 2007;ROBERTS, 2007;TARAZI et al, 2007;BROUSSEAU;PANEPINTO, 2009;BOULET et al, 2010;NUNEST et al, 2010;HIJMANS et al, 2011;KNIGHT-MADDEN et al, 2011;SANTOS, 2013; VENKATARAMAN; ADAMS, 2014).…”

Section: Introductionunclassified

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Habilidades do desenvolvimento infantil em crianças com Doença Falciforme: enfoque na linguagem

Gejão¹

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Child development skills in sickle cell disease: focus on language The Sickle Cell Disease is an inherited autosomal recessive hemolytic alteration with great clinical variability and epidemiological importance involving african descent. Acute complications are usually related to vascular occlusion, causing musculoskeletal and abdominal pain and chronic involvement of multiple organs and systems. These complications involve the central nervous system, causing strokes or silent cerebral infarcts. There is evidence that the cumulative effect of these complications associated with environmental factors and genotype interfere with cognitive, language, personal-social and motor performance, leading to decreased academic performance at school age and reflecting on the quality of life of these individuals. The objectives of this study were to verify the child development skills, with a focus on language, in preschool children with sickle cell disease without proven stroke compared to healthy children for the disease; verify the influence of motor, personal-social and cognitive aspects on language development; and verify the influence of environmental factors on child development. Thirty-four children (17 with Sickle Cell Disease-SCDG and 17 without the disease-Control Group: CG) with chronological age between one and five years and 11 months, underwent assessment of motor, cognitive, personal-social, linguistic and psycholinguistic skills through the following instruments, according to their age: DENVER

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Neurologic complications of sickle cell disease

Cited by 17 publications

References 99 publications

Retrospection of the effect of hydroxyurea treatment in patients with sickle cell disease

Retrospection of the effect of hydroxyurea treatment in patients with sickle cell disease

Age and neuroinflammation are important components of the mechanism of cognitive and neurobehavioral deficits in sickle cell disease

Habilidades do desenvolvimento infantil em crianças com Doença Falciforme: enfoque na linguagem

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