Intellectual function evaluation of first generation immigrant children with sickle cell disease: the role of language and sociodemographic factors

Montanaro, Maria; Colombatti, Raffaella; Pugliese, Marisa; Migliozzi, Camilla; Zani, Fabiana; Guerzoni, Maria Elena; Manoli, Sheila; Manara, Renzo; Meneghetti, Giorgio; Rampazzo, Patrizia; Cavalleri, Francesca; Giordan, Marco; Paolucci, Paolo; Basso, Giuseppe; Palazzi, Giovanni; Sainati, Laura

doi:10.1186/1824-7288-39-36

Cited by 26 publications

(28 citation statements)

References 26 publications

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“…MRI and MRA studies were performed as previously described [22]. Cardiac ultrasound and TRV measurement were performed as previously described [23].…”

Section: Methodsmentioning

confidence: 99%

Coagulation Activation in Children with Sickle Cell Disease Is Associated with Cerebral Small Vessel Vasculopathy

et al. 2013

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BackgroundThrombotic complications in Sickle Cell Disease (SCD) arise since infancy, but the role of the coagulation system in children has been poorly explored. To determine its role in the development of clinical complications in childhood we measured coagulation and endothelial parameters in children with SCD at steady state.MethodsMarkers of thrombin generation, fibrin dissolution and endothelial activation were evaluated in 38 children with SS-Sβ°, 6 with SC disease and 50 age and blood group matched controls. Coagulation variables were correlated with markers of hemolysis and inflammation, with the presence of cerebral and lung vasculopathy and with the frequency of clinical complications.ResultsSS-Sβ° patients presented higher levels of factor VIII, von Willebrand factor antigen (VWF:Ag) and collagen binding activity, tissue plasminogen activator antigen (t-PA:Ag), D-dimer, p-selectin, prothrombin fragment1+2 (F1+2) and lower ADAMTS-13:activity/VWF:Ag (p<0.05) compared to controls and SC patients. In SS-Sβ° patients coagulation variables correlated positively with markers of inflammation, hemolysis, and negatively with HbF (p<0.05). Patients with cerebral silent infarcts showed significant decrease in t-PA:Ag and ADAMTS-13 Antigen and a tendency toward higher D-dimer, F1+2, TAT compared to patients without them. D-dimer was associated with a six fold increased risk of cerebral silent infarcts. No correlation was found between coagulation activation and large vessel vasculopathy or other clinical events except for decreased t-PA:Ag in patients with tricuspid Rigurgitant Velocity >2.5m/sec.ConclusionsSS-Sβ° disease is associated with extensive activation of the coagulation system at steady state since young age. ADAMTS-13 and t-PA:Ag are involved in the development of cerebral silent infarcts.

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“…MRI and MRA studies were performed as previously described [22]. Cardiac ultrasound and TRV measurement were performed as previously described [23].…”

Section: Methodsmentioning

confidence: 99%

Coagulation Activation in Children with Sickle Cell Disease Is Associated with Cerebral Small Vessel Vasculopathy

et al. 2013

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“…Vários estudos tem mostrado que o AVE nestes indivíduos acarreta prejuízos cognitivos verificados por pobre desempenho em testes de quociente intelectual (QI), habilidades cristalizadas, memória de trabalho, atenção, velocidade de processamento e em tarefas que requerem funções executivas, tais como organização, planejamento, processamento sequencial, inibição, monitoramento de respostas, tomada de decisão, julgamento, flexibilidade mental e memória de trabalho (SCHATZ et al, 2002;HOGAN et al, 2006;ANGULO, 2007;BERKELHAMMER et al, 2007;MCCAULEY;PEDROZA, 2010;WILLS et al, 2010;HIJMANS et al, 2011;BERG;KING, 2012;MONTANARO et al, 2013). Alterações em habilidades sensório-motoras foram relacionadas ao histórico de AVE (RUFFIEUX et al, 2013).…”

Section: Doença Falciforme E Aves: Manifestações Clínicasunclassified

“…Há diferença no desempenho em teste de QI verbal, de execução e total de crianças com Doença Falciforme sem alteração em exame de neuroimagem quando comparadas com crianças saudáveis (BERKELHAMMER et al, 2007). Em um estudo italiano (MONTANARO et al, 2013), foi relatado pior desempenho em avaliação de QI verbal, indicando alterações de linguagem e verbais em crianças com a doença. Há indícios de que ocorre menor prejuízo nas funções cognitivas de crianças com Doença Falciforme na ausência de AVEs do que quando analisado na presença desses eventos (SCHATZ et al, 2002).…”

Section: Doença Falciforme E Aves: Manifestações Clínicasunclassified

“…A linguagem é descrita na literatura como uma das habilidades prejudicadas nas crianças com Doença Falciforme (DAVIS et al, 1997;TARAZI et al, 2007;NUNEST et al, 2010) e, é até relacionada à alterações em avaliações de QI verbal (VICHINSKY et al, 2010;MONTANARO et al, 2013).…”

Section: Doença Falciforme E Aves: Manifestações Clínicasunclassified

“…Não houve correlação significativa entre o nível socioeconômico e o nível de Hb ou sintomas da Doença Falciforme de diferentes genótipos (HbSS, HbSC e Hbβ + ).Baixos escores em testes de QI verbal do que de execução em crianças com Doença Falciforme, em idade escolar, foram relacionados às faltas escolares, sendo o baixo desempenho considerado efeito indireto à um fator psicossocial e não uma manifestação da doença. A presença de fatores sócio culturais, principalmente em países com imigrantes Problemas comportamentais também são descritos na literatura, tais como: lentidão na execução dos testes; baixa alta-estima; ansiedade; dificuldade em compreender a tarefa; impulsividade; comportamento opositivo; déficits de atenção; hiperatividade; ansiedade;depressão(WILLS et al, 2010;MONTANARO et al, 2013).Alguns problemas são mais comuns na adolescência, entre ele podem ser citados também o tabagismo, uso de álcool e de drogas e a gravidez. Nesta época da vida, a manutenção do acompanhamento médico torna-se um desafio.…”

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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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Randomization is not associated with socio-economic and demographic factors in a multi-center clinical trial of children with sickle cell anemia

Roberts

Covert

Rodeghier³

et al. 2014

Pediatr Blood Cancer

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Background Few studies have investigated factors influencing participation rates for minority children with a chronic disease in clinical trials. The Silent Cerebral Infarct Multi-Center Clinical (SIT) Trial provides an opportunity to study the impact of demographic and socio-economic factors on randomization in a clinical trial among Black children. Our primary objective was to characterize the factors associated with successful randomization of children with sickle cell disease (SCD) and silent cerebral infarct (SCI) in the SIT Trial after initial consent. Procedure Differences in socio-economic and demographic variables, family history and disease-related variables were determined between eligible participants who were successfully randomized and those who were not randomized following initial consent. Head of household educational level and family income were examined separately for US versus non-US sites. Results Of 1,176 children enrolled in the SIT Trial, 1016 (86%) completed screening. Of 208 (20%) children with SCI on screening MRI, 196 (94%) were successfully randomized. There were no differences in socio-economic, demographic or disease-related variables between children who were or were not randomized. Participants from non-US sites were more likely to be randomized (22% vs. 12%, p = 0.011), although randomization by country was associated with neither head of household education nor family income. Conclusion In the SIT Trial, randomization after initial consent does not appear to be associated with socio-economic or demographic factors. Although these factors may represent barriers for some participants, they should not bias investigators caring for children with SCD in their approach to recruitment for clinical trial participation.

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Intellectual function evaluation of first generation immigrant children with sickle cell disease: the role of language and sociodemographic factors

Cited by 26 publications

References 26 publications

Coagulation Activation in Children with Sickle Cell Disease Is Associated with Cerebral Small Vessel Vasculopathy

Coagulation Activation in Children with Sickle Cell Disease Is Associated with Cerebral Small Vessel Vasculopathy

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

Randomization is not associated with socio-economic and demographic factors in a multi-center clinical trial of children with sickle cell anemia

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