title: Intellectual function evaluation of first generation immigrant children with sickle cell disease: the role of language and sociodemographic factors.
abstract: BACKGROUND
  Sickle Cell Disease (SCD) is the most common genetic disease worldwide. Neurological events are among the most worrisome clinical complications of SCD and are frequently accompanied by cognitive impairment. Intellectual function in SCD may vary according to genetic and environmental factors. Immigrant children with SCD are increasing at a global level and display specific health care needs. The aim of our multicenter study was to describe the intellectual function of first generation African immigrants with SCD and the influence of sociodemographic factors on its characteristics.
  
  
  METHODS
  The Wechsler Intelligence Scales were administered to evaluate broad intellectual functions in children with SCD and in age-matched healthy siblings. Patients' clinical, socio-demographic, Magnetic Resonance Imaging (MRI) and Angiography (MRA) data were correlated to intellectual function scores.
  
  
  RESULTS
  68 children, mean age 8.95 years were evaluated. 72% spoke three languages, 21% two. FSIQ was <75 in 25% of the children. Mean VIQ was lower than PIQ in 75%. Mean verbal subtest scores were lower than performance scores. Female gender, number of languages spoken at home and mother's employment were associated with single subtest performances (p < 0.05). MRA was abnormal in 73.4% and MRI in 35.9%. No significant correlation was established between silent lesions and intellectual function, even if patients with lesions performed worse. Fifteen siblings performed better than patients on cognitive domains, including language (p < 0.05).
  
  
  CONCLUSIONS
  Immigrant bilingual children with SCD seem to display a rate of cognitive impairment similar to their monolingual counterparts but a more pronounced and precocious onset of language difficulties. Adjunctive tests need to be considered in this group of patients to better define their specific deficits.
authors.affiliation: Clinic of Pediatric Hematology-Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, Padua, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Montanaro
authors.forename: Raffaella
authors.initials: R
authors.lastname: Colombatti
authors.forename: Marisa
authors.initials: M
authors.lastname: Pugliese
authors.forename: Camilla
authors.initials: C
authors.lastname: Migliozzi
authors.forename: Fabiana
authors.initials: F
authors.lastname: Zani
authors.forename: Maria Elena
authors.initials: ME
authors.lastname: Guerzoni
authors.forename: Sheila
authors.initials: S
authors.lastname: Manoli
authors.forename: Renzo
authors.initials: R
authors.lastname: Manara
authors.forename: Giorgio
authors.initials: G
authors.lastname: Meneghetti
authors.forename: Patrizia
authors.initials: P
authors.lastname: Rampazzo
authors.forename: Francesca
authors.initials: F
authors.lastname: Cavalleri
authors.forename: Marco
authors.initials: M
authors.lastname: Giordan
authors.forename: Paolo
authors.initials: P
authors.lastname: Paolucci
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Basso
authors.forename: Giovanni
authors.initials: G
authors.lastname: Palazzi
authors.forename: Laura
authors.initials: L
authors.lastname: Sainati
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications / Q000175:diagnosis / Q000208:ethnology* / Q000523:psychology*; D044383:Black People / Q000706:statistics & numerical data*; D002648:Child; D003072:Cognition Disorders / Q000175:diagnosis / Q000208:ethnology* / Q000209:etiology / Q000523:psychology*; D054242:Emigrants and Immigrants; D005260:Female; D006801:Humans; D007558:Italy / Q000453:epidemiology; D007802:Language; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008297:Male; D011203:Poverty; D011237:Predictive Value of Tests; D012680:Sensitivity and Specificity; D012959:Socioeconomic Factors; D014888:Wechsler Scales