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Rev Bras Hematol Hemoter. 2017 Jan - Mar;39(1):32-39. doi: 10.1016/j.bjhh.2016.09.004. Epub 2016 Oct 07.

Comprehensive neuropsychological evaluation of children and adolescents with sickle cell anemia: a hospital-based sample.

Revista brasileira de hematologia e hemoterapia

Samantha Nunes, Nayara Argollo, Marivania Mota, Camilo Vieira, Eduardo Pondé de Sena

Affiliations

  1. Universidade Federal da Bahia (UFBA), Salvador, BA, Brazil. Electronic address: [email protected].
  2. Universidade Federal da Bahia (UFBA), Salvador, BA, Brazil.

PMID: 28270343 PMCID: PMC5339374 DOI: 10.1016/j.bjhh.2016.09.004

Abstract

BACKGROUND: Individuals with sickle cell anemia may suffer symptomatic or silent cerebral infarcts leading to neurocognitive complications. This study investigated the cognitive and intellectual performance of children and adolescents with sickle cell anemia.

METHODS: The socioeconomic status, clinical aspects and behavioral profile of 15 young individuals with sickle cell anemia were evaluated. The Wechsler Intelligence Scale for Children, the Developmental Neuropsychological Assessment Test, and the Child Behavior Checklist were applied.

RESULTS: Participants with a history of stroke had lower intelligence quotient (IQ) scores. Alterations were found in attention and executive functioning, language, verbal and visual memory, visuospatial processing and sensorimotor skills. These alterations were found both in the children and adolescents who had had a cerebral infarction and in those who apparently had not. In the majority of cases, there were learning difficulties, a history of repeating school years and a need for specialist educational support. The most common additional diagnoses in accordance with the Diagnostic and Statistical Manual of Mental Disorders IV were depressive disorder, anxiety disorder and somatic disorder, as well as conditions associated with physical and psychosocial repercussions of sickle cell anemia.

CONCLUSION: As sickle cell anemia is considered a progressive cerebral vasculopathy, it is a potential risk factor for neurocognitive and psychosocial development. Therefore, periodic neuropsychological and behavioral evaluations of children and adolescents with sickle cell anemia may represent a useful measure to reduce long-term biopsychosocial repercussions.

Copyright © 2016 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier Editora Ltda. All rights reserved.

Keywords: Anemia, sickle cell; Behavioral disorders; Intelligence tests; Neurobehavioral manifestations; Neuropsychological tests

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