Abstract
ABSTRACTObjective: To evaluate sociodemographic and clinical aspects of children with sickle cell disease (SCD) and their behavioral characteristics.Methods: Interview with parents of patients with SCD from four to ten years old, addressing socioeconomic aspects and other health conditions, and using the Strengths and Difficulties Questionnaire (SDQ). Clinical data were obtained from medical records. Exclusion criteria were the use of hydroxyurea, previous diagnosis of stroke, chronic encephalopathy and/or intellectual disability.Results: 45 patients (19 girls and 26 boys) were assessed. The median age was seven years. Diagnosis of SCD: 26 hemoglobinopathy SC; 19 hemoglobinopathy SS. Socioeconomic class: D: 24.4%; C2: 44.4%; C1: 28.9%; B2: 2.2%. Clinical history: acute chest syndrome: 40%; transfusions: 66.7%; hospitalizations: 82.2%. SDQ findings: 88.9% clinical impact (emotional subscale: 68.9%); total score: impact in 48.9%. It was not possible to establish a relation between the severity of the disease and the results of the SDQ. Regarding socioeconomic class: among individuals of classes B2 and C1, 21.4% had impact at the total score; in classes C2 and D, this percentage was 61.3%. Regarding the schooling of the head of the family, with Elementary School at least, 39.3% of the children had impacts; for fewer education, this percentage was 64.7%.Conclusions: Behavioral impacts are highly prevalent in children with SCD. Individuals in socioeconomic classes C2 and D suffered more behavioral impacts than individuals in classes B2 and C1.
Highlights
Sickle cell disease (SCD) is one of the most frequent genetic conditions in Brazil
We must highlight the possibility of the occurrence of silent cerebral infarctions, which affects approximately 25% of children with sickle cell disease until the age of six, and up to a third of these children until the age of 14, which can lead to worse performance in cognitive tests when compared to the general population, specific functional deficiencies and impaired school performance.[3]
Considering these possible clinical manifestations in pediatric patients, studies demonstrate the relation of SCD with behavioral, emotional, academic and social interaction problems in children, which can be attributed to issues pertinent to the chronicity of the disease, and to specific issues of SCD.[2,4]
Summary
Sickle cell disease (SCD) is one of the most frequent genetic conditions in Brazil It is a monogenic disease resulting from a mutation in the gene responsible for the production of the hemoglobin A beta globin, which determines changes in the resulting hemoglobin molecule (hemoglobin S).[1] In children, SCD is the main cause of cerebrovascular accidents (CVA), occurring in 8 to 17% of patients.[2] We must highlight the possibility of the occurrence of silent cerebral infarctions, which affects approximately 25% of children with sickle cell disease until the age of six, and up to a third of these children until the age of 14, which can lead to worse performance in cognitive tests when compared to the general population, specific functional deficiencies and impaired school performance.[3] vaso-occlusive crises are the main clinical symptom of SCD and the first cause of hospitalization by patients, so that about 60% of patients with SCD have at least one episode of severe pain per year related to vaso-occlusive crises, and the first episodes can occur at around six months of age.[2,3]. Considering these possible clinical manifestations in pediatric patients, studies demonstrate the relation of SCD with behavioral, emotional, academic and social interaction problems in children, which can be attributed to issues pertinent to the chronicity of the disease (such as missing classes due to prolonged periods of hospitalization and the need for special care), and to specific issues of SCD (such as the suffering related to unpredictable painful crises and the cognitive deficit resulting from silent cerebral infarctions, for example).[2,4]
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