Cognitive and behavioral abnormalities in children after hematopoietic stem cell transplantation for severe congenital immunodeficiencies

Social outcome in children treated by haematopoietic cell transplant for congenital immunodeficiency

“…The only significant finding (between performance on Digit Span and social competence) may in fact reflect the relationship between IQ and social functioning that exists both generally 30 and in this group of children. 2 This general lack of a relationship between executive function and social competence is in Table 4 Correlations between the social competence and physical appearance measures Social outcome in children treated by HSCT E Skucek et al contrast to findings from studies of other groups of neurologically compromised children. [15][16][17] It is possible that the lack of association in this study was due to the types of measures used, which were computer-based tasks and, therefore, may not reflect the children's ability in everyday social interactions.…”

“…Children with a diagnosis of adenosine deaminase deficient SCID and Chediak-Higashi syndrome were excluded from the study because these conditions are characterized by more severe cognitive and behavioural difficulties that are markedly different from the rest of the cohort. 2 Of 41 potential participants, 31 (76%) completed the assessment.…”

“…For example, the largest long-term follow-up study showed that HCT was only associated with long-term cognitive difficulties in children treated for malignant or haematological conditions if the child had received cranial irradiation, 10 whereas children with congenital immunodeficiencies have been shown to have lower levels of general cognitive function than their healthy siblings and test norms despite the fact that they do not have cranial irradiation. 2 These difficulties were more severe in specific diagnostic groups for example, adenosine deaminase deficient-SCID and Chediak Higashi syndrome. Furthermore, among the group of children with SCID, those with a molecular diagnosis, indicating a specific immune defect, had better cognitive outcome than children with no identified underlying molecular defect.…”

“…However, studies of psychological functioning post transplant have shown that this group of children are at increased risk of both cognitive difficulties and emotional and behavioural problems in the long term, including increased rates of peer relationship difficulties. 1,2 These problems are of concern because childhood social difficulties are strongly associated with later negative outcomes in adulthood and affect the development of later relationships and social skills. 3,4 Although the reported cognitive and behavioural difficulties have been shown to be at least partly associated with the child's underlying genetic condition, these children may also be more vulnerable to social difficulties because they have a very abnormal experience in early childhood, including extended periods of illness and social isolation as part of the management and treatment of their immunodeficiency.…”

“…Evidence from studies of children treated for other chronic illnesses shows that those whose condition (or treatment) results in reduced cognitive abilities are at increased risk of social difficulties 11 and this is consistent with one study of children with immunodeficiencies that showed an association between cognitive ability and difficulties in peer relationships. 2 Studies to date have mostly used measures of general cognitive ability (for example, intelligence quotient (IQ)) rather than measuring specific cognitive functions, but it has been suggested that the executive functions of attention, concentration and working memory may be particularly associated with social difficulties. 5,12 Executive dysfunction has been shown to be the most frequent cognitive impairment in adult HCT survivors, 13,14 but has not yet been investigated in children.…”