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Blood, 1 November 2008, Vol. 112, No. 9, pp. 3907-3913. Prepublished online as a Blood First Edition Paper on July 21, 2008; DOI 10.1182/blood-2008-04-151332. This Article Full Text (PDF) Supplemental Tables All Versions of this Article: blood-2008-04-151332v1 112/9/3907    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Titman, P. Articles by Gaspar, H. B. Search for Related Content PubMed PubMed Citation Articles by Titman, P. Articles by Gaspar, H. B. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted April 15, 2008 Accepted June 16, 2008 Cognitive and behavioural abnormalities in children following haematopoietic stem cell transplantation for severe congenital immunodeficiencies Penny Titman*, Elizabeth Pink, Emily Skucek, Katherine O'Hanlon, Tim J Cole, Jane Gaspar, JinHua Xu-Bayford, Alison Jones, Adrian J. Thrasher, E Graham Davies, Paul A. Veys, and H. Bobby Gaspar Psychosocial Services, Great Ormond Street Hospital, London, United Kingdom Centre for Paediatric Epidemiology and Biostatistics, UCL Institute of Child Health, London, United Kingdom Immunology Department, Great Ormond Street Hospital, London, United Kingdom Molecular Immunology Unit, UCL Institute of Child Health, London, United Kingdom Dept of Bone Marrow Transplant, GOSH, London, United Kingdom * Corresponding author; email: titmap{at}gosh.nhs.uk. Haematopoietic stem cell transplantation (HSCT) is a highly successful treatment for severe congenital immunodeficiencies. However, some studies have suggested that children may experience cognitive difficulties following HSCT. This large scale study assessed cognitive and behavioural function for the cohort of children treated by HSCT at one centre between 1979 - 2003 to determine the frequency and severity of problems and to identify risk factors. 105 patients were assessed on standardized measures of cognitive and emotional and behavioural function together with a control group of unaffected siblings. The average IQ for the cohort was 85 (95 percent confidence interval 81 - 90), significantly lower than both the population average of 100 (p<0.001) and unaffected siblings. Multivariate analysis indicated that the underlying genetic defect, diagnosis of ADA deficient severe combined immunodeficiency and consanguinity were associated with worse outcome, but that age at transplant and chemotherapy conditioning were not. Children treated by HSCT for severe immunodeficiency have an increased risk of long term cognitive difficulties and associated emotional and behavioural difficulties. The specific genetic diagnosis, consanguinity and severe clinical course are associated with poor outcome. Long term follow up of these patients should include screening to identify and manage these problems more effectively. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: H. B. Gaspar, A. Aiuti, F. Porta, F. Candotti, M. S. Hershfield, and L. D. Notarangelo How I treat ADA deficiency Blood, October 22, 2009; 114(17): 3524 - 3532. [Abstract] [Full Text] [PDF] B. Neven, S. Leroy, H. Decaluwe, F. Le Deist, C. Picard, D. Moshous, N. Mahlaoui, M. Debre, J.-L. Casanova, L. Dal Cortivo, et al. Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency Blood, April 23, 2009; 113(17): 4114 - 4124. [Abstract] [Full Text] [PDF]

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