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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 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 Citation Map 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. Related Collections Transplantation Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  TRANSPLANTATION Cognitive and behavioral abnormalities in children after hematopoietic stem cell transplantation for severe congenital immunodeficiencies Penny Titman1, Elizabeth Pink1, Emily Skucek1, Katherine O'Hanlon1, Tim J. Cole2, Jane Gaspar3, JinHua Xu-Bayford3, Alison Jones3,4, Adrian J. Thrasher3,4, E. Graham Davies3,4, Paul A. Veys5, and H. Bobby Gaspar3,4 1 Department of Psychosocial Services, Great Ormond Street Hospital National Health Service (NHS) Trust, London; 2 Centre for Paediatric Epidemiology and Biostatistics, University College London (UCL) Institute of Child Health, London; 3 Department of Immunology, Great Ormond Street Hospital NHS Trust, London; 4 Molecular Immunology Unit, UCL Institute of Child Health, London; and 5 Department of Bone Marrow Transplantation, Great Ormond Street Hospital NHS Trust, London, United Kingdom Hematopoietic stem cell transplantation (HSCT) is a highly successful treatment for severe congenital immunodeficiencies. However, some studies have suggested that children may experience cognitive difficulties after HSCT. This large-scale study assessed cognitive and behavioral function for the cohort of children treated by HSCT at one center between 1979 and 2003 to determine the frequency and severity of problems and to identify risk factors. A total of 105 patients were assessed on standardized measures of cognitive and emotional and behavioral function together with a control group of unaffected siblings. The average IQ for the cohort was 85 (95% confidence interval, 81-90), significantly lower than both the population average of 100 (P < .001) and unaffected siblings. Multivariate analysis indicated that the underlying genetic defect, diagnosis of adenosine deaminase-deficient severe combined immunodeficiency, and consanguinity were associated with worse outcome but that age at transplantation 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 behavioral 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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