Cognitive and behavioral abnormalities in children after hematopoietic stem cell transplantation for severe congenital immunodeficiencies
| Autor: | Tim J Cole, Jinhua Xu-Bayford, Paul Veys, Jane Gaspar, Alison Jones, E. Graham Davies, H. Bobby Gaspar, Katherine O'Hanlon, Emily Skucek, Adrian J. Thrasher, Elizabeth Pink, Penny Titman |
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| Rok vydání: | 2008 |
| Předmět: |
Adult
Male Pediatrics medicine.medical_specialty Time Factors Adolescent Adenosine Deaminase medicine.medical_treatment Intelligence Immunology Population Child Behavior Disorders Hematopoietic stem cell transplantation Biochemistry Cohort Studies Consanguinity Humans Medicine Affective Symptoms Child education Immunodeficiency education.field_of_study business.industry Cognitive disorder Age Factors Hematopoietic Stem Cell Transplantation Immunologic Deficiency Syndromes Case-control study Cell Biology Hematology medicine.disease Transplantation Case-Control Studies Child Preschool Cohort Female Severe Combined Immunodeficiency Cognition Disorders business Follow-Up Studies Cohort study |
| Zdroj: | Blood. 112:3907-3913 |
| ISSN: | 1528-0020 0006-4971 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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