Adaptive Behavior in Young Children with Neurofibromatosis Type 1

Neurofibromatosis-1 is the most common single gene disorder affecting 1 in 3000. In children, it is associated not only with physical features but also with attention and learning problems. Research has identified a downward shift in intellectual functioning as well, but to date, there are no publis...

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Main Authors: Bonita P. Klein-Tasman, Alina M. Colon, Natalie Brei, Faye van der Fluit, Christina L. Casnar, Kelly M. Janke, Donald Basel, Dawn H. Siegel, Jasmine A. Walker
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:International Journal of Pediatrics
Online Access:http://dx.doi.org/10.1155/2013/690432
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author Bonita P. Klein-Tasman
Alina M. Colon
Natalie Brei
Faye van der Fluit
Christina L. Casnar
Kelly M. Janke
Donald Basel
Dawn H. Siegel
Jasmine A. Walker
author_facet Bonita P. Klein-Tasman
Alina M. Colon
Natalie Brei
Faye van der Fluit
Christina L. Casnar
Kelly M. Janke
Donald Basel
Dawn H. Siegel
Jasmine A. Walker
author_sort Bonita P. Klein-Tasman
collection DOAJ
description Neurofibromatosis-1 is the most common single gene disorder affecting 1 in 3000. In children, it is associated not only with physical features but also with attention and learning problems. Research has identified a downward shift in intellectual functioning as well, but to date, there are no published studies about the everyday adaptive behavior of children with NF1. In this study, parental reports of adaptive behavior of 61 children with NF1 ages 3 through 8 were compared to an unaffected contrast group (n=55) that comprised siblings and community members. Significant group differences in adaptive skills were evident and were largely related to group differences in intellectual functioning. In a subsample of children with average-range intellectual functioning, group differences in parent-reported motor skills were apparent even after controlling statistically for group differences in intellectual functioning. The implications of the findings for the care of children with NF1 are discussed.
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publishDate 2013-01-01
publisher Wiley
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series International Journal of Pediatrics
spelling doaj-art-0dedb7fcd5f040218b665dc25a699e3f2025-08-20T02:02:30ZengWileyInternational Journal of Pediatrics1687-97401687-97592013-01-01201310.1155/2013/690432690432Adaptive Behavior in Young Children with Neurofibromatosis Type 1Bonita P. Klein-Tasman0Alina M. Colon1Natalie Brei2Faye van der Fluit3Christina L. Casnar4Kelly M. Janke5Donald Basel6Dawn H. Siegel7Jasmine A. Walker8University of Wisconsin - Milwaukee, Milwaukee, WI 53201, USAUniversity of Wisconsin - Milwaukee, Milwaukee, WI 53201, USAUniversity of Wisconsin - Milwaukee, Milwaukee, WI 53201, USAUniversity of Wisconsin - Milwaukee, Milwaukee, WI 53201, USAUniversity of Wisconsin - Milwaukee, Milwaukee, WI 53201, USAUniversity of Wisconsin - Milwaukee, Milwaukee, WI 53201, USAMedical College of Wisconsin, Milwaukee, WI 53226, USAMedical College of Wisconsin, Milwaukee, WI 53226, USAUniversity of Wisconsin - Milwaukee, Milwaukee, WI 53201, USANeurofibromatosis-1 is the most common single gene disorder affecting 1 in 3000. In children, it is associated not only with physical features but also with attention and learning problems. Research has identified a downward shift in intellectual functioning as well, but to date, there are no published studies about the everyday adaptive behavior of children with NF1. In this study, parental reports of adaptive behavior of 61 children with NF1 ages 3 through 8 were compared to an unaffected contrast group (n=55) that comprised siblings and community members. Significant group differences in adaptive skills were evident and were largely related to group differences in intellectual functioning. In a subsample of children with average-range intellectual functioning, group differences in parent-reported motor skills were apparent even after controlling statistically for group differences in intellectual functioning. The implications of the findings for the care of children with NF1 are discussed.http://dx.doi.org/10.1155/2013/690432
spellingShingle Bonita P. Klein-Tasman
Alina M. Colon
Natalie Brei
Faye van der Fluit
Christina L. Casnar
Kelly M. Janke
Donald Basel
Dawn H. Siegel
Jasmine A. Walker
Adaptive Behavior in Young Children with Neurofibromatosis Type 1
International Journal of Pediatrics
title Adaptive Behavior in Young Children with Neurofibromatosis Type 1
title_full Adaptive Behavior in Young Children with Neurofibromatosis Type 1
title_fullStr Adaptive Behavior in Young Children with Neurofibromatosis Type 1
title_full_unstemmed Adaptive Behavior in Young Children with Neurofibromatosis Type 1
title_short Adaptive Behavior in Young Children with Neurofibromatosis Type 1
title_sort adaptive behavior in young children with neurofibromatosis type 1
url http://dx.doi.org/10.1155/2013/690432
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