Developmental Trajectories in Syndromes With Intellectual Disability, With a Focus on Wolf-Hirschhorn and Its Cognitive-Behavioral Profile

被引:25
作者
Fisch, Gene S. [1 ,2 ,3 ]
Carpenter, Nancy [4 ]
Howard-Peebles, Patricia N. [5 ]
Holden, Jeanette J. A. [6 ]
Tarleton, Jack [7 ]
Simensen, Richard [8 ]
Battaglia, Agatino [9 ]
机构
[1] NYU, Coll Dent, New York, NY 10003 USA
[2] NYU, Coll Nursing, New York, NY 10003 USA
[3] Yeshiva Univ, New York, NY 10033 USA
[4] Ctr Genet Testing, Tulsa, OK USA
[5] Genet & IVF Inst, Fairfax, VA 22039 USA
[6] Queens Univ, Ongwanada Resource Ctr, Kingston, ON, Canada
[7] Fullerton Genet Ctr, Asheville, NC USA
[8] Greenwood Genet Ctr, Greenwood, SC 29646 USA
[9] Stella Maris Clin Res Inst Child & Adolescent Neu, Pisa, Italy
来源
AJIDD-AMERICAN JOURNAL ON INTELLECTUAL AND DEVELOPMENTAL DISABILITIES | 2012年 / 117卷 / 02期
关键词
intellectual disability (ID); fragile X syndrome; FMR1; mutation; Williams-Beuren syndrome; Wolf-Hirschhorn syndrome; IQ; adaptive behavior; developmental quotient; GENOTYPE-PHENOTYPE CORRELATION; FRAGILE-X-SYNDROME; LONGITUDINAL CHANGES; ADAPTIVE-BEHAVIOR; WILLIAMS-SYNDROME; NATURAL-HISTORY; CRITICAL REGIONS; YOUNG BOYS; CHILDREN; FEATURES;
D O I
10.1352/1944-7558-117.2.167
中图分类号
G76 [特殊教育];
学科分类号
040109 ;
摘要
Few studies exist of developmental trajectories in children with intellectual disability, and none for those with subtelomeric deletions. We compared developmental trajectories of children with Wolf-Hirschhorn syndrome to other genetic disorders. We recruited 106 children diagnosed with fragile X, Williams-Beuren syndrome, or Wolf-Hirschhorn syndrome, assessing their intellectual and adaptive behavior abilities. We retested 61 children 2 years later. We compared Time 1 and Time 2 difference scores related to genetic disorder, age, initial IQ, or adaptive behavior composite. Results show genetic disorder and initial IQ score were significant factors for IQ differences, but only genetic disorder affected adaptive behavior differences. Results suggest different gene-brain-behavior pathways likely exist for these genetic disorders. Different developmental trajectories will influence the type and intensity of intervention implemented by caregivers.
引用
收藏
页码:167 / 179
页数:13
相关论文
共 43 条
[1]   Longitudinal Course of Cognitive, Adaptive, and Behavioral Characteristics in Costello Syndrome [J].
Axelrad, Marni E. ;
Schwartz, David D. ;
Fehlis, Julie E. ;
Hopkins, Elizabeth ;
Stabley, Deborah L. ;
Sol-Church, Katia ;
Gripp, Karen W. .
AMERICAN JOURNAL OF MEDICAL GENETICS PART A, 2009, 149A (12) :2666-2672
[2]  
Bailey DB, 1998, AM J MENT RETARD, V103, P29, DOI 10.1352/0895-8017(1998)103<0029:EDTOMW>2.0.CO
[3]  
2
[4]   Natural history of Wolf-Hirschhorn syndrome: Experience with 15 cases [J].
Battaglia, A ;
Carey, JC ;
Cederholm, P ;
Viskochil, DH ;
Brothman, AR ;
Galasso, C .
PEDIATRICS, 1999, 103 (04) :830-836
[5]   Update on the Clinical Features and Natural History of Wolf-Hirschhorn (4p-) Syndrome: Experience With 87 Patients and Recommendations for Routine Health Supervision [J].
Battaglia, Agatino ;
Filippi, Tiziana ;
Carey, John C. .
AMERICAN JOURNAL OF MEDICAL GENETICS PART C-SEMINARS IN MEDICAL GENETICS, 2008, 148C (04) :246-251
[6]  
BORGHGRAEF M, 1987, CLIN GENET, V32, P179
[7]   LONGITUDINAL RESEARCH IN DOWN-SYNDROME [J].
CARR, J .
INTERNATIONAL REVIEW OF RESEARCH IN MENTAL RETARDATION, 1992, 18 :197-223
[8]   PROFILES, CORRELATES, AND TRAJECTORIES OF INTELLIGENCE IN PRADER-WILLI SYNDROME [J].
DYKENS, EM ;
HODAPP, RM ;
WALSH, K ;
NASH, LJ .
JOURNAL OF THE AMERICAN ACADEMY OF CHILD AND ADOLESCENT PSYCHIATRY, 1992, 31 (06) :1125-1130
[9]   TRAJECTORY OF ADAPTIVE-BEHAVIOR IN MALES WITH FRAGILE X-SYNDROME [J].
DYKENS, EM ;
HODAPP, RM ;
ORT, SI ;
LECKMAN, JF .
JOURNAL OF AUTISM AND DEVELOPMENTAL DISORDERS, 1993, 23 (01) :135-145
[10]   Cognitive-Behavioral Features of Children With Wolf-Hirschhorn Syndrome: Preliminary Report of 12 Cases [J].
Fisch, Gene S. ;
Battaglia, Agatino ;
Parrini, Barbara ;
Youngblom, Janey ;
Simensen, Richard .
AMERICAN JOURNAL OF MEDICAL GENETICS PART C-SEMINARS IN MEDICAL GENETICS, 2008, 148C (04) :252-256