International WAGR Syndrome Association

11p Deletion Syndrome

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It is important to remember that a given individual with WAGR syndrome may or may not have or develop the condition listed below.  



Cognitive Impairment
or Developmental Delay 

There is great variability in the intellectual abilities of people with WAGR Syndrome.  Most have an IQ that falls within the mild to moderate range of mental retardation.   A small number of people with WAGR Syndrome are profoundly impaired, but there are some who have an “average” or normal IQ.   It is impossible to predict a child’s intellectual capacity in the first years of life, so early and intensive educational intervention is recommended for all children with WAGR syndrome. 


Some individuals with WAGR syndrome have developmental disability, disorders of behavior and/or psychiatric conditions.

These conditions may include:

Anxiety Disorder
Attention Deficit Disorder
(with or without hyperactivity)
Autism
Autism Spectrum Disorder
Developmental Disability -
also called Learning Disability [UK] Intellectual Disability [Australia, Europe, Canada] and Cognitive Impairment)
Pervasive Developmental Disorder
Pervasive Developmental Disorder (NOS) - not otherwise specified
Auditory Processing Disorder
(also called Central Auditory Processing Disorder)
Depression
Obsessive-Compulsive Disorder
Sensory Integration/Sensory Processing Disorder

These conditions may be mild, moderate or severe, and some individuals with WAGR syndrome may have a combination of two or more of these conditions. 
For more information on diagnosis and treatment of these conditions:

Anxiety Disorder:

Anxiety disorders in children and adolescents:
http://www.nami.org/Content/ContentGroups/Helpline1/Anxiety_Disorders_in_Children_and_Adolescents.htm

The Children's Center for OCD and Anxiety
WorryWiseKids.org 
online newsletter & resources 
email:
contacts@worrywisekids.org  
web:
http://www.worrywisekids.org/ 

Attention Deficit Disorder:

Children with Attention Deficit Disorder: http://www.chadd.org/AM/Template.cfm?Section=Understanding

Autism/Autism Spectrum Disorder/Pervasive Developmental Disorder:

The National Institute of Neurologic Disorders and Stroke:
http://www.ninds.nih.gov/disorders/autism/detail_autism.htm

Autism Support Network
Box 1525
Fairfield, CT 06824 (USA)
Tel: (203) 404-4929
Fax: (203) 404-4969
info@AutismSupportNetwork.com
http://www.autismsupportnetwork.com

Auditory Processing Disorder:

The National Institute on Deafness and Other Communication Disorders:
http://www.nidcd.nih.gov/health/voice/auditory.asp

Depression:

The National Institute of Mental Health:
http://www.nimh.nih.gov/health/publications/depression/complete-index.shtml

Obsessive Compulsive Disorder:

The National Institute of Mental Health:
 http://www.nimh.nih.gov/health/topics/obsessive-compulsive-disorder-ocd/index.shtml

Obsessive-Compulsive Foundation
PO Box 961029
Boston, MA 02196
Tel: (617) 973-5801
http://www.ocfoundation.org/ocd-in-children.html

Sensory Integration/Sensory Processing Disorder:

WebMD:
http://www.webmd.com/mental-health/features/help-for-sensory-integration-disorder-in-kids

Sensory Processing Disorder Foundation
5655 S. Yosemite St., Suite 305     
Greenwood Village, CO 80111  
(303) 794-1182
http://www.spdfoundation.net


References:

WAGR Syndrome:  A Clinical Review of 54 Cases.  Fischbach BV, Trout KL, Lewis J, Luis CA, Sika M. 
Pediatrics 2005; 116: 984-988.

WAGR syndrome. Clericuzio C. Management of Genetic Syndromes, Cassidy SB, Allanson JE, Eds.
John Wiley & Sons; 2005: 645-653.

Auditory interhemispheric transfer deficits, hearing difficulties, and brain magnetic resonance imaging abnormalities in children with congenital aniridia due to PAX6 mutations. 
Bamiou DE, Free SL, Sisodiya SM, Chong WK, Musiek F, Williamson KA, van Heyningen V, Moore AT, Gadian D, Luxon LM. Arch Pediatr Adolesc Med. 2007 May;161(5):463-9.

Pax6 3' deletion results in aniridia, autism and mental retardation.  Davis LK, Meyer KJ, Rudd DS, Librant AL, Epping EA, Sheffield VC, Wassink TH. Hum Genet. 2008 May;123(4):371-8.


Last Reviewed: April, 2009

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