Beckwith Wiedemann Syndrome

Important
It is possible that the main title of the report Beckwith Wiedemann Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

Beckwith-Syndrome
BWS
EMG Syndrome
Exomphalos-Macroglossia-Gigantism Syndrome
Hypoglycemia with Macroglossia
Macroglossia-Omphalocele-Visceromegaly Syndrome
Omphalocele-Visceromegaly-Macroglossia Syndrome
Visceromegaly-Umbilical Hernia-Macroglossia Syndrome
Wiedmann-Beckwith Syndrome

Disorder Subdivisions

None

General Discussion

Beckwith-Wiedemann syndrome (BWS) is a rare genetic overgrowth disorder. It is characterized by a wide spectrum of symptoms and physical findings that vary in range and severity from case to case. However, in many individuals, associated features include above-average birth and weight and increased growth after birth (postnatally); an usually large tongue (macroglossia); enlargement of certain internal organs (visceromegaly); and protrusion of a portion of the intestines and abdominal organs through a tear in the wall of the stomach or bellybutton (abdominal wall defects). BWS may also be associated with low blood sugar levels within the first few days or the first month of life (neonatal hypoglycemia); advanced bone age, particularly up to age four; distinctive grooves in the ear lobes and other facial abnormalities; abnormal enlargement of one side or structure of the body (hemihyperplasia) may occur, resulting in unequal (asymmetric) growth; and an increased risk of developing certain childhood cancers.

In approximately 85 percent of cases, BWS results from genetic changes that appear to occur randomly (sporadically). Approximately 10-15 percent of cases of this syndrome run in families and show autosomal dominant inheritance. Researchers have determined that BWS results from various abnormalities affecting the proper expression or structure of certain genes within a specific region of chromosome 11.

Resources

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)428-7100
Fax: (914)997-4763
Tel: (888)663-4637
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com

The Arc (a national organization on mental retardation)
1010 Wayne Ave
Suite 650
Silver Spring, MD 20910
Tel: (301)565-3842
Fax: (301)565-3843
Tel: (800)433-5255
TDD: (817)277-0553
Email: info@thearc.org
Internet: http://www.thearc.org/

Candlelighters Childhood Cancer Foundation
P.O. Box 498
Kensington, MD 20895-0498
Tel: (301)962-3520
Fax: (301)962-3521
Tel: (800)366-2223
Email: staff@candlelighters,org
Internet: http://www.candlelighters.org

National Cancer Institute Physician Data Query (PDQ) Cancer Information Service
9000 Rockville Pike
Bethesda, MD 20892
Tel: (800)422-6237
Internet: http://www.cancernet.nci.nih.gov/pdq.html

National Cancer Institute
6116 Executive Blvd, MSC 8322, Room 3036A
Bethesda, MD 20892-8322
USA
Tel: 3014353848
Tel: 8004226237
TDD: 8003328615
Internet: http://www.cancer.gov

Neuroblastoma Children's Cancer Society
P.O. Box 957672
Hoffman Estates, IL 60195
USA
Tel: 8476051245
Fax: 8476050705
Tel: 8005325162
Email: Info@neurblastomacancer.org
Internet: http://www.neuroblastomacancer.org

Childhood Cancer Foundation - Candlelighters Canada
55 Eglinton Avenue East
Suite 401
Toronto, Ontario, Intl M4P 1G8
Canada
Tel: 4164896440
Fax: 4164899812
Tel: 8003631062
Email: staff@candlelighters.ca
Internet: http://www.childhoodcancer.ca

OncoLink: The University of Pennsylvania Cancer Center Resource
3400 Spruce Street
2 Donner
Philadelphia, PA 19104-4283
USA
Tel: 2153495445
Fax: 2153495445
Email: editors@oncolink.upenn.edu
Internet: http://www.oncolink.upenn.edu

National Childhood Cancer Foundation
PO Box 60012
Suite 402
Arcadia, CA 91066-6012
USA
Tel: 6264471674
Fax: 6264476359
Tel: 8004586223
Email: nccf-info@nccf.org
Internet: http://www.nccf.org/nccf/

NIH/National Institute of Child Health and Human Development
31 Center Dr
Building 31, Room 2A32
MSC2425
Bethesda, MD 20892
Tel: (301)496-5133
Fax: (301)496-7101
Internet: http://www.nih.gov/hichd/

Beckwith-Wiedemann Family Forum
105 Yehudah St. Apt 2
Modi'in, Intl 71700
Israel
Tel: 011 972-8-971-4544
Email: julie@netor.co.il
Internet: http://www.beckwith-wiedemann.info/

Beckwith-Wiedemann Children's Foundation
9031 Cascadia Ave
Everett, WA 98208
Tel: (425)338-4610
Fax: (425)357-8575
Email: thefoundation@beckwith-wiedemannsyndrome.org
Internet: http://www.beckwith-wiedemannsyndrome.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org
Last Updated: 8/7/2007
Copyright 1985, 1988, 1989, 1990, 1993, 1994, 1997, 1999, 2000, 2002, 2007 National Organization for Rare Disorders, Inc.

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