Beckwith Wiedemann Syndrome

Important
It is possible that the main title of the report Beckwith Wiedemann Syndromeis not the name you expected.

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
800: (888)663-4637
Askus@marchofdimes.com
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
800: (800)433-5255
TDD: (817)277-0553
info@thearc.org
http://www.thearc.org/

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

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

National Cancer Institute
6116 Executive Blvd, MSC 8322, Room 3036A
Bethesda
MD
20892-8322
USA
Tel: (301)435-3848
800: (800)422-6237
TDD: (800)332-8615
http://www.cancer.gov

Neuroblastoma Children's Cancer Society
P.O. Box 957672
Hoffman Estates
IL
60195
USA
Tel: (847)605-1245
Fax: (847)605-0705
800: (800)532-5162
Info@neurblastomacancer.org
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
800: 8003631062
staff@candlelighters.ca
http://www.childhoodcancer.ca

OncoLink: The University of Pennsylvania Cancer Center Resource
3400 Spruce Street
2 Donner
Philadelphia
PA
19104-4283
USA
Tel: (215)349-5445
Fax: (215)349-5445
editors@oncolink.upenn.edu
http://www.oncolink.upenn.edu

National Childhood Cancer Foundation
PO Box 60012
Suite 402
Arcadia
CA
91066-6012
USA
Tel: (626)447-1674
Fax: (626)447-6359
800: (800)458-6223
nccf-info@nccf.org
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
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
julie@netor.co.il
http://www.beckwith-wiedemann.info/

MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
150 Custer Court
Green Bay
WI
54301-1243
USA
Tel: (920)336-5333
Fax: (920)339-0995
800: (877)336-5333
mums@netnet.net
http://www.netnet.net/mums/

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

HemiHypertrophy Support
4581 Magnolia Dr.
Suffolk
VA
23435
Tel: (757)271-9000
administrator@hemisupport.com
http://www.hemisupport.com

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.

WebMD Medical Reference from the National Organization of Rare Disorders

Last Updated: August 07, 2007

This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.