41. Beckwith-Wiedemann Syndrome beckwithwiedemann syndrome. Definition Causes, incidence, and risk factors Thecause of beckwith-wiedemann syndrome is unknown, but it appears to be genetic. http://www.pennhealth.com/ency/article/001186.htm

Disease Injury Nutrition Poison ... Prevention Beckwith-Wiedemann syndrome Definition: Beckwith-Wiedemann syndrome is a consistent grouping of findings of unknown etiology (cause) and characterized by a large tongue ( macroglossia ), large organs ( visceromegaly ) and large body size ( macrosomia umbilical hernia or omphalocele (hernia of the navel), and low blood sugar in the newborn (neonatal hypoglycemia Causes, incidence, and risk factors: The cause of Beckwith-Wiedemann syndrome is unknown, but it appears to be genetic. Some cases may be associated with a defect in chromosome number 11. Affected children are often large at birth. Many have an abdominal wall defect, such as an umbilical hernia or omphalocele. They have a characteristic facial appearance with a gaping mouth and large tongue. Infancy can be a critical period because of low blood sugar (hypoglycemia), omphalocele (when present), and an increased rate of tumor development ( Wilm's tumor and adrenal carcinoma being most common). Review Date: 2/1/2002 Reviewed By: Adam Ratner, M.D., Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

42. Beckwith-Wiedemann Syndrome beckwithwiedemann syndrome. Treatment A support group for this syndrome can befound at http//www.beckwith-wiedemann.org. Expectations (prognosis) http://www.pennhealth.com/ency/article/001186trt.htm

Disease Injury Nutrition Poison ... Prevention Beckwith-Wiedemann syndrome Treatment: In infancy, hypoglycemia may be treated with intravenous solutions. Enlargement of the tongue can cause problems with feeding and sleeping. Defects of the abdominal wall may need to be repaired. In addition, affected children must be followed carefully for the appearance of tumors. Support Groups: A support group for this syndrome can be found at http://www.beckwith-wiedemann.org Expectations (prognosis): Children who survive infancy do well, although there is no long term follow-up available. Mental function appears to be normal to very slightly decreased. Complications: Hypoglycemia Seizures Respiratory difficulties from obstruction due to large tongue Feeding problems Development of tumors Calling your health care provider: If you have a child with Beckwith-Wiedemann syndrome at home, any worrisome symptoms develop, call your health care provider immediately. Review Date: 2/1/2002 Reviewed By: Adam Ratner, M.D., Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

43. Questions And Answers About Living With Beckwith-Wiedemann Questions and Answers About Living With beckwithwiedemann syndrome.CancerMail from the National Cancer Institute. CANCER FACTS National http://cancerweb.ncl.ac.uk/cancernet/600367.html

44. Beckwith-Wiedemann Syndrome Seattle. beckwithwiedemann syndrome. BWS, EMG use. Table 1. MolecularGenetic Testing Used in beckwith-wiedemann syndrome. Patients with http://www.geneclinics.org/profiles/bws/details.html

Beckwith-Wiedemann Syndrome [BWS, EMG Syndrome, Exomphalos-Macroglossia-Gigantism Syndrome, WBS, Wiedemann-Beckwith Syndrome] Authors: Cheryl Shuman, MS, CGC Rosanna Weksberg, MD, PhD, FRCPC, FCCMG, FACMG The Hospital for Sick Children Initial Posting: 3 March 2000 Last Revision 2 January 2003 Summary Disease characteristics. Beckwith-Wiedemann syndrome (BWS) is a disorder of growth characterized by macrosomia (large body size), macroglossia, visceromegaly, embryonal tumors (i.e., Wilms tumor, hepatoblastoma, neuroblastoma, rhabdomyosarcoma), omphalocele, neonatal hypoglycemia, and ear creases/pits. Diagnosis/testing. The majority of patients with BWS have normal chromosome studies; however, chromosome abnormalities involving 11p15 are found in 1% or less of cases. Paternal uniparental disomy for chromosome 11p15 or mutations in the gene (chromosomal locus 11p15) are found in 15-30% of patients with normal chromosome studies. Detection of these molecular genetic abnormalities can be helpful in clinical diagnosis and genetic counseling. Genetic counseling.

45. Beckwith-Wiedemann Syndrome : Meddie Health Search ITEMS LINKS Adam.com An Overview A definition of beckwithwiedemann syndrome,along with a look at the alternate names, causes, incidence and risk factors. http://www.meddie.com/search/Health/Conditions_and_Diseases/Genetic_Disorders/Be

HOME ADD A LINK MODIFY A LINK NEW LINKS ... TOP RATED Search Meddie: the entire directory only this category More search options Home Health Conditions and Diseases ... Genetic Disorders : Beckwith-Wiedemann Syndrome ITEMS: LINKS: Adam.com: An Overview A definition of beckwith-wiedemann syndrome, along with a look at the alternate names, causes, incidence and risk factors. (Rating: 0.00 Votes: 0) Rate It Beckwith-Wiedemann Family Created as a way for people interested in BWS to get support and find/share information quickly. (Rating: 10.00 Votes: 1) Rate It BWSN: Beckwith-Wiedemann Syndrome Information about this organization, created for parents, professionals, and others interested in this disease. An invitation to join an email support group. (Rating: 0.00 Votes: 0) Rate It Keep Kids Healthy A discussion of beckwith-wiedemann syndrome, its features and treatments. (Rating: 0.00 Votes: 0) Rate It National Library of Medicine weckwith-wiedemann syndrome, a list of synonyms, a summary and a major features. (Rating: 0.00 Votes: 0) Rate It The CaF Directory A description of beckwith-wiedemann syndrome, features, and possible complications. Also information about a support group.

46. Image: Macroglossia In Beckwith-Wiedemann Syndrome Click here to return to the Pediatric Surgery at Brown Home Page.Key words macroglossia, tongue, macrosomia, hernia of umbilical http://bms.brown.edu/pedisurg/Brown/IBImages/AbdWallDefects/Macroglossia.html

Click here to return to the Pediatric Surgery at Brown Home Page Key words: macroglossia, tongue, macrosomia, hernia of umbilical cord, omphalocele, exomphalos, umbilical cord, Beckwith-Wiedeman, Wiedemann, Wilms, hepatoblastoma, insulin, hypoglycemia, hemi-hypertrophy, aniridia, peel, gastroschisis, abdominal wall defect, laparoschisis, scaphoid abdomen, amniotic fluid, short bowel, short gut, atresia, umbilical cord, fetus, ultrasound, newborn, prenatal diagnosis, amnioinfusion

47. Beckwith-Wiedemann Syndrome. Three Case Report... 172. beckwithwiedemann syndrome. Three case report. ABSTRACT. Beckwith-Wiedemannsyndrome was first described in 1963. It http://www.imbiomed.com.mx/ADM/Odv58n5/english/Zod015-03.html

Article in Spanish Toranzo FJM, Duarte HS, Rodríguez PA Síndrome de Beckwith Wiedemann: Reporte de tres casos Rev ADM Beckwith Wiedemann syndrome. Three case report ABSTRACT Beckwith Wiedemann syndrome was first described in 1963. It is a rare congenital disease asso ciated with macrosomy, onfalocele, craniofacial dismorfism, macroglosia, visceromegaly, hemihypertrophy and Wilms tumors. Head and neck signsare of variable expression including dental and cranio facial anomalies. Three cases are reviewed and treated with partial glossectomy. Key words: Beckwith Wiedemann, syndrome, macroglosia Main index Return to index H O M E Solicitude of articles ... Search

48. Health Library - Beckwith Wiedemann Syndrome Beckwith Wiedemann Syndrome. Synonyms Disorder Subdivisions General DiscussionResources beckwithwiedemann syndrome (BWS) is a rare genetic disorder. http://www.laurushealth.com/library/healthguide/illnessconditions/topic.asp?hwid

49. Beckwith Wiedemann Syndrome (Exomphalos-Macroglossia-Gigantism Syndrome) HOME Beckwith Wiedemann Syndrome (ExomphalosMacroglossia-Gigantism EMG Syndrome). BECKWITH-WIEDEMANNSYNDROME; BWS; beckwith-wiedemann syndrome; Support Group. http://www.bdid.com/beckwith.htm

HOME Beckwith Wiedemann Syndrome (Exomphalos-Macroglossia-Gigantism [EMG] Syndrome) Beckwith-Wiedemann syndrome Beckwith-Wiedemann syndrome Beckwith BECKWITH ... HOME

50. Beckwith-Wiedemann Syndrome - Overview beckwithwiedemann syndrome. Alternative names Causes, incidence, andrisk factors The cause of beckwith-wiedemann syndrome is unknown. http://www.wfubmc.edu/besthealth/ency/article/001186.htm

Disease Injury Nutrition Poison ... Prevention Beckwith-Wiedemann syndrome Alternative names: Beckwith syndrome; Wiedemann-Beckwith Definition: Beckwith-Wiedemann syndrome is a consistent grouping of findings of unknown etiology (cause) and characterized by a large tongue ( macroglossia ), large organs ( visceromegaly ) and large body size ( macrosomia umbilical hernia or omphalocele hernia of the navel) and low blood sugar in the newborn (neonatal hypoglycemia Causes, incidence, and risk factors: The cause of Beckwith-Wiedemann syndrome is unknown. Autosomal dominant transmission has been tentatively reported in a few cases (possibly with variable expressivity and penetrance). Evidence shows increased occurrences within a family, but a definitive genetic inheritance has not been fully extablished. Infants with this condition that also exhibit low blood sugar hypoglycemia ) may have a defect in chromosome Affected children are large and at birth may have weighed more than 8 pounds. Following birth accelerated growth rapidly takes them to greater than the 90th percentile for height and weight. Most of these children have an abdominal wall defect such as an umbilical hernia or omphalocele. They have a characteristic facial appearance with a gaping mouth and large tongue and mild microcephaly. They may also have creases in their earlobes. Infancy is a critical period because of bouts of severe hypoglycemia

51. Beckwith-Wiedemann Syndrome - General Practice Notebook beckwithwiedemann syndrome. A baby with beckwith-wiedemann syndromeis born large, macrosomic, and grows into a gigantic infant. http://www.gpnotebook.co.uk/cache/705036312.htm

Beckwith-Wiedemann syndrome A baby with Beckwith-Wiedemann syndrome is born large, macrosomic, and grows into a gigantic infant. Another name for this association is the EMG syndrome because of the triad of classical features: exomphalos macroglossia gigantism Click here for more information...

52. Beckwith-Wiedemann Syndrome beckwithwiedemann syndrome. BASICS DESCRIPTION A syndrome of multipledefects characterized primarily by umbilical hernia, macroglossia http://www.5mcc.com/Assets/SUMMARY/0114.html

53. Resourses BECKER DISEASE (MYOTONIA CONGENITA) . BeckerDisease beckwithwiedemann syndrome .. http://sargon.mmu.ac.uk/RESORCE2.HTM

Health and Disability-Related Web Sites Page 2 of 26 Updated 11/08/97 BACTERIAL DISEASES...................... Bacterial diseases: food and water borne BATTEN DISEASE........................... Batten Disease Listserv Causes of Batten Disease Diagnosis and treatment of Batten Disease Research on Batten Disease and other Neurogenetic Disorders ... What is Batten disease BECKER DISEASE (MYOTONIA CONGENITA)............................ Becker Disease BECKWITH-WIEDEMANN SYNDROME............................ Beckwith-Wiedemann Syndrome Beckwith-Wiedemann Syndrome, from Medline Beckwith-Wiedemann Syndrome, from Grand Rounds Archives Beckwith-Wiedemann Syndrome, neonatal onse ... Macroencephaly with Beckwith Syndrome BEDWETTING.................... What Parents Need to Know About Bedwetting bedwetting bedwetting (a report) Bedwetting is not a disease, but a symptom ... Most children can be successfully treated for bedwetting BELL'S PALSY................................ Bell's Palsy Bell's Palsy Bell's Palsy, series of articles Bell's Palsy, a case study ... Facial paralysis BERNARD-SOULIER SYNDROME....................... Bernard-Soulier Syndrome, a blood platete disorder

54. PAOR 6. Kötet 4. Szám 295. Oldal REPORT. Acid Sphingomyelinase Deficiency in Beckwith –Wiedemann Syndrome. References.1. M Eliot , E R Maher beckwithwiedemann syndrome. http://www.webio.hu/por/2000/6/4/0295/index.php

(c) 2003. WB Saunders Co. Dátum: 2003.03.31. Vol 6, No 4, 2000 Publication is available online at http://www.WEBio.Hu/por/2000/6/4/0295 on WEBio Bioscience Portal REPORT Acid Sphingomyelinase Deficiency in Beckwith –Wiedemann Syndrome 2nd Department of Paediatrics, Semmelweis University, Budapest, Hungary Bethesda Children’s Hospital of the Hungarian Reformed Church, Budapest, Hungary Key words: Beckwith-Wiedemann syndrome; acidic sphingomyelinase deficiency; embryonal rhabdomyosarcoma; simultaneous occurrence Received: OCTOBER 12; 2000; accepted: DECEMBER 13, 2000 Correspondence: retlaj@yahoo.com References M Eliot , E R Maher : Beckwith-Wiedemann syndrome. J Med Genet 31: 560 -564 , 1994. C Sotelo-Avila , F Gonzalez-Crussi , J W Fowler : Complete and incomplete forms of Beckwith-Wiedemann syndrome: Their oncogenic potential. J Pediatr 96: 47 -50 , 1980. H R Wiedemann : Tumors and hemihypertrophy associated with Wiedemann-Beckwith syndrome. Eur J Pediatr 414 -429 , 1983. W Reik : Imprinting in clusters: lessons from Beckwith-Wiedemann syndrome Trends Genet 13: 330 -334 , 1997.

55. Page Not Found beckwithwiedemann syndrome. Syndrome, beckwith-wiedemann syndrome.Gene Name, Cyclin-dependent kinase inhibitor 1C. Gene Symbol, CDKN1C. http://www.nidr.nih.gov/cranio/detail/130650.htm

Please check to see if you entered the correct URL. If you did, it might be that the link no longer exists. As an option, you may do a search of our site. If all else fails, please fill in the form below and send it to the webmaster to report the broken link. Your Name: Email Address: Please indicate the broken URL here: Your browser doesn't support JavaScript. It will, however, affect only two minor things: it will distort the page appearance and will disable the "printer-friendly version" link. NIDCR Home Page Contact us Accessibility Privacy statement ... Search Image in banner: magnified fluoride crystal National Institute of Dental and Craniofacial Research National Institutes of Health Bethesda, MD 20892-2190 e-mail: nidcrinfo@mail.nih.gov phone: 301/496-4261 National Institutes of Health Department of Health and Human Services

56. Health Information Resource Database: Beckwith-Wiedemann Support Network Abstract. beckwithwiedemann syndrome (BWS) is a congenital growth related disorder.There are roughly thirty characteristics which can be associated with BWS. http://www.health.gov/NHIC/NHICScripts/Entry.cfm?HRCode=HR2420

57. Service Page - Pathologie Information DISEASE beckwithwiedemann syndrome, Synonym(s) Exomphalos-macroglossia-gigantismsyndrome Wiedemann-Beckwith syndrome, CIM Q87.3, http://orphanet.infobiogen.fr/Site/Exp.stm?Lng=GB&Expert=116

58. Beckwith-Wiedemann Syndrome This presentation contains content that your browser may not be ableto show properly. This presentation was optimized for more http://icarus.med.utoronto.ca/Pathobiology ppt/DistLectSadowski.htm

This presentation contains content that your browser may not be able to show properly. This presentation was optimized for more recent versions of Microsoft Internet Explorer. If you would like to proceed anyway, click here

59. Beckwith-Wiedemann Syndrome http://icarus.med.utoronto.ca/Pathobiology ppt/DistLectSadowski_files/frame.htm

60. Press_release_Jan_15_2003 The condition, called beckwithwiedemann syndrome, predisposes children to birthdefects and cancer, and is linked with a genetic phenomenon known as imprinting http://www.babraham.ac.uk/public/press_releases/Jan_15_2003.htm

Media Schools News Publications ... Useful links Media Information Strict embargo: 23:01hrs Wednesday, January 15, 2003 HEALTH RISK FROM IVF IS A CAUTION ON CLONING SCIENTISTS funded by Cancer Research UK are today (Wednesday) issuing a warning on the potential dangers of cloning following their discovery that a rare childhood illness is four times as common in babies born after IVF treatments. The condition, called Beckwith-Wiedemann syndrome, predisposes children to birth defects and cancer, and is linked with a genetic phenomenon known as imprinting. This means that certain genes act differently depending on whether they are inherited from the mother or the father. The researchers believe that IVF increases the risk of the syndrome because it interferes with imprinting. Cloning, which is a more radical technique than IVF, is known to destroy normal imprinting and could dramatically increase the risk of conditions like Beckwith-Wiedemann syndrome. *Journal of Medical Genetics, Vol. 40, No. 1

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