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
Extractions: Disease Injury Nutrition Poison ... Prevention 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).
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
Extractions: Disease Injury Nutrition Poison ... Prevention 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.
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
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
Extractions: 2 January 2003 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.
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
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
Extractions: 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
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
Extractions: 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
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
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
Extractions: 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
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 beckwithwiedemann syndrome. BASICS DESCRIPTION A syndrome of multipledefects characterized primarily by umbilical hernia, macroglossia http://www.5mcc.com/Assets/SUMMARY/0114.html
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
Extractions: (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 Correspondence: retlaj@yahoo.com 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.
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
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
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
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
Extractions: 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
