Cancer Syndromes wagr syndrome ANIRIDIA AND ABSENT PATELLA deletion of 11p13 resultsin the wagr syndrome Aniridia; AN1 by OMIM Shaw et al. http://ibis-birthdefects.org/start/cancersy.htm
Extractions: Rather numerous instances of multiple sibs with Wilms tumor have been described ... The syndrome of aniridia, hemihypertrophy and other congenital anomalies with Wilms tumor, subsequently known as the WAGR syndrome, was first described by Miller et al. (1964) ... . Riccardi et al. (1978) observed a triad of aniridia, ambiguous genitalia and mental retardation (AGR triad) in 3 patients with an interstitial deletion of the short arm of chromosome 11 ... Marshall et al. (1982) studied 14 patients with aniridia .... 6 had interstitial deletion of 11p of various lengths. Band 11p13 was included in the deletion in all 6 cases ...
JCO -- Abstracts: Breslow Et Al. 21 (24): 4579 We compared demographics, disease characteristics, and treatment outcomes ina large cohort of WT patients who did or did not have the wagr syndrome. http://www.jco.org/cgi/content/abstract/21/24/4579
Extractions: Search for: Limit by: All Topics Original Reports Review Articles Special Articles Biology of Neoplasia Editorials Comments and Controversies Diagnosis in Oncology Art of Oncology Correspondence All Years Browse by Topic or Issue Home Search Subscribe ... Email this article to a colleague Similar articles found in: American Society for Clinical Oncology Norman E. Breslow Robin Norris Patricia A. Norkool Tammy Kang J. Bruce Beckwith Elizabeth J. Perlman Michael L. Ritchey Daniel M. Green Kim E. Nichols Address reprint requests to Norman Breslow, PhD, Department of Biostatistics, Box 357232, University of Washington, Seattle, WA 98195-7232; e-mail: norm@u.washington.edu
Www.nlm.nih.gov/cgi/mesh/2K/MB_cgi?term=WAGR+Syndrome More results from www.nlm.nih.gov MeSHD terms associated to MeSH-C term wagr syndromeMeSH-D terms associated to MeSH-C term wagr syndrome, G2D Home. The number indicatesthe strength of the association of the corresponding term to wagr syndrome. http://www.nlm.nih.gov/cgi/mesh/2K/MB_cgi?term=WAGR Syndrome
Pediatric Research -- Abstracts: Kohler Et Al. 45 (2): 187 Mutations in the WT1 gene causing Wilms tumors were first reported in wagr syndrome(Wilms tumor, Aniridia, Genitourinary malformation, mental Retardation) and http://www.pedresearch.org/cgi/content/abstract/45/2/187
Extractions: Department of Pediatric Endocrinology, Humboldt Universitat zu Berlin, Germany. Mutations in the WT1 gene causing Wilms tumors were first reported in WAGR syndrome (Wilms tumor, Aniridia, Genitourinary malformation, mental Retardation) and Denys Drash syndrome (pseudohermaphroditism, Wilms tumor, nephropathy), but only in a few patients with hypospadias and cryptorchidism without other signs of Denys Drash (DDS) or WAGR syndrome WT1 mutations were identified. We report a boy, who was born in 1989 with
WAGR, Syndrome : Arborescences MeSH Translate this page wagr, syndrome arborescences MeSH. Menu général CISMeF. wagr, syndromeC11.941.375.060.950 page PubMed du motclef page CISMeF du motclef http://www.chu-rouen.fr/navimesh/W/naviwagrsyndrome.html
Aniridia Network International - Aniridic Keratopathy wagr. every 3 months until they are 6 years of age.6 Children with Wilms AniridiaGenitourinaryabnormalities-(mental) Retardation (wagr) syndrome are at http://www.aniridia.org/conditions/wagr.html
Extractions: This is a malignant cancer of the kidneys and is the most common renal tumor in both children that is rarely diagnosed after the age of 12. It is also one of the most treatable cancers especially if it is caught at an early stage. It is caused by the deletion of the wilms tumour suppressor gene WT1. This gene appears to play an important role in the normal development and maturation of the kidneys and gonads (See below). According to the National Cancer Institute (USA) "Patients with aniridia or hemihypertrophy should be screened with ultrasound every 3 months until they are 6 years of age.[6] Children with Wilms' Aniridia-Genitourinary abnormalities-(mental) Retardation (WAGR) syndrome are at increased risk of eventually developing renal failure and should be monitored. Patients with Wilms' tumor and aniridia without genitourinary abnormalities are at lesser risk but should be monitored." (natioanl Cancer Institute -
Extractions: WAGR Complex WAGR-oireyhtymä on harvinainen sairaus, jonka nimi tulee englanninkielisistä sanoista W ilms tumor ( Wilmsin kasvain A niridia ( silmän värikalvon puuttuminen ), G enitourinary abnormalities ( epämuodostuneet suku- ja virtsaelimet ) ja Mental R etardation ( kehitysvammaisuus ). Nimi antaa hyvän kuvan sairauden ilmenemistavasta. Oireet voivat tosin jossain määrin vaihdella ja niitä voi olla enemmänkin. WAGR-oireyhtymä johtuu useimmiten sattumanvaraisesta sikiökehityksen aikaisesta mutaatiosta. Joissakin hyvin harvinaisissa tapauksissa on tavattu autosomaalisesti vallitsevaa periytyvyyttä Tämä v. 1964 ensimmäisen kerran kuvattu oireyhtymä on useamman geenin vaurion aiheuttama. Lisätietoja: WAGR Syndrome, OMIM
Extractions: Syndrome chromosome 11p deletion syndrome Synonyms 11p- syndrome 11p deletion syndrome chromosome 11p monosomy del(11p) syndrome deletion 11p syndrome monosomy 11p partial monosomy 11p aniridia type II aniridia-ambiguous genitalia-mental retardation (AGR) syndrome, triad aniridia-genitourinary-abnormalities-mental retardation triad aniridia-Wilms tumor association, syndrome (aniridia-WT association, AWTA) aniridia-Wilms tumor-gonadoblastoma syndrome del11/aniridia complex oculocerebrorenal (OCR) syndrome Brusa-Toricelli syndrome Miller syndrome Wilms tumor-aniridia syndrome Wilms tumor-aniridia-genitourinary abnormalities-mental retardation triad Wilms tumor-aniridia-gonadoblastoma-mental retardation (WAGR) syndrome, association Summary Deletion of the short arm of chromosome 11 with a variable phenotype: Congenital absence of the iris, genitourinary abnormalities and mental retardation known as aniridia-ambiguous genitalia-mental retardation (AGR) triad; Wilms tumor-aniridia-ambiguous genitalia-mental retardation (WAGR) syndrome; Wilms tumor associated with congenital absence of the iris, genitourinary abnormalities, hemihypertrophy, mental retardation, and other anomalies termed AWTA; Major Features Head and neck: Cranial asymmetry, microcephaly, brachycephaly, prominent forehead, long narrow face, large fontanelles, premature synostosis of metopic sutures, and biparietal foramina.
NORD - National Organization For Rare Disorders, Inc. Search NORD s Databases Read about diseases in NORD s Rare DiseaseDatabase. Find an organization in our Organizational Database. http://www.rarediseases.org/search/zcat_search_results?allfields=medulloblastoma
