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         Wagr Syndrome:     more detail
  1. Aniridia and WAGR Syndrome: A Guide for Patients and Their Families

41. Clinical Dysmorphology - Abstract: Volume 11(1) January 2002 P 73-74 Morbid Obes
Morbid obesity and hyperphagia in the wagr syndrome. A 33year-old man withwagr syndrome is described with morbid obesity associated with hyperphagia
http://www.clindysmorphol.com/pt/re/mcd/abstract.00019605-200201000-00016.htm
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Clinical Dysmorphology. 11(1):73-74, January 2002.
Amor, David J. a Abstract:
A 33-year-old man with WAGR syndrome is described with morbid obesity associated with hyperphagia and an apparent lack of satiety. It is possible that a gene associated with satiety is present at 11p13 although it is premature to conclude that obesity is a specific feature of WAGR syndrome.
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42. ScienceDaily -- Browse Topics: Health/Conditions_and_Diseases/Neurological_Disor
Groups wagr syndrome A brief description of this support group. wagr syndrome - Information for families or physicians interested in learning more
http://www.sciencedaily.com/directory/Health/Conditions_and_Diseases/Neurologica
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43. WAGR Syndrome
wagr syndrome. Wilms tumor aniridia - genitourinary anomalies - mental retardationsyndrome. wagr syndrome wagr syndrome wagr syndrome
http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=460

44. Aniridia Network International - Aniridia Directory
wagr syndrome. Also see Wilms tumour. emedicine.com wagr syndrome wagr syndrome.National Library of Medicine - WAGR -USA
http://www.aniridia.org/directory/WAGR.html
Supporting people with aniridia and their Families Just Diagnosed? click here Homepage About Us About Aniridia ... Make a Donation Web www.aniridia.org
WAGR syndrome
Also see Wilms tumour emedicine.com - WAGR syndrome WAGR Syndrome National Library of Medicine - WAGR -USA Human Genome Mapping Project - WAGR - UK National Library of Medicine - Chromesome 11pdel. syndrome -USA Atlas of Genetics and Cytogenetics in Oncology and Haematology WAGR WAGR in finnish Have a website you would like to add? Submit your URL to Hannah@aniridia-network.net

45. WAGR References Submicroscopic Deletions At The WAGR Locus
PMID 2570677, UI 89377247 Aniridia as part of a wagr syndrome in a girl whosebrother presented wagr syndrome www.bashour.com/examprep/documents/
http://www.aniridia.org/conditions/WAGR.txt
WAGR References Submicroscopic deletions at the WAGR locus, revealed by nonradioactive in situ hybridization. Fantes JA, Bickmore WA, Fletcher JM, Ballesta F, Hanson IM, van Heyningen V MRC Human Genetics Unit, Western General Hospital, Edinburgh, United Kingdom. PMID: 1334370, UI: 93098243 Cytogenet Cell Genet 1989;50(2-3):70-4 Molecular definition of de novo and genetically transmitted WAGR- associated rearrangements of 11p13. Lavedan C, Barichard F, Azoulay M, Couillin P, Molina Gomez D, Nicolas H, Quack B, Rethore MO, Noel B, Junien C Institut National de la Sante et de la Recherche Medicale, Unite 73, Paris, France. PMID: 2570677, UI: 89377247 Aniridia as part of a WAGR syndrome in a girl whose brother presented hypospadias. Lorda-Sanchez I, Sanz R, Diaz-Guillen MA, Fernandez-Toral J, Heine-Suner D, Rodriguez De Alba M, Gonzalez-Gonzalez C, Trujillo MJ, Ramos C, Rodriguez De Cordoba S, Ayuso C. Departmento de Genetica, Fundacion Jimenez-Diaz, Madrid, Spain. ilorda@fjd.es R. Muto1,3, S. Yamamori2, M. Osawa1, H. Ohashi3. 1) Department of Pediatrics, Tokyo Womens Med. Univ., Shinjuku-ku, Tokyo Japan; 2) Research department Mitsubishi Kagaku Bio-Clinical Laboratories, Inc.Tokyo Japan; 3) Division of Medical Genetics, Saitama Children's Medical Center, Saitama Japan. WAGR Syndrome www.bashour.com/examprep/documents/ GLAUCOMA%20Oral%20Questions.pdf The predictive value of FISH analyses for possible occurrence of Wilms tumor in aniridia patients with/without chromosome abnormalities around 11p13. R. Muto1,3, S. Yamamori2, M. Osawa1, H. Ohashi3. 1) Department of Pediatrics, Tokyo Womens Med. Univ., Shinjuku-ku, Tokyo Japan; 2) Research department Mitsubishi Kagaku Bio-Clinical Laboratories, Inc.Tokyo Japan; 3) Division of Medical Genetics, Saitama Children's Medical Center, Saitama Japan.

46. HighWire -- Browse Journals - WAGR Syndrome
Browse Journals publishing on wagr syndrome, (return to Topic List page) Alphabet, , Frequency of articles in wagr syndrome, , Focus of journal on
http://highwire.stanford.edu/lists/topic_dir/608683/618131/622317/622443/622456/
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Sort by: Alphabet Frequency of articles in WAGR Syndrome Focus of journal on WAGR Syndrome What's this? Journals focusing on WAGR Syndrome (in order by highest focus) Pediatric Research info free ISSUES Cancer Research ... Urogenital Abnormalities WAGR Syndrome Home Adv. Search For Institutions For Publishers ... partners/suppliers

47. HighWire -- Browse Journals - WAGR Syndrome
Orthoguide.com wagr syndromeSearch AltaVista for wagr syndrome Global Search Add Url - Free Medline -Contact Us - Search. Enter Keywords to Search and Your Choice of Search
http://highwire.stanford.edu/lists/topic_dir/608683/608684/614552/614553/614679/
ANNOUNCEMENT: CHECK OUT THE PREVIEW RELEASE OF OUR NEW WEB SITE! My Favorite Journals ( HW-hosted journals HW-hosted Medline Author:
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Year: Vol: Page: Home Adv. Search For Institutions For Publishers ... Sign in or register for access to all HighWire Press customization features Browse Journals publishing on WAGR Syndrome: (return to Topic List page) WAGR Syndrome
Sort by: Alphabet Frequency of articles in WAGR Syndrome Focus of journal on WAGR Syndrome What's this? Journals focusing on WAGR Syndrome (in order by highest focus) Pediatric Research info free ISSUES Cancer Research ... Urogenital Abnormalities WAGR Syndrome Home Adv. Search For Institutions For Publishers ... partners/suppliers

48. Parental Origin Of WT1 Mutations And Mental Retardation In WAGR Syndrome - Natur
Parental origin of WT1 mutations and mental retardation in wagr syndrome.Vicki Huff1. 1Department of Experimental Pediatrics, University of Texas MD
http://www.nature.com/ng/journal/v8/n1/abs/ng0994-13a.html
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doi:10.1038/ng0994-13a
Parental origin of mutations and mental retardation in WAGR syndrome
Vicki Huff Department of Experimental Pediatrics, University of Texas M.D. Anderson Cancer Center, Houston, Texas 77030, USA REFERENCES
  • Jinno, Y. et al Nature Genet. Article PubMed ISI ChemPort Huff, V. et al Am. J. hum. Genet. PubMed ISI ChemPort Riccardi, V.M. et al Pediatrics PubMed ISI ChemPort Franke, U. et al Cytogenet. cell Genet. PubMed Riccardi, V.M. et al J. Pediatr. PubMed ISI ChemPort Riccardi, V.M. et al Cancer Genet. Cytogenet. Article ISI Compton, D.A. et al Cell Article PubMed ISI ChemPort Huff, V. et al Am. J. hum. Genet. PubMed ISI ChemPort Lavedan, C. et al Cytogenet. cell Genet. PubMed ISI ChemPort J. Pediatr. PubMed ISI ChemPort J. Pediatr.
  • 49. Accessing Article
    wagr syndrome is a rare contiguous gene disorder characterized by Wilms tumor, A 25year-old girl with wagr syndrome was referred to the ophthalmology
    http://www.nature.com/ejhg/journal/v13/n4/full/5201358a.html
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    I am a member of the European Society of Human Genetics Members of the European Society of Human Genetics receive access to European Journal of Human Genetics as part of their membership.

    50. Our Story - Reaching Out Network
    I was told that Nicholas was born with what doctors call wagr syndrome, resultingfrom a deletion of the 11th chromosome. I was not prepared for what I was
    http://members.aol.com/TheMooZoo/
    Nicholas 7 yrs old Our Story Nicholas is our youngest child, he has a sister who is three years his senior. He was delivered by repeat C-Section in September of 1996. My pregnancy was normal so I had no indication that my child was not going to be a perfectly healthy child.
    Shortly after birth his doctors informed us that he had undescended testicles. Before leaving the hospital Nicholas was seen by a urologist. The urologist had told us undescended testicles was a common condition in boys. We were still a little scared and concerned. We had hoped his testes would eventually drop down, as they often do in many cases. We made arrangements for a follow up meeting with him 10 days later.
    We received a call from our pediatricians office about a week later. We were told that a blood test revealed a problem with one of Nicholas' chromosomes. The doctor did not go into much details. He did recommend that we meet with someone in genetics. We were more scared, confused and in need of some answers. It was at the follow up appointment with urology that everything became much clearer, or much more vague, depending how you look at things.
    I took Nicholas to the appointment alone. I was told that Nicholas was born with what doctors call WAGR Syndrome, resulting from a deletion of the 11th chromosome. I was not prepared for what I was about to hear. His doctor spoke about a kidney tumor, a visual impairment, possible retardation and so much more. Nicholas was crying and my heart was breaking inside. I barely had enough strength or composure to drive home from the doctor's office.

    51. Geneticalliance.org
    wagr syndrome Support Groups Reaching Out WAGR/Aniridia Network 2063 ReginaLincoln Park, MI 48146 Phone 313.381.4302
    http://www.geneticalliance.org/ws_display.asp?filter=support_groups_by_disease&t

    52. Birth Disorder Information Directory - W
    Aniridia-Genitourinary Anomalies-Mental Retardation (WAGR) Syndrome/Triad.wagr syndrome chromosome 11p deletion syndrome GENITOURINARY DYSPLASIA
    http://www.bdid.com/defectw.htm

    HOME
    W
    W Syndrome Waaler Aarskog Syndrome (Hydrocephalus with Costovertebral Dysplasia and Sprengel Anomaly) Waardenburg Syndrome

    53. Syndrome WAGR : Sites Et Documents Francophones
    wagr, syndrome . Arborescence(s)du thesaurus MeSH contenant le mot-clé syndrome WAGR wagr syndrome
    http://www.chu-rouen.fr/ssf/pathol/wagrsyndrome.html
    Syndrome WAGR Synonyme(s) CISMeF aniridie-tumeur de Wilms ; wagr, syndrome .
    Synonyme(s) MeSH Syndrome polymalformatif tumeur de Wilms-aniridie-malformations génito-urinaires-retard mental
    Voir aussi gène néphroblastome
    Arborescence(s) syndrome WAGR wagr syndrome
    maladies de l'appareil génital féminin et complications de la grossesse
    maladies de l'oeil maladies du système nerveux maladies et malformations congénitales, héréditaires et néonatales ... tumeurs Position du mot-clé dans l' (les) arborescence(s) : Vous pouvez consulter Ou consulter ci-dessous une sélection des principales ressources :
    patient 13 août 2005 courriel Menu général CISMeF Haut de page © CHU de Rouen . Toute utilisation partielle ou totale de ce document doit mentionner la source.

    54. APSA :: For Parents: Wilms’ Tumor
    wagr syndrome (lack of an iris, genitourinary abnormalities, mental retardation).Hemihypertrophy (enlarged body parts on one side)
    http://www.eapsa.org/parents/wilms.htm
    What is a Pediatric Surgeon? Finding a Pediatric Surgeon Contact Us APSA Foundation ... Home Background and Incidence
    Beckwith-Wiedemann syndrome
    (enlarged abdominal organs, enlarged tongue, low blood sugar) Denys-Drash Syndrome
    WAGR Syndrome
    (lack of an iris, genitourinary abnormalities, mental retardation) Hemihypertrophy (enlarged body parts on one side) Genitourinary abnormalities Positive family history 11p13 deletion Clinical Presentation
    The tumor also rarely may be a site of erythropoietin production which is the hormone responsible for increasing the number of red blood cells in the blood stream. Laboratory tests are usually otherwise normal except in rare instances in which the bleeding and clotting tests are normal. Diagnostic Evaluation
    The next diagnostic test obtained is a computed tomography (CT) scan of the abdomen. (See Figure 1). CT scan shows the tumor within the kidney, often with a rim of normal kidney tissue around the edge and distortion of the urine collecting system of the kidney and displacement of the kidney toward the center of the body. A chest CT is obtained to evaluate for the presence of tumor spread to the lungs. These studies detect the most common sites of tumor spread: lymph nodes, lung, and liver. A CT scan cannot determine whether the tumor can be removed, however, because this can be assessed accurately only at the time of surgery.

    55. ORPHANET - Maladies Rares - Médicaments Orphelins
    Translate this page MALADIE wagr syndrome. Synonyme(s) Aniridie - tumeur de Wilms. CIM Q87.8.Le syndrome de WAGR (Wilms tumor-Aniridia-Genitourinary anomalies -mental
    http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=893

    56. WAGR Syndrome
    Translate this page Base de données sur les maladies rares et les médicaments orphelins.
    http://www.orpha.net/static/FR/wagr_syndrome.html
    Accès à la base de données Orphanet
    WAGR syndrome
    Accès direct aux détails Résumé
    Le syndrome de WAGR (Wilms tumor-Aniridia-Genitourinary anomalies -mental Retardation) associe une prédisposition à la tumeur de Wilms survenant à un âge variable, une aniridie totale ou partielle pouvant être associée à une cataracte ou un glaucome, un retard mental variable et des anomalies génitales variant de l'ambiguïté sexuelle à l'ectopie testiculaire associée à des anomalies urinaires. Ce syndrome est dû à une microdélétion de la région chromosomique 11p13. Cette délétion apparaît de novo dans la majorité des cas mais peut être héritée par transmission d'une translocation parentale. *Auteur : Equipe Editoriale d'Orphanet (septembre 2002)*. Signes de la maladie
    • ANIRIDIE
    • RETARD MENTAL / PSYCHO-MOTEUR
    • ANOMALIE OREILLE(FORME/STRUCTURE)
    • CATARACTE
    • CECITE
    • HYPOSPADIAS/EPISPADIAS/VERGE COUDEE
    • LEVRES PROEMINENTES
    • MICROCEPHALIE
    • NYSTAGMUS
    • PETITE TAILLE / NANISME
    • PTOSIS
    • RETROGNATHISME/MICROGNATHISME
    • TESTICULE ECTOPIE/CRYPTORCHIDIE
    • AMBIGUITE SEXUELLE
    • BUPHTALMIE/GLAUCOME
    • HERNIE INGUINALE
    • OBESITE GENERALISEE
    • ORTEILS CLINODACTYLIE
    • SCOLIOSE
    Mise à jour : 04/09/2005
    Accès à la base de données Orphanet

    57. Brusa-Torricelli Syndrome (www.whonamedit.com)
    Since the early 1980s this disorder has commonly been referred to as wagr syndrome.We thank Kelly Trout of the International wagr syndrome Association for
    http://www.whonamedit.com/synd.cfm/2404.html

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    Brusa-Torricelli syndrome Also known as: Aniridia-Wilms’ tumour syndrome Aniridia-Wilms' tumor association Miller’s syndrome (Robert W. Miller) Synonyms: Aniridia-nephroblastoma syndrome, oculocerebrorenal syndrome. Associated persons: P. Brusa Robert W. Miller C. Torricelli Max Wilms Description: A congenital syndrome in which aniridia (congenital absence of the iris) and nephroblastoma (Wilms' tumour) is associated with mental retardation, craniofacial defects (microcephaly), growth retardation and skeletal anomalies, deformed pinna, genitourinary anomalies, hamartomas, and umbilical and inguinal hernias. Other frequent features include cataract and glycoma, hypospadias, hemihypertrophy, and horseshoe kidney. The syndrome affects both sexes but is more frequent in males. Since the early 1980s this disorder has commonly been referred to as WAGR syndrome. We thank Kelly Trout of the International WAGR Syndrome Association for for information submitted.

    58. Principal's Office: Welcome (University Of Dundee)
    Another similar disease is the wagr syndrome, but rather than point mutation we have We have developed two transgenic models of DDS and wagr syndrome,
    http://www.dundee.ac.uk/principalsoffice/discoverydays2004/fleming.html
    access keys text only Principal's Office Home Principal ... General University Information (inc. maps)
    DISCOVERY DAYS
    Professor Stewart Fleming, Professor of Cellular and Molecular Pathology
    The Wilms' tumour gene wt1 causes kidney failure and childhood cancer
    "When I die and doctors know not why,
    My friends' curiosity
    Shall have me cut up to survey each part"
    John Donne
    WT1 kidney failure and cancer
    Another similar disease is the WAGR syndrome, but rather than point mutation we have found that this is a contiguous gene syndrome, involving loss of extensive chromosomal material on chromosome 11 including the WT1 gene, resulting in a constitutional loss of one copy of WT1. Recently it has been shown that 38% of WAGR patients subsequently develop renal failure. In both, DDS and WAGR disease develops despite possession of one normal copy of WT1, although the rate of progress and precise nature of the disease differs between the two syndromes. We subsequently found that, in both syndromes, the development of a Wilms' tumour requires a second event at WT1, usually loss of the one remaining normal allele (gene). Transgenic models of WT1 associated disease
    To study the disease processes further and to confirm the causal role of wt1 we needed experimental models. We have developed two transgenic models of DDS and WAGR syndrome, in both of which progressive glomerulosclerosis leading to terminal renal failure is seen.

    59. Characteristics And Outcomes Of Children With The Wilms Tumor-Aniridia Syndrome:
    Results Sixtyfour patients (0.75%) had the wagr syndrome. For WAGR and non-WAGRpatients, respectively, the average birth weights (2.94 and 3.45 kg),
    http://www.jco.org/cgi/content/abstract/21/24/4579
    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/Browse Subscriptions ... Customer Service This Article Full Text Full Text (PDF) Alert me when this article is cited ... Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Download to citation manager PubMed PubMed Citation Articles by Breslow, N. E. Articles by Nichols, K. E. Journal of Clinical Oncology , Vol 21, Issue 24 (December), 2003: 4579-4585
    American Society for Clinical Oncology
    Characteristics and Outcomes of Children With the Wilms Tumor-Aniridia Syndrome: A Report From the National Wilms Tumor Study Group
    Norman E. Breslow Robin Norris Patricia A. Norkool Tammy Kang J. Bruce Beckwith Elizabeth J. Perlman Michael L. Ritchey Daniel M. Green

    60. Characteristics And Outcomes Of Children With The Wilms Tumor-Aniridia Syndrome:
    For most such patients, aniridia or wagr syndrome was mentioned repeatedly The statistical significance of the association of wagr syndrome with RFS and
    http://www.jco.org/cgi/content/full/21/24/4579
    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/Browse Subscriptions ... Customer Service This Article Abstract Full Text (PDF) Alert me when this article is cited ... Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Download to citation manager PubMed PubMed Citation Articles by Breslow, N. E. Articles by Nichols, K. E. Journal of Clinical Oncology , Vol 21, Issue 24 (December), 2003: 4579-4585
    American Society for Clinical Oncology
    Characteristics and Outcomes of Children With the Wilms Tumor-Aniridia Syndrome: A Report From the National Wilms Tumor Study Group
    Norman E. Breslow Robin Norris Patricia A. Norkool Tammy Kang J. Bruce Beckwith Elizabeth J. Perlman Michael L. Ritchey Daniel M. Green

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