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

81. Www.ohsu.edu/cliniweb/C11/C11.250.60.html
12.42.224.225/library/healthguide/enus/illnesscon 100800 ACHONDROPLASIA; ACH 101200 APERT SYNDROME 101400 COMPONENT OF wagr syndrome; GUD 137440 GERSTMANN-STRAUSSLER DISEASE; 194050 WILLIAMS-BEUREN SYNDROME; WBS 194072 wagr syndrome 194080
http://www.ohsu.edu/cliniweb/C11/C11.250.60.html

82. MUMS List Of Disorders - U - Z
WSyndrome (1); wagr syndrome (Wilms Tumor-Aniridia-Gonadoblastoma M.lR.) (8) *;Waardenburg Syndrome (6); Waardenburg Syndrome Hirschsprung Disease (1)
http://www.netnet.net/mums/mum_u-z.htm
Return to MUMS Home Page
MUMS:
List of Disorders
U - Z
Number in parentheses indicates number of matches.
indicates there is a support group which covers that diagnosis.
  • UPJ Obstruction (4)
  • Uhl Anomaly (Ventricular myocardium, aplasia of right) (1)
  • Ulcerated Colitis (4)*
  • Ullich Disease (1)
  • Ulna Bone, absence, bilateral (arm bone) (3) *
  • Ulna Bone, deficiency (arm bone) (4) *
  • Undescended Testes (72)
  • Undiagnosed (498)
  • Unusual Facies Syndrome (5)
  • Unverricht-Lundborg (Baltic Myoclonus) (1)
  • Ureathra, Diverticulum (1)
  • Ureter Reconstruction (5)
  • Ureterostomy (5)
  • Urethra Fistula (2)
  • Urethra Valve Syndrome (6)
  • Urethra, Diverticulum (1)
  • Urogenital Sinus (2)
  • Urorectal Septum Malformation Sequence (2)
  • Urticaria Pigmentosa (9)
  • Urticaria, Cold (1)
  • Usher Syndrome Type II (1) *
  • Uveitis (pars plantis -dots in vision) (3) *
  • Uvulopalatopharyngoplasty (palate reconstruction) (2)
  • VATER Syndrome or Vacterl Association (80) **
  • Vaccine Reaction to HIB Vaccine (6) *
  • Vaccine Reaction to Measles Vaccine (9) *
  • Vaccine Reaction to Pertussis (DPT) Vaccine (306) *
  • Vaccine Reaction to Polio Vaccine (5) *
  • Vaccine, Sudden Deafness after Flu vaccine (1)

83. Health Library -
Oncology Wilms Tumor Wilms tumor have one of three genetic syndromes, including the following.wagr syndrome - the acronym WAGR stands for the four diseases present in WAGR
http://12.42.224.152/library/healthguide/en-us/SelfHelp/topic.asp?hwid=shc29wag

84. Glossary In Congenital Malformations NeuroLearn NeuroHelp
wagr syndrome Wilms’ tumor, aniridia, genitourinarytract malformation, andmental retardation. Waaler-Aarskog syndrome Questionable autosomal dominant
http://moon.ouhsc.edu/kfung/JTY1/NeuroHelp/ZNG0BS01-W.htm
Glossary in Congenital Malformations NeuroLearn NeuroHelp Malformations General ... Z General Syndromes and Sequences Head #WAGR syndrome: Wilms’ tumor, aniridia, genitourinary-tract malformation, and mental retardation. #Waaler-Aarskog syndrome: Questionable autosomal dominant transmission with variable penetration. It includes hydrocephalus, imperforated anus, congenital heart disease, tracheo-esophageal fistula, vertebral and limb abnormalities. #Walker-Warburg Syndrome: Walker-Warburg syndrome is a form of cerebro-ocular dysplasia (muscle-eye-brain syndrome) characterized by hydrocephalus, agyria (a migration disorder), retinal dysplasia or detatchment, and encephalocele (HARD+E). There are also defects of the anterior chamber of the eyes. CNS m alformations: The brain abnormalities include type II lissencephaly, hydrocephalus, malformed cerebellar vermis, marked subpial gliosis, and abnormal cortical vascular architecture. Affected infants are very hypotonic from birth with microcephaly, severe mental retardation, and a variety of ocular abnormalities are the most obvious clinical abnormalities. Usually lethal within the first 4 months of age. #Wildervanck syndrome (oculo-acoustic-cervical syndrome): Congenital perceptive deafness (usually severe and sensataional in sensorineural in type), Kli

85. Index W: Contact A Family - For Families With Disabled Children: Information On
wagr syndrome see Aniridia WWS see Lissencephaly WaardenburgHirschsprung diseasesee Waardenburg syndrome Waardenburg syndrome
http://www.cafamily.org.uk/Idx/w.html
printer friendly home how we can help medical information ... how you can help Please use the index below to access the condition you require information on. It may take longer to find what you are looking for this way compared with our "search this site" facility in the navigator on the left but we try to point you in the most appropriate direction using this index and therefore the results should be better. For speed, this index has been split into separate alphabetical files: numbers 0-9 A B C ... Z Contact a Family also has information on many other specific conditions and rare disorders. If you cannot find the information you require in The Contact a Family Directory Online , you may wish to use our Contact a Family Helpline service. WAGR syndrome see Aniridia
WWS see Lissencephaly
Waardenburg-Hirschsprung disease see Waardenburg syndrome
Waardenburg syndrome

Walker-Warburg syndrome see Congenital Muscular Dystrophy and Lissencephaly
Weil's disease see Meningitis
Werdnig-Hoffman disease see Spinal Muscular Atrophy
Wermer syndrome see Multiple Endocrine Neoplasia Type I
West Nile Encephalitis see Arboviral Encephalitides
West syndrome

Whistling Face Syndome see Freeman Sheldon syndrome White Breath Holding see Reflex Anoxic Seizures Wiedemann-Rautenstrauch syndrome see Metabolic diseases Wildervanck syndrome see Klippel-Feil syndrome Williams-Beuren syndrome see Williams syndrome Williams syndrome Wilm's tumour see Aniridia Wilson's disease see Liver disease Winchester syndrome see Mucopolysaccharide diseases and associated diseases Wiskott-Aldrich syndrome see

86. HEALTHMEDNET
Waardenburg Syndrome. wagr syndrome. Waldenstrom s Macroglobulinemia. Waldmann Disease.Walker Warburg Syndrome. Walking aid. Walking Pneumonia
http://www.epscorp.com/healthmednet/w.htm
Directory Index HEALTHMEDNET Example
Report
Request ... DataBase Note:

The names were derived from the specific illness/disease source directories. Therefore, there are some variations in the names. When ordering a list of URLs for your illness/disease, where possible, include the general usage, technical, acronym, and/or abbreviation names. Waardenburg Syndrome WAGR Syndrome Waldenstrom's Macroglobulinemia Waldmann Disease Walker Warburg Syndrome Walking aid Walking Pneumonia Wallenberg's Syndrome Wandering Spleen Wart Wart, Plantar Wart, Venereal Warts Warts (Common Warts) Warts, Genital Warts, Genital (Genital Warts (Women)) Wasp (Insect Sting Allergies) Wasserman Test Wasting Syndrome Water on the knee Water Retention Water-Electrolyte Imbalance Waterhouse-Friderichsen Syndrome Watering Eyes Weakness Weakness and Fatigue Weal Weaming Weaver Syndrome Webbing of the fingers or toes Weber-Christian Disease Weber's test Wegener's Granulomatosis Weight Weight (Excess) Weight Control Weight Issues Weight loss Weight Loss and Dieting Weight Loss And Maintenance Weight Loss with Thyroid Disease Weight Loss/Dieting Weight Management Weight problem Weil Syndrome Weill Marchesani Syndrome Weil's Disease Weismann Netter Stuhl Syndrome Weismann Netter Stuhl Syndrome Wells Syndrome Wen Werdnig Hoffman Disease Werner Syndrome Wernicke's Encephalopathy (Cerebral Beriberi) West African Trypanosomiasis West Syndrome Wet dream Wet Eyes Wetting the bed Wheals Wheelchair Wheezing Whiplash Whiplash Injury Whipple's Disease Whipworm White Blood Cell White Blood Cells, Neutrophils, and G-CSF

87. Government And Academic Resources On Wagr Syndrome
Government and academic resources on wagr syndrome.
http://books.mongabay.com/health/conditions/Wagr_Syndrome.html
Wagr Syndrome Resources
U.S. Government resources on Wagr Syndrome
WAGR syndrome

WAGR syndrome. Examinations Photographs Movies Links Home Search noJava Home.
photographs

...ulnar-mammary syndrome Ventricular septal defects WAGR syndrome Williams
Mendelian: WAGR Syndrome

Obesity: a new feature of WAGR (del 11p) syndrome. Obesity and WAGR syndrome.
paper: Morbid obesity and hyperphagia in the WAGR syndrome

WAGR Syndrome/complications/genetics. Refers_to, Mendelian, WAGR Syndrome.
The Rare Disease Catalog TZ

Rare Disease Catalog: AC
Penn State Faculty Research Expertise Database (FRED) Complices, WAGR, Syndrome, WAGR. Syndromes, WAGR, WAGR Complices. WAGR Syndromes, HighWire Browse Journals - WAGR Syndrome Browse Journals publishing on WAGR Syndrome:, (return to Topic List page) Health Library - Provides information and support to persons with WAGR Syndrome or aniridia, myhealth.ucsd.edu/library/healthguide/en-us/illnessconditions/topic.asp?hwid=nord1014 MEIOSIS IN HUMAN FEMALES IS PAUSED AT TWO DIFFERENT POINTS ... File Format: PDF/Adobe Acrobat - View as HTML Wilms Tumor WAGR syndrome - the acronym WAGR stands for the four diseases present in WAGR A to Z Encyclopedia Topic: Wilms` Tumor WAGR syndrome - the acronym WAGR stands for the four diseases present in WAGR Results of the search Focal segmental glomerulosclerosis in solitary kidney in WAGR syndrome.

88. Search By Disease
wagr syndrome. 17, WalkerWarburg syndrome. 18, Warburg syndrome. 19,Ward-Romano syndrome (WRS). 20, WBS. 21, Werdnig Hoffmann disease
http://www.eddnal.com/directory/disease.php?letter=W&page=2

89. l–¼A’n–¼‚ðŠ¥‚µ‚½Çó•a–¼ŠŒ©W@“Œ‘å•a‰@ŒŸ
Von Willebrand facter. Voodoo death. wagr syndrome. WardRomano syndrome.Water-hammer pulse. Wegener s granulomatosis. Wegener s granulomatosis
http://square.umin.ac.jp/kennsa/echocardiography/text/disease_with_named.html
l–¼A’n–¼‚ðŠ¥‚µ‚½Çó•a–¼ŠŒ©W@“Œ‘å•a‰@ŒŸ¸•”@ˆÉˆä–쏁Žq‹ZŽt
Addison's disease Alagille syndrome Albright syndrome Allen test Alveolar proteinosis Alveolar-capillary membrane,in pulmonary edema Alzheimer disease Amplatz catheters Angelman syndrome Ankylosing spondylitis Apert syndrome Aschoff nodule Ashman phenomenon Austin Flint murmur Azygos vein Bachmann's bundle Bangungut Barth syndrome Bartter syndrome Bat wing edema Batten disease Bayes theorem Bayesian theory Beall mitral valve prosthesis Becker Becker muscular dystrophy Bed rest Behcet's disease Beriberi Bernheim effect Bernoulli equation Bernstein test Bezold-Jarisch reflex Bifid T wave Blacks Borg scale Bowditch effect Branham's sign Broadbent's sign Brockenbrough maneuver Bronchiolitis obliterans Bruce protocol BS•Ù •Ù‚Ì–‡”‚Í‚P–‡ Bulboventricular loop Bundle of His Bundles of Kent Butterfly wing edema Cafe coronary Carey-Coombs Carpenter syndrome, genetic facter Carpentier-Edwards valve Carvallo's sign, in tricuspid regurgitation Casoni skin test Cat-eye syndrome Chagas' disease Charcot-Marie-Tooth syndrome Cheyne-Stokes respiration Churg-Strauss syndrome Creuzfeldt-Jacob disease(CJD) Cogan's syndrome Conradi-Hunermann syndrome Corrigan (water-hammer) pulse Cromakalim Crouzon's syndrome Cushing's disease Cushing's syndrome Da Costa syndrome Dallas criteria Damus-Kaye-Stansel procedure De Musset's sign DeBakey DiGeorge syndrome Down syndrome Dressler syndrome Duchenne muscular dystrophy Duroziez's sign Earlobe crease

90. Current Opinion In Oncology - UserLogin
wagr syndrome consists of W ilms tumor, a niridia, g enital anomalies, Children with wagr syndrome carry constitutional deletions in a number of genes,
http://www.co-oncology.com/pt/re/cooncology/fulltext.00001622-200105000-00014.ht
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91. Definitions Of Genetic Disorders-W
Wilms TumorAniridia-Gonadoblastoma-Mental Retardation syndrome wagr.htm WilmsTumor Aniridia Gonadoblastoma Mental Retardation aniridia.htm
http://www.icomm.ca/geneinfo/def-w.htm

92. WAGR, Syndrome : Arborescences MeSH
page PubMed du motclef page CISMeF du motclef
http://www.chu-rouen.fr/navimesh/W/naviwagrsyndrome.html
WAGR, syndrome : arborescences MeSH Menu général CISMeF Vous pouvez aussi consulter toutes les arborescences des mots clés utilisés dans CISMeF

93. Gene: [11p13/WT1] Wilms Tumor Suppressor 1; Wilms Tumor 1 (nephroblastoma; Mesot
Wilms tumor suppressor 1; Wilms tumor 1 (nephroblastoma; mesothelioma?); wagrsyndrome (MIM194072); DenysDrash syndrome (MIM194080); wagr WTCR1
http://obi.img.ras.ru/humbio/hugen/11p13_WT1.htm
Gene: [11p13/WT1] Wilms tumor suppressor 1; Wilms tumor 1 (nephroblastoma; mesothelioma?); WAGR syndrome (MIM:194072); Denys-Drash syndrome (MIM:194080); [WAGR WTCR1 ]
GEN The gene length: 50 kbp; exons: 10 (Haber-1991). FUN The WT1 protein isoforms have several characteristics of zinc-finger transcription factors and are thought to regulate gene expression during mesenchymal to epithelial cell differentiation (Pritchard-Jones-1990) especially in the development of the urogenital system." PAT Wilms tumor (WT) is one of the most common solid tumors of childhood, occurring in 1 in 10,000 children and accounting for 8% of childhood cancers. It is believed to result from malignant transformation of abnormally persistent renal stem cells which retain embryonic differentiation potential (Breslow-1982)." MUT By PCR-SSCP analysis of 98 sporadic Wilms tumors, Varanasi-1994 found that mutations in the WT1 gene are rare, occurring in only 6 tumors analyzed. In 1 sample, 2 independent intragenic mutations inactivated both WT1 alleles, providing a singular example of 2 different somatic alterations restricted to the WT1 gene. The data, together with the previously ascertained occurrence of large deletions/insertions in WT1, defined the frequency at which the WT1 gene is altered in sporadic tumors." HET [1] This locus was previously assumed to be related with the combined syndrome WAGR, which included, in addition to Wilms tumor, aniridia, gonadoblastoma, and mental retardation (AGR triade; Riccardi-1978). Many authors found WAGR to be accompanied by deletions in the chr 11 short arm, so WAGR locus was considered as being located in segment 11p13. However, aniridia and genital abnormalities were later demonstrated to occur independently of both Wilms tumor and each other. Therefore, the WAGR locus was excluded from gene catalogues and replaced by three independent genes - GEM:

94. American College Of Surgeons JACS Reprint
In contrast to wagr, children with the DenysDrash syndrome with Wilms tumorhave only point mutations of the WT1 gene.13 These patients account for
http://www.facs.org/jacs/articles/grosfeld3.html
WILMS' TUMOR Wilms' tumor is the most common embryonic malignancy of renal origin. Approximately 500 new patients are seen annually in the United States.13,14 Although the overall management of children with Wilms' tumor is one of the great success stories in cancer therapy and currently more than 90% of children survive 4 years after their diagnosis, there is still room for improvement. Genetic considerations Children susceptible to Wilms' tumor are born with a constitutional DNA mutation in one allele of a gene. One copy of a presumed tumor suppressor gene mutation is inherited from one parent and results from a spontaneous mutation. Under these circumstances, a new genetic event such as deletion of inhibition of the paired allele of the gene would be needed for tumorigenesis to occur. This genetic presentation increases the likelihood of bilateral tumors and earlier age at onset as compared with sporadic cases, in which tumorigenesis requires two independent mutations.2,5,6 Two-event activation of a tumor suppressor gene occurs at two different genetic sites. The first allele is inactivated by mutation of the gene itself; the second allele is inactivated by loss of heterozygosity (a loss of chromosomal material). Wilms' tumor occurring in children with aniridia, genitourinary abnormalities, and mental retardation is known as the WAGR syndrome.2,13 Wilms' tumor will develop in 30% of these children. The karyotypic analysis of these patients demonstrates a deletion of the short arm (the p arm) of one copy of chromosome 11 at the 11p13 locus. The syndrome actually encompasses a number of contiguous genes, including the aniridia gene

95. Aniridie SNOF
syndrome wagr des anglo-saxons (Wilms tumor, Le syndrome wagr est en rapport avec le gène WT1 qui est le gène
http://www.snof.org/maladies/aniridie.html
Accueil Annonces Art Histoire ... Homepage
Aniridie
Syndrome WAGR et syndrome de Gillespie
Aniridia
Rev 02-03-2003
jmm
Aniridie
aniridie comme . Cette anomalie est rapidement visible chez le petit enfant chez l'ophtalmologiste. Cette pathologie rare glaucome
Clinique
L' absence d'iris mydriase ). Il existe toujours une mince collerette d'iris zonule Des Il s'y associe souvent une dystrophie limbique glaucome Une glaucome L'atteinte maculaire est quasi constante, avec un absence de macula ou une hypoplasie maculaire nystagmus et une basse vision (amblyopie). Une cataracte ou des peuvent s'ajouter au tableau clinique.
Bilan familial
type de transmission de cette anomalie, dans la famille de l'enfant. aniridie autosomique dominante par haplo-insuffisance embryologie de l'oeil le premier cas avec une transmission autosomique dominante dans le cadre d'une (mutation de
Pr Patrick Calvas CHU Purpan Toulouse France La SSCA S ingle S trand C onformation A nalysis) et la DGGE D enaturing G radient G el E anomalie chromosomique , une s'accompagne du syndrome WAGR des anglo-saxons ( W ilms tumor

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