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         Von Hippel-lindau Disease:     more detail
  1. The Official Patient's Sourcebook on Von Hippel-Lindau Disease: A Revised and Updated Directory for the Internet Age by Icon Health Publications, 2002-09
  2. Von Hippel-Lindau disease: An entry from Thomson Gale's <i>Gale Encyclopedia of Neurological Disorders</i> by Deepti, MS, CGC Babu, 2005
  3. Von Hippel-Lindau's disease: Analysis of age of onset and gene expression in a human genetic disease ([Theses for the degree of Master of Science - University ... of Hawaii ; no. 1644 : Biomedical Sciences) by Margaret Marshall, 1979
  4. Gale Encyclopedia of Cancer: von Hippel-Lindau disease by M.S., C.G.C. Laura L. Stein, 2002-01-01
  5. Genodermatoses: Turner Syndrome, Joubert Syndrome, Neurofibromatosis, Von Hippel-lindau Disease, Tuberous Sclerosis, Freeman-Sheldon Syndrome
  6. Von Hippel-Lindau disease: an overview.(Continuing Nuring Edcuation)(Disease/Disorder overview): An article from: Nephrology Nursing Journal by Michele Inglese, 2007-07-01
  7. Von Hippel-Lindau Disease Tumor Suppressor Gene (Medical Intelligence Unit Series) by E. Maher, 1996-10
  8. VHL Handbook Kids' Edition: A handbook for parents and kids living with von Hippel-Lindau (Volume 0) by Melissa Kruger, 2009-05-18
  9. Von Hippel-Lindau Syndrome - A Bibliography and Dictionary for Physicians, Patients, and Genome Researchers by Philip M. Parker, 2007-07-19
  10. Von Hippel-Lindau syndrome: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by Mary, MS Freivogel, 2005
  11. What You Need to Know about VHL: A reference handbook for people with von Hippel-Lindau, their families, and support personnel by VHL Family Alliance, 2009-06-18
  12. von HippelLindau disease: An entry from Thomson Gale's <i>Gale Encyclopedia of Cancer, 2nd ed.</i> by Laura, M.S., C.G.C. Stein, Rebecca, PhD Frey, 2006
  13. Neurocutaneous Disorders: Phakomatoses & Hamartoneoplastic Syndromes

61. Log In Problems
A 34year-old white man with von Hippel-Lindau (VHL) disease was referred to the urology service because of a tumor in the left kidney and bilateral
http://www.medscape.com/viewarticle/410218
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62. Von Hippel-Lindau Syndrome - Patient UK
Clinical features and natural history of von hippellindau disease. Couch V, Lindor NM, Karnes PS, Michels VV.von hippel-lindau disease.
http://www.patient.co.uk/showdoc/40001296/
PatientPlus articles are written for doctors and so the language can be technical. However, some people find that they add depth to the articles found in the other sections of this website which are written for non-medical people.
Von Hippel-Lindau Syndrome
This is an hereditary cancer syndrome characterised by the development of vascular tumours of the central nervous system and retina, renal carcinomas, phaeochromocytomas , pancreatic islet cell tumours, endolymphatic sac tumours, and benign cysts affecting a variety of organs. It is one of several neurocutaneous syndromes called phakomatoses. Inheritance Autosomal dominant . The VHL gene is a tumour suppressor gene located on the short arm of chromosome 3 at loci 3p25-26. Inactivation or loss of the remaining wild-type allele in a susceptible cell may lead to tumour development. Incidence 1:36,000 live births Clinical features Common Neoplasms in Von Hippel-Lindau Syndrome
  • Adrenal phaeochromocytomas (7% to 19%). Screen before surgery ( urinary catecholamines) in combination with CT and ultrasound.
  • Epididymal cystadenomas (in 10% to 54% of patients).

63. The Bioline EPrints Archive - Pancreatic Involvement In Von Hippel-Lindau Diseas
BACKGROUND Involvement of the pancreas in von hippellindau disease that is a tumor predisposing syndrome mentioned in literature with some morbid and
http://bioline.utsc.utoronto.ca/archive/00002954/
@import url(http://bioline.utsc.utoronto.ca/eprints.css); @import url(http://bioline.utsc.utoronto.ca/eprints.css); @import url(http://bioline.utsc.utoronto.ca/print.css);
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Pancreatic involvement in Von Hippel-Lindau disease
Eras, Mustafa and Yenigun, Mustafa and Acar, Cengiz and Kumbasar, Baki and Sar, Fuat, and Bilge, Turgay Pancreatic involvement in Von Hippel-Lindau disease Indian Journal of Cancer pp. 159-161 Full text available as:
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Abstract
Item Type: Journal (Paginated) Keywords: Von Hippel-Lindau, pancreatic, involvement, cysts, renal cell carcinoma. cn04030 Subjects: Biological Sciences Botany ID Code: Deposited By: Valentine, Miss Kimberly Deposited On: 23 March 2005 Alternative Locations: http://www.bioline.org.br/request?cn04030 Repository Contact Information © 2005 Bioline International

64. Entrez PubMed
von hippellindau disease is a hereditary cancer syndrome characterized by VHL disease is caused by germline mutations of the von Hippel-Lindau tumor
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=9

65. Calculli L, Et Al. Pancreatic Involvement In Von Hippel-Lindau Disease: The Role
von hippellindau disease is a rare dominant autosomal genetic disease Sano T, Horiguchi H. von hippel-lindau disease. Microsc Res Tech 2003; 60159-64.
http://www.joplink.net/prev/200507/09.html
MULTIMEDIA ARTICLE - Clinical Imaging
Article in PDF format
JOP Home page JOP. J Pancreas (Online) 2005; 6(4):375-379.
Pancreatic Involvement in Von Hippel-Lindau Disease: The Role of Integrated Imaging
Lucia Calculli , Marta Fiscaletti , Riccardo Casadei , Raffaele Pezzilli , Emilia Pascali , Giampaolo Gavelli
Departments of Radiology, Surgery and Internal Medicine, Sant’Orsola-Malpighi Hospital. Bologna, Italy Von Hippel-Lindau disease is a rare dominant autosomal genetic disease determined by the inactivity of an onco-suppressor gene identified in 1993 and located on the short arm of chromosome 3 [ ]. This gene has a role of crucial importance in the regulation of the vascular endothelial growth factor (VEGF); therefore, its inactivation promotes an overexpression of the VEGF and the angiogenesis of the tumor characterizing this syndrome [ ]. The main clinical and radiological manifestations of the disease are hemangioblastoma of the retina and of the nervous central system, clear cell renal tumors, pheochromocytoma, cystadenoma of the epididymis, tumors of the endolymphatic sack, carcinoid tumors, pancreatic tumors, and multiple cystic formations of the pancreas as well as of the kidneys and the epididymis [ ]. Pancreatic gland involvement ranges from 15 to 77% of the cases [

66. Serum Markers Of Angiogenesis In Patients With Von Hippel-Lindau Disease -- Spau
Background von hippellindau disease is an autosomal dominant genetic cancer predisposition syndrome characterized by hemangioblastomas of the retina and
http://meeting.jco.org/cgi/content/abstract/22/14_suppl/1033
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 Journal of Clinical Oncology , 2004 ASCO Annual Meeting Proceedings (Post-Meeting Edition).
Vol 22, No 14S (July 15 Supplement), 2004: 1033
American Society of Clinical Oncology

This Article Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Download to citation manager PubMed Articles by Spaulding, J. Articles by Tomlinson, G. E.
Abstract
Serum markers of angiogenesis in patients with von Hippel-Lindau Disease
J. Spaulding R. Brekken L. Robinson K. Koeneman B. Mickey J. Cadeddu C. Croft A. Sagalowsky N. Ahmad and G. E. Tomlinson University of Texas Southwestern Medical Center, Dallas, TX; Children's Medical Center of Dallas, Dallas, TX Background: Von Hippel-Lindau disease is an autosomal dominant genetic cancer predisposition syndrome characterized by hemangioblastomas of the retina and cerebellum, renal cell carcinoma, and other

67. Von Hippel-Lindau Disease
von hippellindau disease. By Cullen A Taylor and Javad Towfighi Developmental malformations of the nervous system von hippel-lindau disease
http://www.medlink.com/PublicCIP.ASP?access=public&UID=MLT0001F&code=

68. UniProtKB/Swiss-Prot Entry P40337 [VHL_HUMAN] Von Hippel-Lindau Disease Tumor Su
Germline mutations in the von hippellindau disease (VHL) gene in families DISEASE Defects in VHL are the cause of von hippel-lindau disease (VHLD)
http://us.expasy.org/uniprot/P40337
ExPASy Home page Site Map Search ExPASy Contact us Swiss-Prot Search Swiss-Prot/TrEMBL Swiss-Prot/TrEMBL (full text) PROSITE SWISS-2DPAGE ENZYME NEWT Taxonomy HAMAP families ExPASy web site for The ExPASy Server requires Javascript to be fully functional. You may not see all the information available for this page (More information)
UniProtKB/Swiss-Prot entry
Entry info Name and origin References Comments ... Tools
Note: most headings are clickable, even if they don't appear as links. They link to the user manual or other documents Entry information Entry name Primary accession number Secondary accession number Entered in Swiss-Prot in Release 31, February 1995 Sequence was last modified in Release 37, December 1998 Annotations were last modified in Release 48, September 2005 Name and origin of the protein Protein name Von Hippel-Lindau disease tumor suppressor Synonyms pVHL
G7 protein
Gene name Name: VHL From Homo sapiens (Human) TaxID Taxonomy ... References
NUCLEOTIDE SEQUENCE (ISOFORMS 1 AND 2).
PubMed=8493574 [ NCBI ExPASy EBI Israel ... Zbar B. initAuthorHiding(1) Lerman M.I.

69. Von Hippel-Lindau Disease Term Quote Resources From Life Insurance .Net
von hippellindau disease term quote Life Insurance resources and quotes from local agents on whole and term life insurance policies.
http://www.lifeinsurance.net/LifeInsurance/Von_Hippel-Lindau_disease__term_quote

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70. Von Hippel-Lindau Disease Universal Life Quote Resources From Life Insurance .Ne
von hippellindau disease universal life quote Life Insurance resources and quotes from local agents on whole and term life insurance policies.
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Life insurance policies are uniquely tailored to your actual life situation. Your specific health conditions will affect how various companies price your policy or even whether they will take you. Don't let a cookie cutter agency take you to the wrong company. We understand your situation and can get you a great solution your insurance needs. Our Customers are Saying... "Your agent called me within 24 hours and wasknowledgeable, kind and professional. My wife and I boughtinsurance policies from him...
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71. The Health Library — Cardiovascular
von hippellindau disease. von hippel-lindau diseaseNINDS What is Von Hippel-Lindau?VHL Family Alliance Von Hippel-Lindau SyndromeStanford Cancer
http://healthlibrary.stanford.edu/resources/internet/bodysystems/cardio_lesion.h
lindau Diseases and Disorders Use these links to jump directly to your topic of interest in Cardiovascular: Heart Diseases: Angina Arrhythmia Congenital Heart Disease Coronary Artery Disease ... Other Heart Diseases Vascular Diseases: Aneurysms Aortic Diseases Hypertension (High Blood Pressure) Hypotension (Low Blood Pressure) ... Venous Diseases Cardiovascular Disease Treatment: Medication Surgery Heart Transplantation Other Topics: General Cardiovascular Anatomy Cholesterol Diagnostic Procedures ... Homocysteine Vascular Lesions (Jump to: Overviews Arteriovenous Malformations (AVMs) Cavernous Malformations (Angiomas) Hereditary Hemorrhagic Telangiectasia ... Von Hippel-Lindau Disease Overviews Arteriovenous Malformations and Other Vascular Lesions of the Central Nervous System Fact Sheet:NINDS Surgical Management of Cerebrovascular Disorders:UCSF Arteriovenous Malformations (AVMs) Brain Arteriovenous Malformations:Toronto Brain Vascular Malformation Study Group Dural Arteriovenous Malformations:Toronto Brain Vascular Malformation Study Group AVM Treatment:International Radiosurgery Association Recommendations for the Management of Intracranial Arteriovenous Malformation:American Stroke Association [Video] Cavernous Malformations (Angiomas) Cavernous Malformation:NINDS Cavernous Malformations of the Brain (Cavernomas):Toronto Brain Vascular Malformation Study Group Cavernous Angioma Information:Angioma Alliance Cavernous Malformations and Temporal Lobe Epilepsy:Angioma Alliance ... Cerebral Cavernous Malformation:Genetics Home Reference, NLM

72. Von Hippel-Lindau Disease
von hippellindau disease. Elongin is an elongation factor for RNA polymerase II. Interestingly, elongin interacts not only with RNA polymerase II but also
http://www.biochemistry.ucla.edu/biochem/Faculty/Martinson/Hippel.html
von Hippel-Lindau disease Elongin is an elongation factor for RNA polymerase II. Interestingly, elongin interacts not only with RNA polymerase II but also with the von Hippel-Lindau tumor suppressor protein. Mutations in the gene for this protein that prevent its interaction with elongin result in a hereditary predisposition to various types of cancer. The molecular basis for this predisposition is not yet understood. Back to Research Return to Home Page

73. Von Hippel-Lindau Syndrome - Genetics Home Reference
Where can I find additional information about von HippelLindau syndrome? Familial; Hippel-Lindau Disease; VHL syndrome; von hippel-lindau disease
http://ghr.nlm.nih.gov/condition=vonhippellindausyndrome
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Von Hippel-Lindau syndrome
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What is von Hippel-Lindau syndrome?
Von Hippel-Lindau syndrome is an inherited disorder characterized by tumors and fluid-filled sacs called cysts that can develop in many different parts of the body. Tumors may be either benign (noncancerous) or malignant (cancerous) and occur from early childhood to late adulthood. Tumors called hemangioblastomas or angiomas can develop in the brain and spinal cord, the kidneys, and the back of the eye (retina). These tumors are made up of newly formed blood vessels and are typically noncancerous. Other types of tumors, which may be cancerous, often occur in the kidneys, the adrenal glands (hormone-producing glands located above each kidney), the pancreas, certain reproductive organs, and the inner ear. Von Hippel-Lindau syndrome is sometimes divided into two types. Type 1 von Hippel-Lindau syndrome is associated with a high frequency of pheochromocytoma. This type of tumor, which is usually noncancerous, develops in the adrenal gland and can cause dangerously high blood pressure. Type 2 von Hippel-Lindau syndrome tends to involve fewer pheochromocytomas, but is associated with an increased risk of developing kidney cancer.

74. Entrez PubMed
with and without von hippellindau disease the role of genetic mutation, with von Hippel-Lindau (VHL) disease but can also occur sporadically.
http://ghr.nlm.nih.gov/gene=vhl/show/PubMed
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75. Karger Publishers
When to Look for von hippellindau disease in Gastroenteropancreatic Neuroendocrine Von Hippel-Lindau (VHL) disease is a progressive autosomal dominant
http://content.karger.com/ProdukteDB/produkte.asp?Doi=80740

76. Arch Ophthalmol -- Abstract: Clinical Characteristics Of Ocular Angiomatosis In
Retinal Hemangioblastoma in von hippellindau disease A Clinical and Molecular Hemangioblastomas of the Retina Impact of von hippel-lindau disease
http://archopht.ama-assn.org/cgi/content/abstract/117/3/371
Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress
Vol. 117 No. 3, March 1999 Featured Link E-mail Alerts Ophthalmic Molecular Genetics Article Options Full text PDF Send to a Friend Readers Reply Submit a reply Related articles in this issue Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Webster AR Moore AT Articles that cite this article ISI Web of Science (35) ... Contact me when this article is cited Topic Collections Neurology Topic Collection Alerts
Clinical Characteristics of Ocular Angiomatosis in von Hippel-Lindau Disease and Correlation With Germline Mutation Andrew R. Webster, FRCOphth Eamonn R. Maher, MD, FRCP Anthony T. Moore, FRCOphth
Arch Ophthalmol. To examine the epidemiologic and clinical characteristics of the ocular manifestations of von Hippel-Lindau (VHL) disease and to detect phenotype-genotype relationships of disease severity.

77. Arch Ophthalmol -- Abstract: Retinal Vascular Hamartoma In Von Hippel-Lindau Dis
OBJECTIVE To diagnose von hippellindau disease at an early stage in the presence Hemangioblastomas of the Retina Impact of von hippel-lindau disease
http://archopht.ama-assn.org/cgi/content/abstract/113/9/1163
Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress
Vol. 113 No. 9, September 1995 Featured Link E-mail Alerts ARTICLE Article Options Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Schmidt D Neumann HP Articles that cite this article Contact me when this article is cited
Retinal vascular hamartoma in von Hippel-Lindau disease
D. Schmidt and H. P. Neumann
Department of Ophthalmology, University Eye Hospital, Freiburg, Germany. OBJECTIVE: To diagnose von Hippel-Lindau disease at an early stage in the presence of atypical retinal lesions. DESIGN: Case series. METHODS: In an 11-year interdisciplinary clinical follow-up study of von Hippel-Lindau disease, 52 patients with retinal angiomas were investigated. RESULTS: Besides retinal angiomas, in five patients with von Hippel-Lindau disease

78. Central Nervous System Involvement In Von Hippel-Lindau Disease.
Fifty individuals with von hippellindau disease (VHL) were studied with gadolinium-enhanced magnetic resonance imaging (MRI) to determine the frequency and
http://www.arclab.org/medlineupdates/abstract_1985294.html
Aging Research Center Home Page All Previous Aging Related Articles On-line Medical Dictionary National Library of Medicine's PubMed directory of MEDLINE citations.
Central nervous system involvement in Von Hippel-Lindau disease.
- Filling-Katz MR, Choyke PL, Oldfield E, Charnas L, Patronas NJ, Glenn GM, Gorin MB, Morgan JK, Linehan WM, Seizinger BR, et al. Neurology 1991 Jan;41(1):41-6. Fifty individuals with Von Hippel-Lindau disease (VHL) were studied with gadolinium-enhanced magnetic resonance imaging (MRI) to determine the frequency and distribution of CNS lesions. The associated clinical features were also reviewed. Thirty-six (72%) of the 50 had 1 or more CNS tumors. The most frequently affected sites in the CNS excluding the retina were the cerebellum spinal cord (44%), and brainstem (18%). New regional predilections for the craniocervical junction and conus medullaris were demonstrated by this study. Forty-one percent of all VHL patients with CNS tumors were neurologically asymptomatic: cerebellar tumors (50%), spinal cord tumors (50%), and

79. Pheochromocytomas, Multiple Endocrine Neoplasia Type 2, And Von Hippel-Lindau Di
Thirtyeight percent of carriers of von hippel-lindau disease and 24 percent All patients in families with MEN-2 or von hippel-lindau disease should be
http://www.meb.uni-bonn.de/cgi-bin/mycite?ExtRef=MEDL/94019642

80. NEJM -- Sign In
Twelve of the 14 families with von hippellindau disease, with 53 of the 60 gene Identification of the von hippel-lindau disease tumor suppressor gene.
http://content.nejm.org/cgi/content/full/330/15/1090-a

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