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  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

81. NEJM -- Von Hippel-Lindau Disease
Images in Clinical Medicine from The New England Journal of Medicine von hippellindau disease.
http://content.nejm.org/cgi/content/short/340/24/1880
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Figure 1. Evaluation of a 35-year-old man with recent headache and unsteadiness and a history of blindness of the left eye for two years revealed normal blood pressure and renal function, a hemoglobin concentration of 18.2 g per deciliter, and a hematocrit of 0.59. T -weighted magnetic resonance imaging (MRI) of the brain showed a cerebellar tumor (arrow in Panel A), which was found at surgery to be a benign hemangioblastoma. After the tumor was removed, his hemoglobin concentration fell to 14.5 g per deciliter. Ocular examination showed retinal hemangioblastomas, which were treated with laser ablation and led Full Text of this Article
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82. Von Hippel-Lindau Disease Complicated By Pheochromocytoma -- Kashyap And Kashyap
Thomas G, Hillson R. A complicated case of von hippellindau disease. Postgrad Med J 2001;77477. Conlin PR, Faquin WC. Case records of the Massachusetts
http://pmj.bmjjournals.com/cgi/content/full/78/921/443

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The Fellowship of Postgraduate Medicine
LETTER
von Hippel-Lindau disease complicated by pheochromocytoma
A S Kashyap and S Kashyap Department of Medicine
Department of Hospital Administration, Armed Forces Medical College, Pune 411 040; India; Keywords: von Hippel-Lindau disease; pheochromocytoma We read with interest the excellent case report of von Hippel-Lindau disease complicated by pheochromocytoma.

83. Pancreatic Lesions In Von Hippel-Lindau Disease: Prevalence, Clinical Significan
Neuroendocrine Tumors of the Pancreas in von hippellindau disease Spectrum of Appearances at CT and MR Imaging with Histopathologic Comparison
http://www.ajronline.org/cgi/content/abstract/162/5/1091
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ARTICLES
Pancreatic lesions in von Hippel-Lindau disease: prevalence, clinical significance, and CT findings
DM Hough, DH Stephens, CD Johnson and LA Binkovitz
Department of Diagnostic Radiology, Mayo Clinic, Rochester, MN 55905. OBJECTIVE. The purposes of this study were to determine the nature, prevalence, and CT findings of pancreatic lesions in patients with von Hippel-Lindau disease and to determine whether identification of pancreatic cysts and neoplasms is important in establishing the diagnosis of von Hippel-Lindau disease. SUBJECTS AND METHODS. The medical records and radiologic images of 52 patients with von Hippel- Lindau disease who were evaluated at our institution between 1976 and 1992, and who at some stage

84. Von Hippel-Lindau Disease - Healthfinder®
A general overview of von hippellindau disease that includes a description of the disorder, treatment, prognosis and research information.
http://mentalhealth.about.com/library/h/docs/bld02063.htm
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von Hippel-Lindau Disease
internet URL
http://www.ninds.nih.gov/health_and_medical/disorders/vonhippe_doc.htm
sponsoring agency
National Institute of Neurological Disorders and Stroke, National Institutes of Health
description
A general overview of von Hippel-Lindau disease that includes a description of the disorder, treatment, prognosis and research information. Suggested reading for more in-depth information, a list of referrals where users may obtain additional resources about von Hippel-Lindau disease and links to other related NINDS publications are included. Keywords: Adults Consumer Resources von Hippel-Lindau Syndrome
From Leonard Holmes, Ph.D.

85. Von Hippel-Lindau Disease: Genetic, Clinical, And Imaging Features [published Er
Spectrum of Abdominal Imaging Findings in von hippellindau disease Hemangioblastomas of the Retina Impact of von hippel-lindau disease Invest.
http://radiology.rsnajnls.org/cgi/content/abstract/194/3/629
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REVIEWS
von Hippel-Lindau disease: genetic, clinical, and imaging features [published erratum appears in Radiology 1995 Aug;196(2):582]
PL Choyke, GM Glenn, MM Walther, NJ Patronas, WM Linehan and B Zbar
Diagnostic Radiology Department, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD 20892-1182. von Hippel-Lindau (VHL) disease is an autosomal dominant disorder that causes retinal hemangioblastomas, hemangioblastomas of the central nervous system, endolymphatic sac tumors, renal cell carcinomas, pancreatic cysts

86. Silent Adrenal Nodules In Von Hippel-Lindau Disease Suggest Pheochromocytoma --
Patients with von hippellindau disease who have a renal mass and von hippel-lindau disease was diagnosed in 1981 when retinal angiomas were found.
http://www.annals.org/cgi/content/full/120/6/485
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BRIEF REPORT
Silent Adrenal Nodules in von Hippel-Lindau Disease Suggest Pheochromocytoma
Brian S. Aprill Almond J. Drake David H. Lasseter ; and ... K. M. Mohamed Shakir
Von Hippel-Lindau disease is an autosomal-dominant disorder characterized by retinal angiomatosis, cerebellar hemangioblastoma, renal cell carcinoma, and, less commonly, pheochromocytoma. Lindau described adrenal tumors in association with this syndrome in 1927 . Not until 1953, however, did Glushien and coworkers first report pheochromocytoma in association with von Hippel-Lindau disease. A 1987 review summarized 338 cases of this disease in 62 kindreds in which pheochromocytoma was reported in 14% . Patients with von Hippel-Lindau disease who have a renal mass and clinically silent adrenal nodule often are difficult to treat, especially if renal surgery is needed. Physicians

87. Von Hippel-Lindau Syndrome / The Family Village
Links to chat rooms, web sites and information about von Hippel Lindau s disease.
http://www.familyvillage.wisc.edu/lib_vonh.htm
Von Hippel-Lindau Syndrome
Who to Contact
Where to Go to Chat with Others

Learn More About It

Web Sites
Who to Contact
VHL Family Alliance
171 Clinton Road
Brookline, Massachusetts, USA 02146
Fax: (617) 734-8233
e-mail: info@vhl.org
Web: http://www.vhl.org/
VHL (Von Hipple-Lindau Disease) Family Alliance's mission is to improve diagnosis, treatment, and quality of life for people with Von Hipple-Lindau Disease, serving individuals, families, and related medical professionals. They promote education of patients, families, the medical community, and the general public concerning the disease. The Alliance has local support groups, you may call the 800 number or e-mail them for information on the local chapter nearest you and they will assist in starting a new support group in an area where none is presently available. They do parent-to-parent matching for anyone who writes describing their situation and requesting a match. The alliance publishes a quarterly newsletter, VHL Family Forum , that is included in membership fees, and they have brochures available including, "What is VHL?,"

88. HONselect - Hippel-Lindau Disease
disease - von hippel-lindau Syndrome. Français, Maladie de von hippel-lindau
http://www.hon.ch/HONselect/RareDiseases/C10.562.400.html
List of rare diseases: English Deutsch
Language:
MeSH term:
Accepted terms:
English: Hippel-Lindau Disease - Cerebelloretinal Angiomatosis, Familial
- Lindau Disease
- von Hippel-Lindau Disease
- Familial Cerebello-Retinal Angiomatosis
- Lindau's Disease
- von Hippel-Lindau Syndrome
Français: Maladie de von Hippel-Lindau - Angiomatose rétinocérébelleuse - Angiomatose cérébello-rétinienne de von Hippel-Lindau - Angiomatose cérébellorétinienne de von Hippel-Lindau Deutsch: Hippel-Lindau-Syndrom - Von-Hippel-Lindau-Syndrom - Lindau-Krankheit - Zerebelloretinale Angiomatose, familiäre Español: Enfermedad de Hippel-Lindau - Angiomatosis Cerebelorretinal Familiar - Angiomatosis Retiniana - Enfermedad de Lindau - Enfermedad de von Hippel-Lindau Português: Doença de Hippel-Lindau - Angiomatose Cerebelorretiniana Familiar - Angiomatose Retiniana - Doença de Lindau - Doença de von Hippel-Lindau HONselect ressources Definition: Yes Articles: Yes Images: Yes News: No Conferences: No Clinical trials: Yes Web sites: English Yes Français Yes Deutsch No Español Yes Português No Home About us Site map Search ... Contact http://www.hon.ch/HONselect/RareDiseases/C10.562.400.html

89. Von Hippel-Lindau Syndrome
The gene for vonHippel Lindau disease (VHL) is found on chromosome 3, and is inherited in a dominant fashion. If one parent has a dominant gene,
http://www.ncbi.nlm.nih.gov/disease/VHL.html
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VHL

on chromosome 3
Databases
PubMed

the literature
LocusLink

collection of gene-related information
OMIM
catalog of human genes and disorders Information Fact sheet from the National Institute of Neurological Disorders and Stroke, NIH VON HIPPEL-LINDAU SYNDROME is an inherited multi-system disorder characterized by abnormal growth of blood vessels. While blood vessels normally grow like trees, in people with VHL little knots of blood capillaries sometimes occur. These knots are called angiomas or hemangioblastomas. Growths may develop in the retina, certain areas of the brain, the spinal cord, the adrenal glands and other parts of the body.

90. Von Hippel-Lindau Syndrome
von Hippel Lindau Syndrome information, national and international support groups, von Hippel Lindau disease Genetic, Clinical, and Imaging Features
http://www.kumc.edu/gec/support/von_hipp.html
von Hippel-Lindau syndrome
VHL Family Alliance
171 Clinton Road Brookline, Massachusetts 02445 Phone: 617.277.5667, 800.767.4VHL (answered in English, Spanish, and French) Fax: E-mail: info@vhl.org
info-es@vhl.org [Spanish] and info-fr@vhl.org [French] Web site: www.vhl.org/ www.vhl-europa.org (lists support groups throughout Europe) Languages: en español, en français, auf deutsch, dansk, Belgie, in het Nederlands, Japanese
Handbook is being translated into 12 language, including Chinese.
Also See:
Genetic counselors and clinical geneticists:

91. NeuroGenetics Links ~ Neurosurgery At Massachusetts General Hospital/Harvard
von Hippel Lindau disease Genetic, Clinical and Imaging Features National Cancer Institute Kidney Cancer Web Page; Galaxy.Net s VHL Links Page
http://neurosurgery.mgh.harvard.edu/NeurogeneticSurgery/NGlinks.htm

Neurogenetic Surgery Center

The Neurogenetics Clinic
The Neurofibromatosis Clinic
The Von Hippel-Lindau Clinic Neurogenetics Links Neurofibromatosis Links VHL Links ...
Neurogenetic Surgery Guestbook
"Links" are provided only as an informational resource as a "Patient-to-Patient" service. Please note that many of the links provided below are not on one of the MGH Neurosurgical Service servers and are not maintained by us or affiliated with any services provided by us. Other information sources for patients and families of patients with neurogenetic disorders

92. Radiology: Von Hippel Lindau Disease
Genetic, clinical and imaging features from the NIH.
http://www.cc.nih.gov/ccc/papers/vonhip/toc.html
Von Hippel Lindau Disease:
Genetic, Clinical and Imaging Features
Peter L. Choyke, M.D., Gladys M. Glenn, M.D., Ph.D., McClellan M. Walther, M.D., Nicholas J. Patronas, M.D., W. Marston Linehan, M.D., Berton Zbar, M.D.
Radiology (March) 146:629-642,1995
Table of Contents
Abstract
History
Clinical Manifestations
Genetics ...
References

Note: Figures 5-20 have been scanned at 50%. The full image can be viewed with an Helper Application that reads JPEG files and by double-clicking the image.
From the:
  • Diagnostic Radiology Department, Warren Grant Magnuson Clinical Center, Henry M. Jackson Foundation, National Institutes of Health, Bethesda, MD 20892
  • Cancer Diagnosis Branch, Division of Cancer Biology, Diagnosis and Centers, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892
  • Surgery Branch, Urologic Oncology Division, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892
  • Laboratory of Immunobiology, Frederick Cancer Research Facility, National Cancer Institute, National Institutes of Health, Frederick, MD 21701
    Corresponding Author
    Peter L. Choyke, M.D.
  • 93. M. D. Anderson Cancer Center - Von Hippel Lindau Disease
    von Hippel Lindau disease (VHL) is an inherited mutation of the VHL gene, which causes tumors to form in areas of the body that contain large numbers of
    http://www.mdanderson.org/diseases/vhl/
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    94. GeneReviews: Von Hippel-Lindau Syndrome
    Your browser does not support HTML frames so you must view von hippellindau Syndrome in a slightly less readable form. Please follow this link to do so.
    http://www.geneclinics.org/profiles/vhl/
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    95. Von Hippel Lindau Disease
    von Hippel Lindau disease is a rare inherited multi - system disorder characterized by the abnormal growth
    http://my.webmd.com/hw/health_guide_atoz/nord181.asp
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    Who We Are About WebMD Site Map You are in Medical Library Our Content Sources Ask A Question Clinical Trials Health Guide A-Z Health Topics Symptoms Medical Tests Medications ... For a Complete Report Von Hippel Lindau Disease Important It is possible that the main title of the report Von Hippel Lindau Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms
    • Angiomatosis Retinae Angiophakomatosis Retinae et Cerebelli Cerebelloretinal Hemangioblastomatosis Hippel Disease Hippel-Lindau Syndrome HLS Lindau Disease Retinocerebellar Angiomatosis VHL
    Disorder Subdivisions
    • None
    General Discussion Von Hippel-Lindau Disease is a rare inherited multi-system disorder characterized by the abnormal growth of blood vessels in certain parts of the body (angiomatosis). Very small blood vessels (capillaries) "knot" together to form benign growths known as angiomas. These may develop in the retinas of the eyes (retinoangioma) or in the brain (cerebellar hemangioblastoma). Benign growths may also occur in other parts of the brain, spinal cord, the adrenal glands (pheochromocytoma), and other parts of the body. The symptoms of von Hippel-Lindau Disease vary greatly and depend on the size and location of the growths. People with von Hippel-Lindau Disease are also genetically predisposed to certain types of malignant tumors (i.e., renal cell carcinoma).

    96. UrologyHealth.org Glossary: Von Hippel Lindau Disease
    von Hippel Lindau disease. Also referred to as VHL. A rare, genetic multisystem disorder characterized by the abnormal growth of tumors in certain parts of
    http://www.urologyhealth.org/glossary/popup.cfm?id=1241

    97. Von Hippel Lindau Disease, Renal Manifestations
    Complete online version of The Encyclopaedia of Medical Imaging including text and images from The Encyclopaedia of Medical Imaging s eight book volumes
    http://www.amershamhealth.com/medcyclopaedia/medical/Volume IV 2/VON HIPPEL LIN

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    our commitment our company Search Medcyclopaedia for: Search marked text (mark text before you click) Browse entry words starting with: A B C D ... amershamhealth.com Von hippel lindau disease, renal manifestations, von Hippel Lindau disease is an autosomal disorder, with a prevalence of 1 in 40,000, which predisposes to central nervous system haemangioblastomas and a variety of visceral tumours. The von Hippel Lindau disease gene has recently been identified on the short arm of chromosome 3. Genitourinary manifestations include renal cysts and tumours, adrenal tumours, and papillary cystadenoma of the epididymis or broad ligament. On CT, ultrasound and MRI, the renal cysts of von Hippel Lindau disease (which occur in 60% of patients) are indistinguishable from simple cysts, typically ranging in size from 0.5 to 3.0 cm ( Fig.1 ). The number of cysts is variable. Innumerable cysts may suggest the correct diagnosis. The cysts are not equivalent to simple cysts histologically they have a hyperplastic epithelium, which may be a premalignant change. Small lesions may be difficult to characterize with certainty. Thin section, contrast-enhanced CT is the best modality for surveillance. Guidelines for surveillance protocols vary, but an abdominal CT every year or every other year is a reasonable appproach. Renal cell carcinoma occurs in 2550% of von Hippel Lindau disease patients and is multicentric and bilateral in at least 75% ( Fig.2

    98. Von Hippel Lindau Disease
    Complete online version of The Encyclopaedia of Medical Imaging including text and images from The Encyclopaedia of Medical Imaging s eight book volumes
    http://www.amershamhealth.com/medcyclopaedia/medical/Volume IV 1/VON HIPPEL LIN

    financial services
    our commitment our company Search Medcyclopaedia for: Search marked text (mark text before you click) Browse entry words starting with: A B C D ... amershamhealth.com Von hippel lindau disease, (Eugen von Hippel, 18671939, German ophthalmologist and Arvid Lindau, 18921958, Swedish pathologist), autosomal dominant hereditary disorder, linked to chromosome 3p, and characterized by angiomata of the retina and haemangioblastoma. Other features include hypertension due to phaeochromocytoma, hypernephroma-like tumours, polycythaemia due to the renal tumour or the haemangioblastoma, haemangiomas of the spinal cord, lungs and adrenals, cysts, microcystic adenomas and neuroendocrine tumours of the pancreas, kidneys and liver, and epdidymal cystadenoma. Pancreatic lesions may be the only abdominal localization of von Hippel Lindau disease. The extent of the cystic lesions of the pancreas varies from a few cysts to polycystic transformation of the enlarged organ ( Fig.1 ). Microcystic adenomas (serous cystadenomas) are more rare. The cysts always contain serous fluid. No mucinous cysts have been reported. In general, cystic pancreatic lesions in von Hippel Lindau disease are asymptomatic or associated with mild symptoms only. Imaging characteristics of both neoplasms are similar to those of other true cysts of the pancreas. Neuroendocrine tumours in von Hippel Lindau disease consist of a combination of MEN I and MEN II syndromes (see

    99. Clinical Neurogenetics, Wake Forest
    von Hippel Lindau disease Genetic, Clinical and Imaging Features Stereotactic Radiosurgery for Hemangioblastomas of the Brain.
    http://www.bgsm.edu/bgsm/surg-sci/ns/ngenet.html
    Clinical Neurogenetics
    Department of Neurosurgery
    Wake Forest University School of Medicine Links to information on neurogenetic diseases treated by neurosurgeons: Neurofibromatosis, Tuberous Sclerosis, and von Hippel-Lindau Disease.
    Von Hippel-Lindau Disease and Hemangioblastoma

    100. A Family With Coexistent Von Recklinghausen's Neurofibromatosis And Von Hippel-L
    von Recklinghausen s neurofibromatosis (cutaneous neurofibromata, cafeau-lait spots), others have von Hippel- Lindau s disease (angiomatosis retinae,
    http://www.neurology.org/cgi/content/abstract/25/9/840
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    ARTICLES
    A family with coexistent von Recklinghausen's neurofibromatosis and von Hippel-Lindau's disease. Diseases possibly derived from a common gene
    PV Tishler
    A large kindred has two coexistent neurocutaneous syndromes: Certain members appear to have von Recklinghausen's neurofibromatosis (cutaneous neurofibromata, cafe-au-lait spots), others have von Hippel- Lindau's disease (angiomatosis retinae, renal cell carcinomas, pancreatic cysts), and at least one individual has a combined syndrome (neurofibromata, cafe-au-lait spots, pheochromocytomas, cerebellar hemangioblastoma, renal

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