## Abstract Von HippelβLindau (VHL) disease is an uncommon, autosomal dominant hereditary multitumor syndrome caused by germline alterations of the __VHL__ gene, which has been cloned recently and identified as a tumor suppressor gene. The major lesions in VHL disease include hemangioblastomas in t
Anesthesia and von Hippel-Lindau disease associated with pheochromocytoma
β Scribed by Yuko Hoshino; Hidefumi Obara; Katsuya Mikawa; Seizo Iwai
- Publisher
- Springer
- Year
- 1987
- Tongue
- English
- Weight
- 439 KB
- Volume
- 1
- Category
- Article
- ISSN
- 0913-8668
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Von Hippel-Lindau disease (VHL) is an autosomal dominantly inherited disorder, characterised by the development of clear cell renal carcinomas, CNS hemangioblastomas, retinal angiomas, pancreatic tumors, pheochromocytomas and hepatic cysts. Recently a number of families with dominant familial pheoch
## Abstract The Von HippelβLindau __(VHL)__ gene product has a wide spectrum of tissueβspecific functions, and specific germline mutations are associated with clinical phenotypes in VHL disease. In particular, missense mutations are correlated with the susceptibility to pheochromocytomas. An associ