Von Hippel-Lindau disease
| Autor: | Stéphane Richard, Joyce W. Graff, F. Resche, Jan Lindau |
|---|---|
| Rok vydání: | 2004 |
| Předmět: |
Adult
Male Pathology medicine.medical_specialty von Hippel-Lindau Disease endocrine system diseases Disease urologic and male genital diseases Endolymphatic sac Diagnosis Differential Neoplasms Multiple Primary Central nervous system disease medicine Humans cardiovascular diseases Von Hippel–Lindau disease neoplasms business.industry Vascular disease Autosomal dominant trait General Medicine History 20th Century medicine.disease female genital diseases and pregnancy complications medicine.anatomical_structure Pancreatic cysts Differential diagnosis business |
| Zdroj: | The Lancet. 363:1231-1234 |
| ISSN: | 0140-6736 |
| DOI: | 10.1016/s0140-6736(04)15957-6 |
| Popis: | von Hippel-Lindau disease The disease that has perpetuated the names of two prestigious European physicians, Eugen von Hippel and Arvid Lindau, is a familial syndrome characterised by the occurrence of highly vascular tumours in different organs. The main manifestations are haemangioblastomas of the retina and CNS, clear-cell renal cell carcinomas and renal cysts, phaeochromocytomas, neuroendocrine pancreatic tumours and pancreatic cysts, and endolymphatic sac tumours. von Hippel-Lindau disease is an autosomal dominant disease with an incidence of about one in 36 000 people, and with clinical manifestations usually becoming apparent between 18 and 30 years. Clinical Von Hippel-Lindau disease |
| Databáze: | OpenAIRE |
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