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The available diagnostic and therapeutic possibilities allow to treat successfully all manifestations of von Hippel-Lindau disease. Adenocarcinomas of the kidneys, however, still present a serious therapeutic problem due to their mostly bilateral and multifocal occurrence.
O, Masek, V, Krislo
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Management of a Case of von Hippel-Lindau for Coronary Artery Bypass Grafting
von Hippel-Lindau (VHL) disease is a rare autosomal dominant genetic disorder with retinal and nervous system haemangioblastomas, phaeochromocytoma, renal, pancreatic and endolymphatic tumours.
Geetanjali S. Verma
doaj +1 more source
Von Hippel–Lindau disease [PDF]
von Hippel-Lindau (VHL) disease is an inheritable condition with an incidence of 1 in 36000 live births. Individuals with VHL develop benign and malignant tumors including retinal and central nervous system hemangioblastomas, clear cell renal cell carcinomas (RCC), pheochromocytomas, pancreatic neuroendocrine tumors and endolymphatic sac tumors (ELSTs).
Prashant, Chittiboina, Russell R, Lonser
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Von Hippel-Lindau's Disease [PDF]
SUMMARY:A patient with Von Hippel-Lindau's disease was followed from the time of diagnosis to the time of his death 13 years later. He was asymptomatic when the diagnosis was made, although a hemangioblastoma of the medulla was found on angiography. The patient's course and autopsy are described and the features of this disease are reviewed.
R G, Miller +3 more
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[Von Hippel-Lindau disease]. [PDF]
Von Hippel-Lindau disease (VHL) is an autosomal dominant inherited cancer syndrome. The disease was diagnosed in three patients: a 22-year-old woman who presented with decreased vision due to retinal angiomatosis and in whom a renal carcinoma was diagnosed five years later at a routine VHL analysis, a man aged 44 who died with bilateral renal cysts and
Los, M. +3 more
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Von Hippel–Lindau Disease [PDF]
Von Hippel-Lindau disease is an autosomal dominant syndrome which occurs secondary to germline mutations in the VHL tumor suppressor gene, located on chromosome 3. Clinically von Hippel-Lindau disease is characterized by an increased risk of developing simple visceral cysts, most commonly in the pancreas and kidneys, in addition to an increased risk of
Jennifer J, Findeis-Hosey +2 more
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Laparoscopic partial adrenalectomy for bilateral pheochromocytomas in a boy with von Hippel-Lindau disease [PDF]
Objectives: In adults, increasing numbers of adrenalectomies for pheochromocytomas are performed laparoscopically. We report for the first time laparoscopic bilateral subtotal adrenalectomy for pheochromocytomas in an 8-year-old boy with von Hippel ...
Elsner, Romy +5 more
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Isolated familial pheochromocytoma as a variant of von Hippel-Lindau disease. [PDF]
Inherited pheochromocytomas are often part of familial syndromes, especially multiple endocrine neoplasia type 2 (MEN 2), retinal cerebellar hemangioblastomatosis [von Hippel-Lindau (vHL) disease] or neurofibromatosis type 1.
Crossey, P.A. +7 more
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ABSTRACT Introduction and importance: Von Hippel-Lindau (VHL) disease is a rare autosomal dominantly inherited genetic condition. Von Hippel characterized the illness independently in 1911, and Lindau in 1926. Its prevalence is estimated to be about 1 in every 36,000 live births.
Neupane, Durga +5 more
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Structure-guided design and optimization of small molecules targeting the protein-protein interaction between the von hippel-lindau (VHL) E3 ubiquitin ligase and the hypoxia inducible factor (HIF) alpha subunit with in vitro nanomolar affinities [PDF]
E3 ubiquitin ligases are attractive targets in the ubiquitin-proteasome system, however, the development of small-molecule ligands has been rewarded with limited success.
Dias, David M +20 more
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