von Hippel-Lindau disease

(redirected from Hippel-lindau disease)

von Hippel-Lindau disease

Etymology: Eugen von Hippel, German ophthalmologist, 1867-1939; Arvid Lindau, Swedish pathologist, 1892-1958
a hereditary disease characterized by congenital, tumorlike vascular nodules in the retina and hemangioblastomas of the cerebellar hemispheres. Similar spinal cord lesions; cysts of the pancreas, kidneys, and other viscera; seizures; and mental retardation may be present. Also called cerebroretinal angiomatosis, Lindau-von Hippel disease, retinocerebral angiomatosis. Compare Hippel's disease.
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von Hippel-Lindau disease

von Hippel-Lindau disease

Molecular medicine An AD condition characterized by retinal angioma, CNS hemangioblastoma, renal cysts and CA, pheochromocytoma, pancreatic cysts, polycythemia 2º to ↑ erythropoietin production, epididymal cystadenoma.

Hippel disease

, von Hippel-Lindau disease (hip′ĕl) (von hip′ĕl-lin′dow″)
[Eugen von Hippel, Ger. ophthalmologist, 1867–1939; Arvid Lindau, Swedish pathologist, 1892–1958]
Angiomatosis of the retina and various areas of the body including the central nervous system, spinal cord, and visceral organs.

von Hippel-Lindau disease

An autosomal dominant genetic disorder with incomplete penetrance due to a mutation on chromosome 3. The principal feature is a single or multiple tumour of blood-forming tissue (haemangioblastoma) in the retina, the cerebellum, the brainstem or the spinal cord. These are highly vascular lesions that may grow dangerously at puberty or during pregnancy. Some secrete erythropoietin that leads to a large over-production of red blood cells. (Eugen von Hippel, 1867–1939, German ophthalmologist; and Arvid Lindau, b. 1892, Swedish pathologist).
References in periodicals archive ?
M2 EQUITYBITES-February 12, 2018-Elsevier signs partnership with VHL Alliance on PracticeUpdateFree virtual resource about von Hippel-Lindau Disease
M2 PHARMA-February 12, 2018-Elsevier signs partnership with VHL Alliance on PracticeUpdateFree virtual resource about von Hippel-Lindau Disease
The differential diagnosis of renal cysts with autosomal dominant inheritance pattern include ADPKD, tuberous sclerosis, medullary cystic kidney disease and Von Hippel-Lindau disease.
The tumor is very rare in the sporadic form, but more often associated with Von Hippel-Lindau disease.
Renal tumor size is an independent prognostic factor for overall survival in von Hippel-Lindau disease.
Treatment of intramedullary hemangioblastomas, with special attention to von Hippel-Lindau disease.
Clinical features and natural history of von Hippel-Lindau disease.
Clear cell endocrine pancreatic tumor, particularly in the setting of von Hippel-Lindau disease, is known to closely mimic metastatic CCRCC, because tumor cells in this group of neoplasm may be arranged in cords, nests, and tubules, all of which can be seen with central hemorrhage, simulating CCRCC.
Her family history was significant for von Hippel-Lindau disease (VHL), affecting her father and paternal grandmother.
Pheochromocytoma has long been estimated to be hereditary in 10-15% of cases and may occur as part of Von Hippel-Lindau disease (VHL Gene), MEN (Multiple Endocrine Neoplasia) Syndrome Type IIA (medullary carcinoma of thyroid, pheochromocytoma, and parathyroid adenoma) (RET gene) or MEN IIB (medullary carcinoma of thyroid, Pheochromocytoma, Marfan's and neurofibromatosis) (NF1 gene).
Von Hippel-Lindau disease, which is also excluded in the sitting of normal kidneys.
Summary of the mutations in the Chinese families with Von Hippel-Lindau disease