von Hip·pel-Lin·dau syn·drome

(făn hip'el lan'dow), [MIM*193300]

a type of phacomatosis, consisting of retinal vascular malformations, which may be multiple and bilateral, associated with hemangioblastomas primarily of the cerebellum and walls of the fourth ventricle, occasionally involving the spinal cord; sometimes associated with renal cell carcinomas or cysts or hamartomas of kidney, adrenal, or other organs; autosomal dominant inheritance due to mutation in the von Hippel-Lindau gene (VHL) on 3p.

Synonym(s): cerebroretinal angiomatosis, Hippel disease, Lindau disease, von Hippel disease

von Hip·pel-Lin·dau syn·drome

(făn hip'el lan'dow), [MIM*193300]

Synonym(s): cerebroretinal angiomatosis, Hippel disease, Lindau disease, von Hippel disease

Farlex Partner Medical Dictionary © Farlex 2012

Lindau,

Arvid Wilhelm, Swedish pathologist, 1892-1958.

Lindau disease - Synonym(s): von Hippel-Lindau syndrome

Lindau tumor - a benign cerebellar neoplasm. Synonym(s): hemangioblastoma

von Hippel-Lindau syndrome - see under von Hippel

von Hippel,

Eugen, German ophthalmologist, 1867-1939.

Hippel keratoplasty

Hippel trephine

von Hippel disease - retinal hemangiomatosis.

von Hippel-Lindau syndrome - a type of phacomatosis, consisting of hemangiomas of the retina associated with hemangiomas or hemangioblastomas primarily of the cerebellum and walls of the fourth ventricle, occasionally involving the spinal cord. Synonym(s): cerebroretinal angiomatosis; Lindau disease

Medical Eponyms © Farlex 2012

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References in periodicals archive

Pancreatic lesions in the von Hippel-Lindau syndrome. Gastroenterology 1991;101:465-471.

Two Childhood Pheochromocytoma Cases due to von Hippel-Lindau Disease, One Associated with Pancreatic Neuroendocrine Tumor: A Very Rare Manifestation

Once the diagnosis is made, it is imperative to exclude the possibility of a neoplasm arising in the setting of Von Hippel-Lindau syndrome. In the current case, an extensive workup was negative.

Sporadic Retroperitoneal Hemangioblastoma: Report of a Case and Review of the Literature

The most common hereditary renal cancer syndromes are associated with hereditary leiomyomatosis and renal cell carcinoma (HLRCC), Von Hippel-Lindau syndrome (VHL), and hereditary papillary renal carcinoma (HPRC).

Bilateral renal tumour as indicator for Birt-Hogg-Dube syndrome

The sixteen lines of stem cells on the NIH Registry carry genes for various hereditary disorders including Duchenne muscular dystrophy, Huntington's disease, cystic fibrosis, and rarer conditions such as Von Hippel-Lindau Syndrome, Wiskott-Aldrich syndrome, spinal muscular atrophy, myotonic dystrophy and neurofibromatosis.

Disease-specific hESCs placed on NIH Registry

* Inherited diseases associated with secondary hypertension include polycystic kidney disease, multiple endocrine neoplasia type 2 (MEN2), and von Hippel-Lindau syndrome. (12,13) All are inherited in an autosomal dominant pattern.

Next steps when BP won't come down: a poor response to therapy should trigger a search for a secondary cause of hypertension. Use this review as your guide

Additionally, other pancreatic lesions are commonly seen, such as multiple pancreatic cysts in patients with Von Hippel-Lindau syndrome (Figures 12 and 13).

Islet-cell tumors of the pancreas: spectrum of MDCT findings. A pictorial essay

Hearing preservation surgery for small endolymphatic sac tumors in patients with von Hippel-Lindau syndrome. Otol Neurotol 2002;23:378-87.

Obstructing lesions of the endolymphatic sac and duct mimicking Meniere's disease

VHL (Von Hippel-Lindau Syndrome) Family Alliance 171 Clinton Rd.

Annual directory of national organizations: 1993-1994

Some GI tract and pancreatic NETs are associated with hereditary syndromes, including MEN1, von Hippel-Lindau syndrome, neurofibromatosis 1, and tuberous sclerosis.

Recent Updates on Neuroendocrine Tumors From the Gastrointestinal and Pancreatobiliary Tracts

Pheochromocytomas in von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2 display distinct biochemical and clinical phenotypes.

Measurements of plasma methoxytyramine, normetanephrine, and metanephrine as discriminators of different hereditary forms of pheochromocytoma