| VHL | Von Hippel-Lindau Syndrome = Cerebelloretinal Hemangioblastomatosis |
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| VHL | von Hippel-Lindau [syndrome] |
| HLD | hepatolenticular degeneration; herniated lumbar disk; Hippel-Lindau disease; hypersensitivity lung d... |
| HLS | Health Learning System; Hippel-Lindau syndrome |
| NF | Neuro-Fibromatosis = Von Recklinghausen's Disease NF 1; Neuro-Fibroma... |
| VHL | Von Hippel Lindau |
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| VHLD | Von Hippel Lindau disease |
| VHL | Von Hippel-Lindau syndrome |
| VHL | Van Hippel-Lindau disease |
| AVWS | Acquired von Willebrand syndrome |
| von Hippel, Eugen | <person> German ophthalmologist, 1867-1939. See: von Hippel-Lindau syndrome. (05 Mar 2000) |
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| von hippel-lindau disease | <disease> A congenital disease characterised by the development of blood vesse ltumours in the retina of the eye and in the brain, lesions and cysts canalso develop in the spina lcord, pancreas, kidneys, and other organs. (09 Oct 1997) |
| von hippel-lindau syndrome | <radiology> Retinocerebellar angiomatosis, phakomatosis, autosomal dominant (variable penetrance), haemangioblastoma: most frequent cause of death, cerebellar (most common), also medullary and spinal, retinal angiomatosis (45%), renal cell carcinoma: 2nd most common cause of death, pheochromocytoma (17%), cortical renal cysts (75%), cysts in virtually any organ, renal/liver haemangioma/adenoma, pancreatic cystic neoplasms, isleT-cell tumours, paraganglioma (12 Dec 1998) |
| syndrome, von hippel-lindau | The cardinal features of von hippel-lindau (vhl) syndrome are benign blood-vessel tumours that most typically affect the eye and the brain. The eye tumours are termed angiomata and are in the retina. The brain tumours are termed haemangioblastoma and are in the cerebellum. Vhl is complex. There can also be blood-vessel tumours (haemangiomata) in the spinal cord, adrenal glands, liver, and lungs. Pheochromocytoma (a benign tumour of adrenal-like tissue) occurs in some patients. The combination of high blood pressure (hypertension) with angioma may cause bleeding under the skull (subarachnoid haemorrhage). Kidney tumours (like hypernephromas) may be malignant and metastasize. An abnormal elevation of red blood cells (polycythemia) can be due to the haemangioblastoma of the cerebellum or the hypernephroma. Multiple cysts can occur in the pancreas and kidneys. Patients with kidney problems or pancreatic cysts do not have pheochromocytoma, and visa versa. Lab findings in vhl may include high calcium (hypercalcaemia) and low potassium (hypokalaemia) occurring with the pheochromocytoma. Vhl is inherited as an autosomal dominant trait. The gene on one of the non-sex chromosomes is dominant over the normal gene with which it is paired so that one vhl gene is sufficient to cause the vhl syndrome. If a person has vhl, the chance for each of their children to receive the vhl gene is one-half (50%). The vhl gene has been mapped to chromosome 3 (the 3rd volume in the book of life) in region 3p26-p25. The vhl gene has the characteristics of a tumour-suppressor gene. The person with vhl inherits one inactive copy of the vhl gene (a germline mutation) from one of their parents. But the normal gene with which it is paired is still enough to suppress the formation of a tumour. Then, in one cell in the vhl patient's body, another mutation (a somatic mutation) occurs, inactivating the vhl gene. Thus, both copies of the vhl gene are inactivated and a tumour arises in the vhl patient. The syndrome is named for the german ophthalmologist eugen von hippel who described the charcteristic eye blood-vessel tumours in 1904 and the swedish pathologist arvid lindau who recognised the association between the eye tumours and the blood-vessel tumours of the cerebellum and other parts of the central nervous system in 1926-7. (12 Dec 1998) |
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| lindau-von hippel syndrome | <syndrome> The cardinal features of what is more commonly called von hippel-lindau (vhl) syndrome are benign blood-vessel tumours that most typically affect the eye and the brain. The eye tumours are termed angiomata and are in the retina. The brain tumours are termed haemangioblastoma and are in the cerebellum. Vhl is complex. There can also be blood-vessel tumours (haemangiomata) in the spinal cord, adrenal glands, liver, and lungs. Pheochromocytoma (a benign tumour of adrenal-like tissue) occurs in some patients. The combination of high blood pressure (hypertension) with angioma may cause bleeding under the skull (subarachnoid haemorrhage). Kidney tumours (like hypernephromas) may be malignant and metastasize. An abnormal elevation of red blood cells (polycythemia) can be due to the haemangioblastoma of the cerebellum or the hypernephroma. Multiple cysts can occur in the pancreas and kidneys. Patients with kidney problems or pancreatic cysts do not have pheochromocytoma, and visa versa. Lab findings in vhl may include high calcium (hypercalcaemia) and low potassium (hypokalaemia) occurring with the pheochromocytoma. Vhl is inherited as an autosomal dominant trait. The gene on one of the non-sex chromosomes is dominant over the normal gene with which it is paired so that one vhl gene is sufficient to cause the vhl syndrome. If a person has vhl, the chance for each of their children to receive the vhl gene is one-half (50%). The vhl gene has been mapped to chromosome 3 (the 3rd volume in the book of life) in region 3p26-p25. The vhl gene has the characteristics of a tumour-suppressor gene. The person with vhl inherits one inactive copy of the vhl gene (a germline mutation) from one of their parents. But the normal gene with which it is paired is still enough to suppress the formation of a tumour. Then, in one cell in the vhl patient's body, another mutation (a somatic mutation) occurs, inactivating the other vhl gene. Thus, both copies of the vhl gene are inactivated and a tumour arises in the vhl patient. The syndrome is named for the german ophthalmologist eugen von hippel who described the charcteristic eye blood-vessel tumours in 1904 and the swedish pathologist arvid lindau who recognised the association between the eye tumours and the blood-vessel tumours of the cerebellum and other parts of the central nervous system in 1926-7. (12 Dec 1998) |
| Hippel | Eugen von. See: von Hippel. (05 Mar 2000) |
| hippel-lindau disease | A syndrome transmitted as an autosomal dominant trait and characterised chiefly by angiomata of the retina and haemangioblastoma of the cerebellum and walls of the fourth ventricle. Ocular complications are often present, as are haemangiomas of the spinal cord, face, and other sites. Symptoms may not be apparent until the third decade in life. (12 Dec 1998) |
| Alder, Albert von | <person> See: Alder's anomaly, Alder bodies. (05 Mar 2000) |
| Ammon, Friedrich von | <person> German ophthalmologist and pathologist, 1799-1861. See: Ammon's fissure, Ammon's prominence. (05 Mar 2000) |
| Andernach, Johann von | <person> German physician, 1505-1574. See: Andernach's ossicles. (05 Mar 2000) |
| Baer, Karl von | <person> German-Russian embryologist, 1792-1876. See: Baer's law, Baer's vesicle. (05 Mar 2000) |
| Baeyer, Johann von | <person> German chemist and Nobel laureate, 1835-1917. See: Baeyer's theory. (05 Mar 2000) |
| Bamberger, Heinrich von | <person> Austrian physician, 1822-1888. See: Bamberger's albuminuria, Bamberger's disease, Bamberger's sign. (05 Mar 2000) |
| Basedow, Karl von | <person> German physician, 1799-1854. See: Basedow's disease, Basedow's pseudoparaplegia, Jod-Basedow phenomenon, Basedow's goiter. (05 Mar 2000) |
| Baumgarten, P Clemons von | <person> German pathologist, 1848-1928. See: Baumgarten's veins, Cruveilhier-Baumgarten disease, Cruveilhier-Baumgarten murmur, Cruveilhier-Baumgarten sign, Cruveilhier-Baumgarten syndrome. (05 Mar 2000) |
| Bechterew, Vladimir von | <person> Russian neurologist, 1857-1927. See: Bechterew's band, Bechterew's disease, layer of Bechterew, Bechterew's nucleus, Bechterew's sign, line of Bechterew, band of Kaes-Bechterew, Bechterew-Mendel reflex, Mendel-Bechterew reflex. (05 Mar 2000) |
| Bezold, Albert von | <person> German physiologist, 1836-1868. See: Bezold's ganglion, Bezold-Jarisch reflex. (05 Mar 2000) |
| Biesiadecki, Alfred von | <person> Polish physician, 1839-1888. See: Biesiadecki's fossa. (05 Mar 2000) |
Synonyms : VHL Tumor Suppressor Protein, Von Hippel Lindau Tumor Suppressor Protein
| von Hippel-Lindau disease |
An autosomally dominantly inherited rare disease, charactererized by retinal and central nervous system hemangioblastomas, renal cell carcinomas, and pheochromocytomas. Several types of pancreatic lesions have been found in this disease, including pancreatic cysts and cystadenomas, adenocarcinomas and neuroendocrine tumors.
출처: www.gastrolab.net/dictev.htm
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| von Hippel-Lindau syndrome |
A rare inherited disorder in which blood vessels grow abnormally in the eyes, brain, spinal cord, adrenal glands, or other parts of the body. People with von Hippel-Lindau syndrome have a higher risk of developing some types of cancer.
출처: www.seniormag.com/conditions/cancer/cancerglossary...
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| von Hippel-Lindau disease |
A rare genetic disease in which benign and/or malignant tumors and cysts grow in areas of the body that have a rich blood supply.
출처: www.riainvision.com/invision/patientinfo/resources...
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| von Hippel |
see Hippel.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| von Hippel's d. |
hemangiomatosis confined principally to the retina; when associated with hemangioblastoma of the cerebellum, it is known as von Hippel-Lindau d. Called also retinal hemangioblastoma and Hippel's d.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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