| Nussbaum, Johann von | <person> German surgeon, 1829-1890. See: Nussbaum's bracelet. (05 Mar 2000) |
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| 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) |
| disease, von recklinghausen's | Hereditary disorder characterised by cafe-au-lait (coffee-with-milk spots on the skin and a tendency to develop nerve tumours) also known as neurofibromatosis. (12 Dec 1998) |
| Dogiel, Jan von | <person> Russian anatomist and physiologist, 1830-1905. See: Dogiel's cells. (05 Mar 2000) |
| Drigalski, Wilhelm von | <person> German bacteriologist, 1871-1950. See: Drigalski-Conradi agar, Conradi-Drigalski agar. (05 Mar 2000) |
| imbrication lines of von Ebner | Incremental line's in the dentin of the tooth that reflect variations in mineralization during dentin formation; the distance between the line's corresponds to the daily rate of dentin formation. Synonym: incremental lines of von Ebner. (05 Mar 2000) |
| incremental lines of von Ebner | Incremental line's in the dentin of the tooth that reflect variations in mineralization during dentin formation; the distance between the line's corresponds to the daily rate of dentin formation. Synonym: incremental lines of von Ebner. (05 Mar 2000) |
| Eicken, Karl von | <person> German laryngologist, 1873-1960. See: Eicken's method. (05 Mar 2000) |
| Esmarch, Johann von | <person> German surgeon, 1823-1908. See: Esmarch tourniquet. (05 Mar 2000) |
| Jaeger, Eduard Ritter von Jaxthal | <person> Austrian ophthalmologist, 1818-1884. See: Jaeger's test types. (05 Mar 2000) |
| Zambusch, Leo von | <person> 20th century German physician. See: generalised pustular psoriasis of Zambusch. (05 Mar 2000) |
| Fehling, Hermann von | <person> German chemist, 1812-1885. See: Fehling's reagent, Fehling's solution. (05 Mar 2000) |
| Kolliker, Rudolph von | <person> Swiss histologist, 1817-1905. See: Kolliker's layer, Kolliker's reticulum. (05 Mar 2000) |
| Korff, Karl von | <person> 20th century German anatomist and histologist. See: Korff's fibres. (05 Mar 2000) |
| Kupffer, Karl Wilhelm von | <person> B. Munich, Nov. 14th, 1829. Was an Anatomist and Embryologist. Was Professor of Anatomy in Kiel (1867), in Konigsberg (1875) and in Munich (1880). D. Munich, 1902. Kupffer's Cells - "stellate cells" in the lining of blood channels in the liver. Lived: 1829-1902. (05 Dec 1998) |