| cerebral amyloidosis | A condition where there is a deposition of amyloid (insoluble protein) in the walls of the arteries which supply the brain. This results in an increased risk of dementia and-or intracerebral haemorrhage. Cerebral amyloidosis or cerebral amyloid angiopathy, is a complication of primary amyloidosis. (27 Sep 1997) |
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| primary amyloidosis | <immunology, nephrology> A disease which is characterised by the deposition of the fibrous protein amyloid in one or more locations within the body. Amyloid deposition may occur in the kidney, brain, liver, heart, skin and lungs. A recognised complication is a restrictive cardiomyopathy. (05 Mar 1998) |
| secondary amyloidosis | Amyloidosis occurring in association with another chronic inflammatory disease; organs chiefly involved are the liver, spleen, and kidneys, and the adrenal glands less frequently. (05 Mar 2000) |
| senile amyloidosis | A common form of amyloidosis in very old people, usually mild and limited to the heart. See: amyloidosis of aging. (05 Mar 2000) |
| nodular amyloidosis | A localised form of amyloidosis in which amyloid occurs as masses or nodules beneath the skin or mucous membranes, e.g., in the larynx. Synonym: amyloid tumour, focal amyloidosis. (05 Mar 2000) |
| familial amyloidosis | <neurology> A disorder in which various peripheral nerves are infiltrated with amyloid and their functions disturbed, an abnormal prealbumin is also formed and is present in the blood; characteristically, it begins during mid-life and is found largely in persons of Portuguese descent. Other rare clinical types occur. Inheritance: autosomal dominant. Synonym: familial amyloidosis, hereditary amyloidosis. (05 Mar 2000) |
| focal amyloidosis | A localised form of amyloidosis in which amyloid occurs as masses or nodules beneath the skin or mucous membranes, e.g., in the larynx. Synonym: amyloid tumour, focal amyloidosis. (05 Mar 2000) |
| lichen amyloidosis | Localised cutaneous amyloidosis with pruritic brownish-red papules, most commonly on the lower legs, due to amyloid infiltration of the papillary dermis. Synonym: amyloidosis cutis, lichen amyloidosis. Origin: G. Leichen, lichen, a lichen-like eruption + eidos, resemblance (05 Mar 2000) |
| lichenoid amyloidosis | Localised cutaneous amyloidosis with pruritic brownish-red papules, most commonly on the lower legs, due to amyloid infiltration of the papillary dermis. Synonym: amyloidosis cutis, lichen amyloidosis. Origin: G. Leichen, lichen, a lichen-like eruption + eidos, resemblance (05 Mar 2000) |
| light chain-related amyloidosis | A form of primary amyloidosis in which the fibrillar amyloid deposits are derived from the amino terminal variable region of the light chains of immunoglobulin; seen in B-lymphocyte and plasma-cells dyscrasias. (05 Mar 2000) |
| Albright's hereditary osteodystrophy | An inherited form of hyperparathyroidism associated with ectopic calcification and ossification and skeletal defects, notably the small fourth metacarpals, but intelligence is normal. There are dominant, recessive and X-linked forms. See: pseudohypoparathyroidism. Synonym: Albright's syndrome. (05 Mar 2000) |
| angioedema, hereditary | A genetic form of angioedema. (Angioedema is also referred to as Quinke's disease.) Persons with it are born lacking an inhibitor protein (called C1 esterase inhibitor) that normally prevents activation of a cascade of proteins leading to the swelling of angioedema. Patients can develop recurrent attacks of swollen tissues, pain in the abdomen, and swelling of the voice box (larynx) which can compromise breathing. The diagnosis is suspected with a history of recurrent angioedema. It is confirmed by finding abnormally low levels of C1 esterase inhibitor in the blood. Treatment options include antihistamines and male steroids (androgens) that can also prevent the recurrent attacks. Also called hereditary angioneurotic oedema. (12 Dec 1998) |
| angioneurotic oedema, hereditary | A genetic form of angioedema. (Angioedema is also referred to as Quinke's disease.) Persons with it are born lacking an inhibitor protein (called C1 esterase inhibitor) that normally prevents activation of a cascade of proteins leading to the swelling of angioedema. Patients can develop recurrent attacks of swollen tissues, pain in the abdomen, and swelling of the voice box (larynx) which can compromise breathing. The diagnosis is suspected with a history of recurrent angioedema. It is confirmed by finding abnormally low levels of C1 esterase inhibitor in the blood. Treatment options include antihistamines and male steroids (androgens) that can also prevent the recurrent attacks. Also called hereditary angioedema. (12 Dec 1998) |
| canine hereditary blindness | An autosomal dominant condition seen in dogs of the collie and several other breeds. (05 Mar 2000) |
| colourectal neoplasms, hereditary nonpolyposis | A syndrome characterised by autosomal dominant inheritance, a low mean age (41 years) for occurrence of colon cancer, and a marked increase in the proportion of tumours in the proximal colon. (12 Dec 1998) |