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cystic fibrosis transmembrane conductance regulator Gene believed to be defective in cystic fibrosis. Gene encodes a chloride channel, homologous to a family of proteins that actively transport small solutes in an ATP dependent manner (ABC transporters).
The regulator protein is a protein which is embedded in the cell membrane and acts as a channel for certain ions to be transported into or out of the cell. The disease cystic fibrosis is caused by a defect in the gene for this protein.
(09 Oct 1997)
progressive massive fibrosis <radiology> Progressive massive fibrosis, silicosis (properly conglomerate nodules, not progressive massive fibrosis), coal worker's pneumoconiosis, density (conglomerate mass) in upper lobe, retracts toward hilum, leaves emphysematous change (bullae) at lung periphery
(12 Dec 1998)
pulmonary fibrosis Chronic inflammation and progressive fibrosis of the pulmonary alveolar walls, with steadily progressive dyspnea, resulting finally in death from oxygen lack or right heart failure.
(12 Dec 1998)
subadventitial fibrosis Fibrosis in the outer media of arteries, usually the renal arteries of young women, where it causes segmental stenosis and hypertension; a variety of fibromuscular dysplasia.
Synonym: subadventitial fibrosis.
(05 Mar 2000)
nodular subepidermal fibrosis A slowly growing benign skin nodule consisting of poorly demarcated cellular fibrous tissue enclosing collapsed capillaries with scattered haemosiderin-pigmented and lipid macrophages. They are common, usually about 1 cm in diameter and occur in the dermis. Simple excision is always curative.
(12 Dec 1998)
Symmers' fibrosis A characteristic pipe-shaped fibrosis formed around hepatic portal veins in some cases of long-continued heavy infection with Schistosoma mansoni; thought to be induced by the presence of large numbers of schistosome eggs in the hepatic tissues.
Synonym: Symmers' clay pipestem fibrosis, Symmers' fibrosis.
(05 Mar 2000)
idiopathic pulmonary fibrosis <radiology> Aka: usual interstitial pneumonia (UIP), 5-6th decade; M:F = 1:1, clubbing of fingers (83%), lymphocytosis on lavage, increased occurence of bronchogenic CA, average survival of 4-6 years; 87% mortality CXR findings: occasional ground glass pattern in early stage of alveolitis, diffuse reticulations (60%) predominantly at bases, honeycombing, pleural effusion (4%); pleural thickening (6%), pneumothorax in 7% (late stage), progressive volume loss see: interstitial pulmonary fibrosis
(12 Dec 1998)
oral submucous fibrosis A precancerous condition of the oral mucosa and upper aerodigestive tract characteristically in a native of India.
(05 Mar 2000)
endocardial fibrosis Scarring or collaginosis of the endocardium.
Synonym: endocardial sclerosis.
(05 Mar 2000)
endomyocardial fibrosis A disease characterised by thickening of the endocardium, and frequently the inner third of the myocardium. The left ventricle is most frequently involved. Cardiomegaly and congestive heart failure may also be present.
(12 Dec 1998)
fibrosis The formation of fibrous tissue, fibroid or fibrous degeneration
(18 Nov 1997)
leptomeningeal fibrosis A fibrous reaction within the subarachnoid space; sometimes a sequel to infectious or chemical meningitis.
See: adhesive arachnoiditis.
(05 Mar 2000)
acute interstitial nephritis <nephrology> A relatively uncommon condition which is characterised by inflammation of the renal tubules, glomeruli and surrounding tissue. Interstitial nephritis is usually be temporary and often associated with the use of a particular medication. Chronic and progressive forms do exist.
Drugs that have been associated with interstitial nephritis include penicillins, ampicillin, sulpha drugs, non-steroidal anti-inflammatory agents, furosemide and thiazide diuretics. May result in mild kidney dysfunction or acute renal failure. Treatment may be with corticosteroids.
Incidence: 1 in 25,000.
(29 Dec 1997)
acute interstitial pneumonia A severe and usually fatal form of pneumonia occurring primarily in infants usually considered a form of hypersensitivity pneumonitis.
(05 Mar 2000)
acute interstitial pneumonitis Usually considered a form of hypersensitivity pneumonitis.
(05 Mar 2000)
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