| 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) |
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| 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) |
| diffuse interstitial fibrosis | Progressive fibrous changes and scarring within the lung tissue, eventually interfering with normal gas exchange. (27 Sep 1997) |
| diffuse interstitial pulmonary fibrosis | This represents a group of pulmonary disorders (lower respiratory tract) that leads to a functional loss in the alveolar air sacs and a compromise in the diffusion of oxygen from air to blood. There is widespread inflammation and scar tissue (fibrosis) formation within the lung. The causes are varied and include: inorganic and organic dusts, gases, fumes, vapors, infections, radiation, medications, coal dust, silicosis and byssinosis. When the cause is not identifiable it is referred to as idiopathic diffuse interstitial pulmonary fibrosis (27 Sep 1997) |
| idiopathic interstitial fibrosis | A progressive inflammatory condition starting with diffuse alveolar damage and resulting in fibrosis and honeycombing over a variable time period; also a common feature of collagen-vascular diseases. Synonym: fibrosing alveolitis, Hamman-Rich syndrome, idiopathic interstitial 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) |
| interstitial pulmonary fibrosis | <radiology> Idiopathic, idiopathic pulmonary fibrosis (AKA: usual interstitial pneumonitis), Hamman-Rich syndrome (rapidly progressive), drug-related, collagen-vascular disease, rheumatoid lung disease, scleroderma (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) |
| Aarskog-Scott syndrome | A syndrome of ocular hypertelorism, anteverted nostrils, broad upper lip, saddle-bag scrotum, and laxity of ligaments resulting in genu recurvatum, flat feet, and hyperextensible fingers; X-linked and autosomal dominant forms. Synonym: Aarskog-Scott syndrome. (05 Mar 2000) |
| Aarskog syndrome | <syndrome> Grier et al. (1983) reported father and 2 sons with typical Aarskog syndrome, including short stature, hypertelorism, and shawl scrotum. They tabulated the findings in 82 previous cases. X-linked recessive inheritance has been repeatedly suggested. The family reported by Welch (1974) had affected males in 3 consecutive generations. Thus, there is either genetic heterogeneity or this is an autosomal dominant with strong sex-influence and possibly ascertainment bias resulting from use of the shawl scrotum as a main criterion. Stretchable skin was present in the cases of Grier et al. (1983). Teebi et al. (1993) reported the case of an affected mother and 4 sons (including a pair of monozygotic twins) by 2 different husbands. They suggested that the manifestations were as severe in the mother as in the sons and that this suggested autosomal dominant inheritance. Actually, the mother seemed less severely affected, compatible with X-linked inheritance. Clinical signs: Mild to moderate short stature,normocephaly, Widow's peak hair, maxillary hypoplasia, broad nasal bridge, anteverted nostrils, long philtrum, broad upper lip, curved linear dimple below the lower lip, hypertelorism, ptosis, down-slanted palpebral fissures, ophthalmoplegia, strabismus, hyperopic astigmatism, large cornea, floppy ears, lop-ears,cleft lip/palate, shawl scrotum, saddle-bag scrotum, cryptorchidism, brachydactyly, digital contractures, clinodactyly, mild syndactyly, transverse palmar crease, lymphoedema of the feet, ligamentous laxity, osteochondritis dissecans, proximal finger joint hyperextensibility, flexed distal finger joints, genu recurvatum, flat feet, stretchable skin, cervical spine hypermobility, odontoid anomaly, macrocytic anaemia, hemochromatosis, hepatomegaly, portal cirrhosis, imperforate anus, rectoperineal fistula, interstitial pulmonary disease, sternal deformity. Inheritance: Sex-influenced autosomal dominant form, also X-linked form. (05 Aug 1998) |
| abdominal muscle deficiency syndrome | <syndrome> Congenital absence (partial or complete) of abdominal muscles, in which the outline of the intestines is visible through the protruding abdominal wall; in males, genitourinary anomalies (urinary tract dilation and cryptorchidism) are also found; genetics unclear. (05 Mar 2000) |