Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Scleroderma or Systemic Sclerosis (SSC) is a disorder characterized by fibrosis of the skin and multiple internal organs. The pathological lesion is a triad of small artery intimal proliferation, medial thinning and adventitial scarring. Autoamputation of fingers and toes is often seen, but only a few cases of limb amputation in scleroderma patients have been reported. The Pittsburgh Scleroderma databank includes 1,030 patients with SSC. Among these were seven patients who sustained lower limb amputation. There were four patients with the CREST variant of SSC, two with diffuse scleroderma, and one who had SSC/rheumatoid arthritis/polymyositis overlap who sustained limb amputation. Of the seven, three were male and five had a significant smoking history. Ages ranged from 46 to 71 years. All patients underwent amputation for nonhealing ulcerations. No problems with postoperative wound healing were seen. Pathologic changes typical of SSC in addition to atherosclerotic peripheral vascular disease were described in one case. Three patients were successfully fitted with prostheses and became independent ambulators. Four patients could not be fitted with prostheses. No skin problems were reported related to prosthetic use. Our review demonstrates that SSC patients who undergo amputation can become successful prosthetic users and should be considered for prosthetic prescription.
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PMID:Lower extremity amputation in scleroderma. 151 89

Scleroderma is a rare disease in children: the clinical presentation in childhood is even more varied than in adult life. It is characterized by 'hard skin' with cutaneous features including hypo- and hyperpigmentation, thickening or thinning and loss of elasticity. It ranges from circumscribed and self-limiting pigmentary disorders to disabling and disfiguring involvement of an extremity and a rapidly fatal outcome. Scleroderma must be differentiated from many scleroderma-like conditions. Therapeutic problems are also discussed.
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PMID:[Localized scleroderma in childhood]. 318 14

A case of progressive systemic sclerosis (PSS) is described in which painful bone resorption of both upper arms occurred. Computerized tomographic examination revealed cortical thinning with periosteal new bone formation, and pathologic examination revealed only a healing, fibrotic lesion. The cause of this rare occurrence in PSS remains unknown.
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PMID:Localized bone resorption in systemic sclerosis. 319 3

Forty patients with neuromuscular disorders of the gastrointestinal tract were evaluated histologically and radiologically. Eighteen patients with progressive systemic sclerosis had predominant circular muscle thinning and fibrosis. Visceral myopathy (11 patients) was characterized by vacuolar degeneration of the smooth muscle cells with thinning and fibrosis typically affecting the longitudinal layers. Visceral neuropathy (five patients) had degeneration of myenteric plexus neurons with various patients having intranuclear inclusions, Schwann cell proliferation, or inflammatory cell infiltration. Radiologically, these syndromes had diffuse abnormalities of gastrointestinal motor function manifested by small and large intestinal dilatation, esophageal hypomotility (progressive systemic sclerosis and visceral myopathy), or disordered hypercontractility (visceral neuropathy). Marked duodenal enlargement typified visceral myopathy, and although all types may have dilated small intestine, only progressive systemic sclerosis has packing of valvulae. Colonic sacculations were found in progressive systemic sclerosis, lack of haustrations and increased colonic caliber in visceral myopathy, and hypercontractility in visceral neuropathy. Complete barium contrast examination will assist in differentiation of true obstruction from pseudoobstruction, will define the diffuse nature of the syndrome, and will help establish an accurate diagnosis by identifying features specific for these entities.
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PMID:Radiologic and histologic differentiation of neuromuscular disorders of the gastrointestinal tract: visceral myopathies, visceral neuropathies, and progressive systemic sclerosis. 633 63

Microangiopathy is an essential clinicopathological feature of systemic scleroderma, comprising capillary thinning, thickening of the basement membrane and abnormalities of the endothelium which has an abnormally high uptake of radioactive thymidine. The identification of a circulating cytotoxic factor for endothelial cells suggests that the microangiopathy may play a role in the physiopathogenesis of the disease. In practice, microangiopathy can be fully assessed by capillaroscopy in patients with systemic scleroderma.
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PMID:[Sclerodermal microangiopathy: a cause or a consequence?]. 652 53

A prospective study in 30 patients with systemic sclerosis included phonocardiographic (PCG) recordings in all cases and an echocardiogram (ECHO) in 18 patients. A specific diagnosis of myocardial fibrosis was established in 2 cases, based on anatomical criteria, and was suspected in 5 patients from combined clinical, electrical, and radiological findings. Ventricular diameters, the slope of mitral valve closure, and systolic parameters of ventricular function (haemodynamic quotient, left ventricle ejection fraction, and mean rate of circumferential shortening) were rarely abnormal, and did not correlate with possible myocardial fibrosis. Diastolic pick-up (PCG) was abnormal in 30 p. cent of the cases but this is not specific to myocardial fibrosis. Total myocardial relaxation interval, as measured by PCG, was significantly increased to 146 +/- 23 ms (mean +/- one standard deviation) in relation to a control group (p less than 0.001). A relaxation interval (measured by PCG and/or ECHO) superior to 150 ms is suggestive of myocardial fibrosis. The rate of thinning of the left ventricular posterior wall was diminished in the 14 cases in which it was measured. These results are in favour of a myocardial relaxation disorder during scleroderma.
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PMID:[Sclerodermic cardiomyopathy : prospective study employing phonocardiography and echocardiography. Value of diastolic parameters of ventricular function (author's transl)]. 708 70

The disaccharide content of the chondroitinase-digestible glycosaminoglycans (GAGs) extracted from 6-mm skin punch biopsies from the atrophic and sclerotic skin of two patients with Werner's syndrome (WS) were determined using high-performance liquid chromatography after 1-phenyl-3-methyl-5-pyrazolone labelling. The total amount of main disaccharides was significantly decreased in the atrophic lesions of WS. In the atrophic forearm skin, the decrease in the main disaccharide unit of hyaluronic acid, delta Di-HA, and the increase in the ratio of the main disaccharide unit of dermatan sulphate, delta Di-4S, to delta Di-HA were significant vs. normal control (P < 0.01 and 0.05, respectively). The sclerotic skin showed an increase in delta Di-4S (DS) (P < 0.05) and a decrease in delta Di-HA (P < 0.02) compared with normal controls, as well as a significantly higher ratio of delta Di-4S (DS)/delta Di-HA compared with normal controls (P < 0.0002) and systemic sclerosis patients (SSc; P < 0.02). No other statistical difference was found in the amount of each main disaccharide unit between the sclerotic skin of WS and SSc. Histological examination revealed that the atrophic skin showed thinning of the dermis with a slight increase of fine collagen bundles, whereas the sclerotic skin demonstrated a thickened dermis with prominent deposition of fine collagen bundles in the deep dermis. In SSc, thickening of the whole dermis, composed of hyalinized or swollen collagen bundles, was found.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Disaccharide analysis of the skin glycosaminoglycans in patients with Werner's syndrome. 788 70

Myocardial thickness, left ventricular functions, and right heart overloading were investigated in 80 patients with systemic sclerosis by echocardiography. Based on the left ventricular wall thickness, three groups were formed. Fifteen patients with asymmetrical left ventricular wall hypertrophy (Group 1) showed shorter mean disease duration with marked diastolic and mild systolic left ventricular functional impairment. The 25 patients with wall thinning (Group 3) had a slight increase in left ventricular diameters, impaired systolic functions and longer disease duration. Group 2 with wall thickness exhibited mixed changes. Patients with increased wall thickness tended to have a shorter disease duration than patients with wall thinning. Patients with systemic sclerosis showed systolic and diastolic function impairment as compared to the 18 control healthy individuals. Pericardial disease was found in 23 cases (28.7%).
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PMID:Left ventricular wall thickness and disease duration in systemic sclerosis. 832 92

Membranous nephropathy, mesangial proliferative glomerulonephritis and renal amyloidosis are common renal pathology in RA patients. However, IgA nephropathy and diffuse thinning of glomerular basement membrane are described as common and characteristic renal lesions in Japanese RA patients. Glomerular filtration rate may decrease significantly in active lupus nephritis, but renal plasma flow does not change or even increase. These findings seem to be characteristic of SLE patients with active renal disorders. Therefore, filtration fraction may be a useful clinical parameter to evaluate SLE patients. Scleroderma renal crisis(SRC) has been believed to be the most serious renal disorder in systemic sclerosis (SSc). Recently, the presence of an antibody to RNA polymerase has been associated with a high prevalence of SRC.
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PMID:[Renal disorders in patients with collagen vascular diseases]. 1007 13

Systemic scleroderma is a progressive multi-system connective tissue disease. Ocular involvement includes keratoconjunctivitis sicca, progressive shallowing of conjunctival fornices, peripheral ulcerative keratitis and eyelid tightness. No association has been reported between scleroderma and pellucid marginal degeneration, which is a rare bilateral corneal ectasia. Pellucid marginal degeneration is characterised by non-inflammatory and progressive peripheral corneal thinning inferiorly, often with high against-the-rule astigmatism. We report a case of a 55-year-old woman with systemic scleroderma who presented with rapidly progressing against-the-rule astigmatism. The differential diagnosis of peripheral corneal thinning and the challenge of the surgical management of pellucid marginal degeneration are briefly discussed.
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PMID:Pellucid marginal degeneration and scleroderma. 1518 10


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