Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Internal organ function, biochemical, and immunological variables were assessed in 100 patients with progressive systemic sclerosis (PSS), 37 with diffuse scleroderma and 63 with the CREST syndrome. The frequency of oesophageal symptoms and dysmotility did not differ between the two subsets, but patients with diffuse scleroderma had more pronounced motility disturbances. Calcinosis, Raynaud's phenomenon, and telangiectasia were equally frequent in the two groups. Vital capacity, total lung capacity, and static lung compliance were lower among patients with diffuse scleroderma (p less than 0.001), who also had a higher frequency of lung fibrosis (p less than 0.001) and cardiomegaly (p less than 0.01). Sixty eight per cent of the patients with diffuse scleroderma and 62% of those with the CREST syndrome had a positive antinuclear antibody test, which was associated with oesophageal hypomotility (p less than 0.001), fat malabsorption (p less than 0.01) and pulmonary dysfunction (p less than 0.05). Thus, the severity of organ manifestations varied not only with the form of PSS, but also with the absence/presence of antinuclear antibody. This study shows that the term 'CREST syndrome' could be replaced by 'limited cutaneous systemic sclerosis'.
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PMID:Organ manifestations in 100 patients with progressive systemic sclerosis: a comparison between the CREST syndrome and diffuse scleroderma. 278 82

Systemic scleroderma involves the gastro-intestinal tract in over 50 p. 100 of cases, the commonest target organs being the oesophagus, the small intestine, the colon and the stomach in that order. The G-I symptoms of this collagenosis are all related to disorder of motility secondary to disturbances of innervation and then to atrophy of the smooth muscle and fibrous infiltration. Oesophageal involvement results in gastro-oesophageal reflux and/or dysphagia due to the lack of tonicity of the lower oesophageal sphincter and a reduction of peristalsis. Disease of the small intestine may cause pseudo-intestinal obstruction or a secondary malabsorption syndrome due to abnormal intraluminal bacterial flora. Colonic involvement causes severe constipation with formation of faecoliths. Finally, scleroderma may be complicated by an acute abdominal syndrome: occlusion due to diffuse reduction in small intestinal motility, peritonitis due to perforation of the small intestine, ileo-colonic infarction, gastro-intestinal haemorrhage complicating telangiectasia. Treatment is purely symptomatic: classical remedies for gastro-oesophageal reflux and its complications, and antibiotics for malabsorption syndromes.
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PMID:[Digestive localizations of scleroderma]. 652 55