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

Hematological abnormalities in scleroderma indicate a specific complication of the disease itself, or an associated illness. Among 180 patients with scleroderma, anemia was detected in 25% and was attributed to chronic inflammatory disease (usually an overlap syndrome), bleeding mucosal telangiectases as part of the CREST syndrome, intestinal malabsorption, and microangiopathic hemolysis. Leukocytosis, present in 14%, was correlated with active myopathy and/or advanced visceral involvement while leukopenia was suggestive of a crossover with systemic lupus erythematosus. Thrombocytopenia was often a manifestation of microangiopathy, and thrombocytosis was associated with an arteritis or a tumor syndrome.
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PMID:Hematological abnormalities in scleroderma. A study of 180 cases. 677 74

Unclear perianal bleeding may cause diagnostic and therapeutic difficulty, particularly when the bleeding source cannot be detected. In this case record we report on a 64-year-old woman with systemic sclerosis and incomplete CRES(T) syndrome diagnosed more than 10 years ago with no detectable teleangiectasia/angiodysplasia at that time. During the course of the disease the initially incomplete CRES(T) syndrome developed into a complete CREST syndrome with repeated bleeding from teleangiectatic/angiodysplastic lesions of the rectum, stomach/duodenum. In addition, chronic diarrhoea with malabsorption, bacterial overgrowth and severe anal incontinence were present which all were seen as intestinal manifestations of the existing underlying disease. A complete motoric and sensoric insufficiency of the anal sphincter was found manometrically, anal endosonography with elastography revealed changes compatible with sclerosis. In the absence of a causal therapy symptomatic treatment strategies are described and discussed on the basis of existing pathophysiologic knowledge.
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PMID:[A 64-year-old woman with perianal bleeding, chronic diarrhea and severe fecal incontinence]. 1935 63