Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036421 (PSS)
 10,989 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

1. Cisapride is a novel prokinetic drug which facilitates or restores motility throughout the gastrointestinal tract. Its mechanism of action is thought to involve enhancement of acetylcholine release in the myenteric plexus of the gut. 2. The effect of intravenous cisapride 10 mg on gastro-oesophageal dysfunction was investigated in 20 patients with systemic sclerosis, using a double-blind, randomised, cross-over, placebo-controlled manometric study design. 3. The increase in lower oesophageal sphincter pressure was significantly higher after cisapride (mean +/- s.e. mean, 8.3 +/- 2.1 cm H2O) than after placebo (mean +/- s.e. mean. 0.1 +/- 0.3 cm H2O) (P less than 0.001). The increase in the number of fundic gastric contractions during the 30 min study period was significantly higher after cisapride (mean +/- s.e. mean, 7.7 +/- 2.3) than after placebo (mean +/- s.e. mean, 0.9 +/- 0.6) (P less than 0.01). 4. No serious clinical adverse effects were observed. 5. The study demonstrates that intravenous cisapride induces a significant increase in lower oesophageal sphincter pressure and in the number of fundic gastric contractions, which may be beneficial in the treatment of scleroderma gastro-oesophageal dysfunction. Further long-term studies of the effect of oral cisapride in patients with systemic sclerosis are warranted.
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PMID:The effect of cisapride on gastro-oesophageal dysfunction in systemic sclerosis: a controlled manometric study. 186 62

Scleroderma (progressive systemic sclerosis) is a systemic collagen disease in which the upper gut is frequently involved. In particular, most patient show altered esophageal motility, which frequently result in severe esophagitis, often resistant to therapeutic measures. The small bowel is also frequently involved by the disease, especially in the late stage of scleroderma. Small bowel alterations are sometimes clinically silent, but can also be the origin of malabsorption syndrome, small intestine perforation, pneumatosis cystoides or chronic intestinal pseudo-obstruction. The occurrence of an altered gastrointestinal motility in scleroderma can be detected by means of manometric techniques; their use in the wide area of collagenopathies may help understanding the pathophysiology of the altered gastrointestinal function frequently existing in these diseases.
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PMID:[Changes in upper gastrointestinal motility during scleroderma]. 207 74

A series of 21 subjects (2 males and 19 females) affected with systemic sclerosis, was examined by small bowel (oral and intubation methods) and colon enema. The underlying process responsible for abnormalities in the small bowel and colon in systemic sclerosis is a variable and pacthy destruction of the muscularis propria, that produces the structural and functional changes detected on X-ray. Pathologic condition is the same affecting the esophagus. The scout film of the abdomen often reveals colonic distension and fecal impaction, so that it may be quite difficult to prepare adequately the patients for a barium enema. Peristalsis may be virtually absent in short segments, and transit time may be several time longer than that in normal patients. For these reasons, intestinal pseudo-obstruction may appear in systemic sclerosis. The observed radiographic changes are: 1) in the small bowel: a) dilatation of the gut, especially in its proximal portions (duodenum and jejunum), in which the valvulae conniventes are straightened, normal or thinned; b) presence of diverticula, 2-4 cm in diameter, with hemispherical shape without the neck-like opening into the bowel lumen; 2) in the colon, the characteristic finding is an increase in size of individual haustra, forming sacculations or pseudo-diverticula, usually on the antemesenteric border of the transverse colon, better demonstrated on post-evacuation film. Moreover, loss of colonic haustration is also observed associated to colonic elongation and dilatation.
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PMID:[Changes in the small and large intestines in systemic sclerosis]. 228 Nov 70

Two hypotheses were tested: first, the postcibal motility disorders occur in both the antrum and small bowel of systemic sclerosis patients with gastrointestinal symptoms; second, that dysmotility may result from a neuropathic stage in this disease. Upper gut perfusion manometry (3 h fasting, 2 h fed) was performed and compared with data from similarly studied healthy laboratory controls (n = 15). Only 1 of 8 scleroderma patients had an interdigestive motor complex during the 3-h fasting period [0.1 +/- 0.1 (SEM) for systemic sclerosis vs. 1.4 +/- 0.16 (SEM) for controls, p less than 0.05]; 2 other patients had fasting duodenal incoordinated bursts of phasic pressure activity in the duodenum or proximal jejunum. Distal antral motility index [MI = In (sum of amplitude x number of contractions + 1)] was lower (12.4 +/- 0.5, p less than 0.01) than that in controls (14.4 +/- 0.14); both the amplitude and frequency of antral pressure activity were lower (p less than 0.05) in systemic sclerosis. The intestinal fed pattern was characterized by reduced amplitude and frequency of contractions in 6 patients; 2 patients had excessive phasic or tonic small bowel pressure activity, or both. Thus, antral hypomotility is present in most symptomatic scleroderma patients; proximal small bowel postcibal motility is characteristically reduced; a minority of such patients have incoordianted fasting or postcibal hypermotility suggestive of a neuropathy. Manometry identifies the type and region of dysmotility in systemic sclerosis; our results suggest that there are neuropathic and myopathic stages of the upper gut involvement in systemic sclerosis.
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PMID:Abnormal postcibal antral and small bowel motility due to neuropathy or myopathy in systemic sclerosis. 290 17

Liquid esophageal transit and gastric emptying, mouth-to-cecum transit, and whole gut transit of a solid-liquid meal were measured in 14 patients with PSS, 16 control subjects (esophageal transit), and 20 control subjects (gastrointestinal transit), respectively, by using scintigraphic techniques, the hydrogen breath test, and stool markers. In patients with PSS, the glucose hydrogen breath test for detection of small intestinal overgrowth was performed and various gastrointestinal symptoms were determined. Esophageal transit and gastric emptying were significantly prolonged in PSS patients with 11 of 14 PSS patients (79%) disclosing delayed esophageal transit and eight of 14 PSS patients (57%) disclosing delayed gastric emptying. All PSS patients with prolonged gastric emptying also had delayed esophageal transit and there was a significant positive correlation between esophageal transit and gastric emptying (r = 0.696, P < 0.01). No significant differences between PSS patients and controls were detected concerning mouth-to-cecum transit and whole gut transit, but abnormally delayed mouth-to-cecum transit was found in four of 10 PSS patients (40%) and abnormally prolonged whole gut transit was detected in three of 13 PSS patients (23%). Small bacterial overgrowth was diagnosed in three of 14 PSS patients (21%). Delayed esophageal transit and gastric emptying were associated with dysphagia, retrosternal pain, and epigastric fullness, while prolonged whole gut transit was associated with constipation. It is concluded that delayed gastric emptying is frequently associated with esophageal transit disorders in PSS patients and may be one important factor for the development of gastroesophageal reflux disease in these patients.
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PMID:Gastrointestinal transit through esophagus, stomach, small and large intestine in patients with progressive systemic sclerosis. 792 44

Two female patients aged 62 and 44 years with progressive systemic sclerosis and passive faecal incontinence are described. Both had the typical gut motility disorders of dysphagia, heartburn, and constipation. Anorectal physiology tests showed a low resting pressure in both and an absent rectoanal inhibitory reflex in one. In both patients anal endosonography showed a thin internal anal sphincter with changed reflectivity suggestive of fibrosis. In both patients anorectal sensation and pudendal nerve function were normal. Histological examination of the rectum in one patient showed collagenous replacement of the rectal muscularis propria with prominent atrophy of the musculature. This study suggests that the internal sphincter may be selectively affected by progressive systemic sclerosis, which may lead to passive faecal incontinence.
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PMID:Progressive systemic sclerosis of the internal anal sphincter leading to passive faecal incontinence. 802 Aug 21

Gastric emptying, antral motility, and gallbladder emptying after a liquid fatty meal were studied by ultrasound in 25 patients with progressive systemic sclerosis and in 25 sex- and age-matched controls. In patients with systemic sclerosis, the possible role of autonomic dysfunction was evaluated by four noninvasive cardiovascular reflex tests. Despite a significant delay of gastric emptying and a significant postprandial antral hypomotility in the patients with systemic sclerosis, the fat-induced gallbladder emptying was only slightly reduced, reaching no significant level when compared to the controls. The prolongation of gastric emptying correlated significantly with the duration of the disease. Although 36% of the patients in the systemic sclerosis group exhibited signs of autonomic cardiac dysfunction, there was no evidence of an association between these signs and gastric motor dysfunction. In conclusion motility disorders of the gallbladder seem to play a minor role in the upper gut involvement of systemic sclerosis, whereas motility disorders of the stomach are frequent and can be easily recognized noninvasively by real-time ultrasound.
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PMID:Gastrobiliary motility after liquid fatty meal in progressive systemic sclerosis. A sonographic study. 813 94

Intestinal pseudo-obstruction is a rare and heterogeneous syndrome caused by severe disorders of gastrointestinal motility. It affects the entire gastrointestinal tract or only segments, giving rise to symptoms and physical signs of a mechanical obstruction of the gut despite negative results of all imaging procedures. The disease may occur in an acute or chronic form. The acute and some of the chronic forms develop as complication of other gastrointestinal or extragastrointestinal diseases, e.g. pancreatitis or systemic sclerosis. The primary forms of chronic intestinal pseudo-obstruction are most often caused by genetic neuromuscular disorders of the gastrointestinal tract, e.g. familial visceral neuropathies. The diagnosis of intestinal pseudo-obstruction is based on the exclusion of a mechanical obstruction of the gut by fluoroscopy and endoscopy. Manometric studies may disclose the underlying disorder of gastrointestinal motility. In a few patients, results of all imaging procedures as well as motility studies are inconclusive, and laparatomy (with full thickness biopsy of the gut wall) has to be performed to exclude mechanical obstruction of the gut. Acute intestinal pseudo-obstruction is treated by elimination of the underlying intestinal or extraintestinal disease. In case of extensive colonic dilatation with imminent colonic perforation endoscopic decompression should be evaluated. Treatment of chronic pseudo-obstruction aims to correct the underlying motility disorder. Usually, restoration of normal gastrointestinal motility is attempted by prokinetic drugs, but often their effect is limited. Surgery may be helpful in the few patients in whom the disease is confined to small segments of the gut, leaving all other parts unaffected. Some patients with otherwise intractable disease may need long-term parenteral nutrition.
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PMID:[Intestinal pseudo-obstruction]. 816 Jan 67

Octreotide inhibits intestinal motility and secretions of the gastro-intestinal tract and pancreas and mediators of diarrhoea and so is very useful in managing refractory diarrhoea. It is safe and effective in 75-80% of the 10-20% of cancer chemotherapy patients who develop severe diarrhoea, and is useful in the management of persistent diarrhoea associated with neuroendocrine tumours, particularly VIPoma and carcinoid tumours, congenital microvillus atrophy, some patients with the short bowel syndrome (giving them a reduced need for intravenous fluids), and AIDS-related diarrhoea that does not respond to antibiotics or conventional anti-diarrhoeal drugs. Some studies suggest a 50% effectiveness in graft-versus-host disease. Preliminary studies suggest that octreotide is also of value in persistent diarrhoea caused by neuromuscular disorders of the gut, particularly diabetes mellitus and systemic sclerosis, suggesting that it may have wider application in the future. Octreotide may prove useful as a tool for studying the pathogenesis of diarrhoea of diverse aetiologies, particularly those associated with disturbances of intestinal motility, such as irritable bowel syndrome.
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PMID:The role of somatostatin analogues in the treatment of refractory diarrhoea. 881 86

We reported a typical case of watermelon stomach which occurred during the course of a limited cutaneous systemic sclerosis. Watermelon stomach is an important source of upper gastrointestinal bleeding which requires endoscopic treatment. Such an association has already been described and we suggest that watermelon stomach could be an unrecognized localization of scleroderma stomach involvement. Because gut involvement may precede skin manifestations. A search of progressive systemic sclerosis should be done with clinical examination, antinuclear antibodies research (especially anticentromere antibodies) and nailfold capillaroscopy when a such endoscopic appearance is noted.
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PMID:[Should watermelon stomach be considered to be an unrecognized localization of scleroderma?]. 896 50


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