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)

We report a case of systemic sclerosis (SSc) complicated with benign pneumoperitoneum without apparent pneumatosis cystoides intestinalis (PCI). A 43-year-old woman was admitted to our hospital because of prominent abdominal distension in April 1997. Raynaud's phenomenon has been detected since 1991. She was suffering from recurrent diarrhea, constipation, and subileus. The diagnosis of SSc was made in 1996 based on the sclerosis in her face, forearms, and chest, and hypomotility of the esophagus. On admission, she presented no signs of peritoneal irritation. The laboratory data revealed that white blood cell count was 7,400/mm3 and C-reactive protein was 0.1 mg/dl. Chest and abdominal roentgenograms showed massive free air under the diaphragm, dilatation of small and large intestine, and air-fluid level. PCI was not apparent. Pneumoperitoneum was improved after four weeks with intravenous hyperalimentation. But she presented recurrent severe diarrhea and high fever whenever she tried to take food orally. Klebsiella pneumoniae was proved in her jejunal juice by bacteriologic examination. Intravenous prostaglandin F2 alpha and oral fosfomycin calcium intake made her condition better. Benign pneumoperitoneum without PCI is rarely reported in the patients with SSc. In her condition, weakness of intestinal wall, hypomotility of intestine, unusual bacterial overgrowth, and elevated intraluminal pressure made intraluminal gas go through the wall of the fragile intestine of SSc. As operation of intestine of SSc usually cause miserable outcome, pneumoperitoneum accompanied with SSc even if PCI is apparent or not must be treated with conventional manner while there is no signs of peritoneal irritation.
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PMID:[Pneumoperitoneum with systemic sclerosis]. 1004 21

The gastrointestinal tract is one of the main sites of manifestation of systemic scleroderma. We describe a case of a 62-year-old patient with systemic scleroderma and progressive severe constipation that led to stercoral obstruction and perforation. We make a brief review of the literature on the gastrointestinal manifestations and the management of systemic scleroderma.
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PMID:Chronic constipation--a lethal danger in patients with systemic scleroderma. 1178 5

Scleroderma is a multisystem disorder frequently resulting in disturbed GI motility. Although, especially early in the disease, symptomatic improvement is achieved with prokinetic agents, more severe GI manifestations of scleroderma may be difficult to treat, leading to parenteral feeding and hospitalization. Recently, a new serotonin (5-HT4) receptor agonist prucalopride was shown to have remarkable prokinetic properties, resulting in symptomatic improvement and increased frequency of defecation in patients with chronic functional constipation. Here we report two cases of scleroderma with GI manifestation in which previous prokinetic treatment failed, but where the patients were successfully treated with prucalopride. Our data suggest that prucalopride may be a promising and effective drug to treat GI motility disorders in scleroderma. However, further placebo-controlled double blind studies are needed for full documentation of the usefulness of prucalopride in patients with scleroderma.
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PMID:Treatment of GI dysmotility in scleroderma with the new enterokinetic agent prucalopride. 1181 Nov 66

The changes in PYY in several gastrointestinal disorders and their possible clinical implications are reviewed. The changes in PYY seem to be an adaptive response to alterations in the patho-physiological condition caused by the disease. This becomes evident in gastrointestinal disorders such as diabetes gastroenteropathy, inflammatory bowel diseases, celiac disease, systemic sclerosis and post-intestinal resection state. On the other hand, changes in PYY in chronic idiopathic slow transit constipation appear to be primary and could be one of the etiologic factors of the disease. PYY does not seem to be involved in colorectal carcinoma. Although gastrointestinal dysmotility in neuro-muscular diseases is evident, PYY is not affected. The changes in PYY in gastrointestinal disorders could be beneficial in clinical practice. Thus, in cases where an increase or decrease in PYY is desirable, a diet that increases or decreases PYY synthesis and release can be followed, or a receptor agonist or antagonist can be utilized.
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PMID:Peptide YY in gastrointestinal disorders. 1182 55

Progressive systemic sclerosis (PSS) may involve any portion of the gastrointestinal tract, including the colon. Constipation is common in patients with PSS. Cisapride, a benzamide derivative, is a potentially useful agent in the treatment of chronic idiopathic constipation. The effect of cisapride on colonic transit was evaluated in 16 PSS patients by a radionuclide colonic transit method. Each patient received cisapride orally three times a day for a week. The results showed acceleration in colonic transit in response to cisapride. We conclude that cisapride is effective in the treatment of constipation in patients with PSS.
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PMID:Effects of cisapride on colonic transit in patients with progressive systemic sclerosis. 1218 51

Gastrointestinal abnormalities in systemic sclerosis (SSc) involve both myogenic and neural mechanisms. The aims of this study were to evaluate the rectoanal inhibitory response (RAIR) in SSc patients and to correlate RAIR with duration and subtype of disease, antibody status, and lower gastrointestinal symptoms. Thirty-five patients with SSc completed a questionnaire and underwent anorectal manometry (ARM). Forty-five patients without SSc served as controls. In the 35 SSc patients, 62.3% reported diarrhea, 57.1% reported constipation, and 37.1% reported fecal incontinence. Twenty-five of the 35 scleroderma patients (71.4%) demonstrated an impaired or absent RAIR, compared with none of the 45 controls (P < 0.001). Eleven of 13 incontinent SSc patients (84%) had an impaired RAIR. No correlation was found between RAIR and duration or subtype of SSc, antibody status, or presence of diarrhea or constipation. Impaired RAIR was closely correlated with fecal incontinence, suggesting a possible neural mechanism for maintenance of continence.
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PMID:Impaired rectoanal inhibitory response in scleroderma (systemic sclerosis): an association with fecal incontinence. 1530 98

Systemic sclerosis (SSc) is a generalised connective tissue disease of unknown origin, which clinically shows by skin thickening and sclerosis of different extent (scleroderma) and by typical involvement of visceral organs. At the same time fibrotic and sclerotic changes occur in the blood vesel walls. SSc usually involves females at young and middle age. Myalgias, arthralgias and arthritis are nonspecific, tendon friction rubs in fingers are more typical for this diagnosis. Gastrointestinal involvement starts early in the oropharyngeal part, esophagus and proceeds into the distal parts. Fibrotic changes lead to slow transit dysmotility and pseudoobstruction and/or dilation of the bowels. The main symptoms are dysphagia, pyrosis, malabsorption and constipation. SSc produces two major patterns of abnormality within the lungs a fibrosing alveolitis or a primary pulmonary vascular disease. More frequently an insterstitial process develops which can be followed by pulmonary arterial hypertension. Cardiac involvement can also have different forms. Myocardial fibrosis usually appears at first in the conduction system by arrhythmias and various conduction blocks while pericarditis is mostly asymptomatic. Renal manifestation of SSc is observed in 8-10% patients. The most severe form--scleroderma renal crisis is characterised by the new onset of accelerated hypertension and rapidly progressive oliguric renal failure. No therapies have been proven to modify the course of SSc. Some of the drugs can affect only the skin changes. Majority of the currently applied agents have only a symptomatic effect.
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PMID:[New trends in diagnosis and treatment of systemic sclerosis]. 1696 13

Systemic sclerosis is a chronic disorder of connective tissue that affects the gastrointestinal tract in more than 80% of patients. Changes in neuromuscular function with progressive fibrosis of smooth muscle within the muscularis propria impair normal motor function, which may secondarily alter transit and nutrient absorption. Esophageal manifestations with gastroesophageal reflux and dysphagia are the most common visceral manifestation of the disease, often requiring more intense acid-suppressive medication. Gastric involvement may lead to gastroparesis, which can be found in up to 50% of patients. Severe small bowel disease can present as chronic intestinal pseudo-obstruction with distended loops of small intestine, bacterial overgrowth, impaired absorption and progressive development of nutritional deficiencies. While not studied as extensively, systemic sclerosis often also affects colorectal function resulting in constipation, diarrhea or fecal incontinence. Nutritional support and prokinetics have been used with some success to manage gastric and small or large bowel involvement in patients with systemic sclerosis. Despite advances in management, significant gastrointestinal manifestations of systemic sclerosis still carry a poor prognosis with a five-year mortality exceeding 50%.
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PMID:Gastrointestinal manifestations of systemic sclerosis. 1793 61

In systemic sclerosis esophagus is affected in 90% followed by anal and rectal involvement (50-70%), stomach (40-70%), colon (10-50%) and small bowel (40%). The main clinical findings are esophagic: dysphagia and sign of gastroesophageal reflux and its complications. Gastric symptomatology is a consequence of gastroparesis (postprandial fullness, nausea). Intestinal affection produces hypomotility (abdominal distention, deficient intestinal absorption, bacterial overgrowth). In colon there are diverticula, intestinal constipation, and fecal incontinence. For the diagnosis of GIT involvement the following are useful: studies of digestive tract with contrast media, endoscopy of upper GIT; gastric emptying; pH metry; esophageal and rectal manometry, as well as test to investigate deficient intestinal absorption syndrome. The prokinetic are a cornerstone for the treatment of hypomotility of GIT, along with pump proton inhibitors for the esophageal alteration and antibiotics for bacterial overgrowth.
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PMID:[Systemic sclerosis and the gastrointestinal tract. Diagnostic and therapeutic approach]. 2179 84

Systemic sclerosis is a connective tissue disease characterized by inflammation and fibrosis of multiple organs (skin, gastrointestinal tract, lung, kidney and heart). After the skin, the organ most affected with a frequency of 75 to 90%, the gastrointestinal tract is more often involved. Gastrointestinal tract involvement is manifested by the appearance of oropharyngeal dysphagia, esophageal dysphagia, gastroesophageal reflux, gastroparesis, pseudo-obstruction, bacterial overgrowth and intestinal malabsorption, constipation, diarrhea and/or fecal incontinence. These effects influence food intake and intestinal absorption leading to the gradual emergence of nutritional deficiencies. About 30% of patients with systemic sclerosis are at risk of malnutrition. In 5-10%, gastrointestinal disorders are the leading cause of death. Therapeutic strategies currently available are limited and aimed at reducing clinical symptoms. The multidisciplinary management of these patients, including nutritional intervention, helps improve gastrointestinal symptoms, and avoid malnutrition, morbidity and improve quality of life.
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PMID:Nutrition in systemic sclerosis. 2219 34


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