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)

A patient had systemic mastocytosis of 16 years' duration, with urticaria pigmentosa, osteopetrosis with severe bone pain, mastocytosis of the bone marrow with myeloid metaplasia, gastrointestinal malabsorption, and Cushingold features associated with prolonged usage of corticosteroids. Treatment with a single dose of mithramycin resulted in an abrupt disappearance of the patient's generalized bone pain, but it had little effect on his histamine release-type symptomatology.
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PMID:Mithramycin in the treatment of systemic mastocytosis. 12 7

A 63-year-old man presented with fever, splenomegaly, steatorrhea, diarrhea, and weight loss. A tissue diagnosis of systemic mastocytosis was made. This case is unusual in that diarrhea and steatorrhea were present in the absence of skin lesions and because fever was a prominent symptom. Thus, systemic mastocytosis should be included in the differential diagnosis of intestinal malabsorption even when the skin shows no abnormalities. The gastrointestinal manifestations of systemic mastocytosis are reviewed.
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PMID:Systemic mastocytosis with review of gastrointestinal manifestations. 75 38

Mastocytosis is characterized by increased proliferation of mast cells. Two patients had systemic mastocytosis involving the skin and gastrointestinal tract, complicated by malabsorption and tetany. Absorption studies in these patients suggested that the entire small bowel was involved and that the defect was mild in the absence of diarrhea. Small bowel biopsies disclosed infiltration of the lamina propria and submucosa by mast cells, and gastrointestinal tract x-ray films showed nodular densities, edema, and thickening of the bowel wall. Tetany was due in part to combined hypocalcemia, hypomagnesemia, and hypokalemia. Diarrhea and malabsorption were due to mast cell infiltration of the bowel rather than to histamine. patients with signs of systemic mastocytosis should have careful evaluations and be followed up to prevent development of malabsorption and tetany.
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PMID:Tetany, malabsorption, and mastocytosis. 119 Sep 35

Four consecutive patients with systemic mastocytosis were studied. One patient had a malabsorption syndrome with only minor histological changes of the intestinal mucosa. Another patient with ulcer diathesis had a gastric secretory pattern resembling Zollinger-Ellison syndrome. Serum gastrin and histamine levels were consistently normal in all patients. Endoscopy of stomach and colon disclosed urticaria-like papulae either spontaneously or after topical provocation in all patients. No increase of mast cells was found in multiple mucosal biopsies. A markedly increased gastric tissue content of histamine was found, however, in the three patients studied. The findings suggest that urticaria-like lesions associated with a high tissue content of histamine may be more important that hyperhistaminaemia in causing the various gastrointestinal symptoms.
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PMID:Gastrointestinal involvement in systemic mastocytosis. 126 81

Tixocortol pivalate is a steroid reportedly without significant adrenal-pituitary axis suppression when administered via the gastrointestinal tract. To determine whether this steroid would suppress the gastrointestinal manifestations of systemic mastocytosis, we performed an open clinical trial for safety and efficacy with tixocortal pivalate in four patients for periods of 8-15 weeks. All patients showed a decrease in the symptoms of abdominal pain and frequency of stools. Laboratory parameters of malabsorption improved in parallel with symptom relief. Histopathologic abnormalities of the small bowel improved in one patient. There was no significant suppression of the pituitary-adrenal axis. Two patients developed fluid retention while on tixocortol pivalate, which was attributed to a mineralocorticoid effect. One patient had a fall in AM cortisol. In summary, this study strongly suggests that tixocortol pivalate, when administered orally, has gastrointestinal anti-inflammatory activity comparable to conventional steroids, but may not be entirely without adrenal-suppressive effect and may lead to fluid retention in some patients. Further studies are warranted to assess the value of tixocortol pivalate in the therapy of inflammatory diseases of the upper gastrointestinal tract.
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PMID:Effects of tixocortol pivalate on gastrointestinal disease in systemic mastocytosis: a preliminary study. 204 86

We retrospectively analyzed the small bowel radiographies, performed by enteroclysis in 73 patients presenting a malabsorption disease. The etiology are: celiac disease (44 cases), abetalipoprotidemia (2 cases), Whipple's disease (7 cases), mastocytosis (1 case), amyloidosis (1 case), ischemia enteritis (4 cases), radiation injury (4 cases), lymphangiectasia (4 cases), Crohn's disease (4 cases) and NH lymphoma (2 cases). With enteroclysis, the radiological diagnosis of malabsorption disease is improved and it is able to differentiate malabsorption diseases based on radiological features: increased or decreased number of folds (celiac disease, mastocytosis, abetalipoprotidemia), nodularity of folds (Whipple's disease) and wall thickness (amyloidosis, lymphangiectasia, ischemic enteritis and radiation injury). It is no possible to differentiate NH lymphoma from Crohn's disease.
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PMID:[Enteroclysis in malabsorption syndrome in adults. Apropos of 73 cases]. 273 73

In 16 consecutive patients with systemic mastocytosis, we prospectively evaluated a variety of gastrointestinal functions and examined how they relate to the occurrence of gastrointestinal symptoms. Nine patients had either a duodenal ulcer or duodenitis. Hypersecretion of gastric acid was present in 6 patients, and in these patients the mean basal acid output was 20.7 +/- 4.1 mEq/h (range 14-39 mEq/h). Impaired small intestinal absorption occurred in 5 patients, although this was usually mild. The mean fractional emptying rate of liquids for all patients (14.7% +/- 2.3% per minute) did not differ from that for controls (10.7% +/- 0.6% per minute). Mean mouth-to-cecum transit time measured by breath hydrogen testing was the same among patients (87.7 +/- 6.7 min) and controls (86.7 +/- 8.0 min). Plasma histamine concentrations were increased in all patients (mean 1886 pg/ml, range 480-7450) and correlated with the basal acid output (r = 0.64, p less than 0.02) but not maximal acid output or the presence or absence of pain or diarrhea. Mean fasting plasma concentrations of motilin, substance P, and neurotensin from 6 patients did not differ significantly from controls, whereas gastrin and vasoactive intestinal peptide were significantly less than in controls (p less than 0.01). Gastrointestinal symptoms, consisting of abdominal pain or diarrhea, occurred in 80% of patients. Abdominal pain classified as dyspeptic was usually associated with acid-peptic disease of the duodenum and hypersecretion of gastric acid, whereas abdominal pain of a nondyspeptic character was not. Only in those cases of diarrhea consisting of greater than 200 g stool/day was gastric acid hypersecretion frequently found. Neither fecal urgency nor nondyspeptic pain could be accounted for by alterations of gastrointestinal transit. These results demonstrate that gastrointestinal symptoms, peptic disease, and mild malabsorption are much more common than described previously in patients with systemic mastocytosis. Furthermore, the results provide no evidence for the contention that altered gastrointestinal transit is involved in the pathogenesis of these symptoms.
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PMID:Gastrointestinal dysfunction in systemic mastocytosis. A prospective study. 339 14

Mastocytosis is an unusual disorder of unknown etiology characterized by an abnormal proliferation of tissue mast cells. We present a case of a young woman with systemic mastocytosis with malabsorption secondary to small bowel invasion by mast cells, myelofibrosis, and massive exudative ascites secondary to mass cell invasion of the intraabdominal lymph nodes. The patient responded favorably to a combined therapy with histamine H1 and H2 receptor antagonists and corticosteroids. We include a review of the literature.
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PMID:Mastocytosis with malabsorption, myelofibrosis, and massive ascites. 379 81

Alpha-fluoromethylhistidine, alpha-FMH (L-641.575, Merck, Sharp and Dohme), a specific irreversible inhibitor of the mammalian histidine decarboxylase, was investigated for its effect on possible histamine symptoms in mastocytosis. Two patients were treated for 28 and 22 weeks respectively. The first patient had systemic mastocytosis and a severe malabsorption causing weight loss, excessive fecal fat losses and electrolyte disturbances, the main symptom being frequent diarrhoea. The second patient had mainly skin manifestations diagnosed as urticaria pigmentosa and the main symptom was pruritus. There were no side effects of the drug. The first patient produced formed stools after one week of treatment, concomitant with a decrease in plasma histamine and in urinary excretion of the main histamine metabolite tele-methylimidazoleacetic acid (MeImAA). In the second patient definite benefit of the treatment was observed after two months. However, changes in histamine parameters occurred earlier. Plasma histamine and urinary MeImAA were reduced after one week and in two or three weeks reached a steady-state level of about 25% of pretreatment values. The results indicate an effective inhibition of histamine synthesis in both patients but only the diarrhoea seemed to be causally related to the change in histamine metabolism.
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PMID:Treatment of two mastocytosis patients with a histidine decarboxylase inhibitor. 401 13

Systemic mastocytosis, with its diffuse infiltration of mast cells into various organs, has resulted in intestinal malabsorption and bleeding diatheses. The pathophysiology underlying these phenomena is unclear, but may be related to the release of histamine and heparin containing mast cell granules. A patient with systemic mastocytosis had malabsorption and developed massive bleeding after percutaneous liver biopsy. Histologic involvement of skin, duodenum, rectum, liver, and bone marrow was documented. Mastocytosis should be considered in the differential diagnosis of malabsorption.
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PMID:Bleeding after liver biopsy in a patient with systemic mastocytosis and malabsorption. 404 81


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