Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of acute Mast-cell leukemia was studied. A 39 years old female presenting with a brief history of abdominal pain and attacks of flushing; peripheral blood and bone marrow contained up to 60% of poorly differentiated blasts with clumping of deep purpule granules. Peroxydase reaction stains were negative, chloroacetate esterase were strongly positive. Toluidine blue revealed metachromatic stain. Histamine content of the cells was highly greater than normal but nos heparinoid activity could be demonstrated. These abnormal mast-cells have been investigated with the electron microscope; only the dense particular type of granule substructure was found, without any lamellae component. The cells were temptatively classified as "immature" mast-cell. The disease was interpretated as an acute leukemic variety of systemic mastocytosis.
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PMID:[Acute mast-cell leukemia. Cytochemical and ultrastructural study, about a particular case (author's transl)]. 5

A double-blind crossover study of the efficacy of disodium cromoglycate given by mouth to control the cutaneous, gastrointestinal and central-nervous-system manifestations of systemic mastocytosis was carried out in five patients for periods of eight to 32 months. In 15 of 18 trials, disodium cromoglycate produced marked amelioration of the clinical manifestations of pruritus, whealing, flushing, diarrhea, abdominal pain and disorders of cognitive function. By contrast, in all 19 trials with placebo, there was no improvement in these symptoms and signs. Histaminuria and peripheral-blood eosinophilia were unrelated to disease activity and were unaffected by drug therapy. Although it is poorly absorbed after administration by mouth, disodium cromoglycate is of clinical benefit to patients with systemic mastocytosis.
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PMID:Oral disodium cromoglycate in the treatment of systemic mastocytosis. 11 Nov 24

The case histories of two patients with benign systemic mastocytosis with skin involvement are presented. The first patient had urticaria pigmentosa diagnosed at the age of 2 months, and developed systemic disease within two years. The second presented urticaria pigmentosa at the age of 22 years while benign systemic mastocytosis was diagnosed 30 years later. The clinical findings in both cases were: skin involvement, hepatosplenomegaly and abdominal pain. The second patient had myelofibrosis. There was a favorable response to H1 and H2 histamine antagonist and ketotifen.
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PMID:[Benign systemic mastocytosis: report of 2 cases]. 184 75

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

A multicenter, double-blind, placebo-controlled trial of the efficacy of oral cromolyn sodium (200 mg orally four times per day) was conducted in 11 patients with systemic mastocytosis who had been maintained with the drug on an individualized compassionate-need basis. Efficacy was measured by physician assessment of overall disease severity based on history and physical examination at specified intervals and by the average daily patient symptom diary scores for each of three mastocytosis-related symptoms that had previously appeared to be alleviated by the use of this drug. Efficacy variables were compared for a 4-week baseline period, during which patients received open-labeled cromolyn sodium, and at 4-week intervals during a 16-week period of random assignment to cromolyn sodium or placebo. Overall disease severity and symptoms recorded in patient diaries were graded on a scale of 0 (absent) to 5 (incapacitating). The average physician assessment of disease severity and symptom scores of the patients in the placebo-treated group increased significantly during the randomization phase relative to patients in the cromolyn sodium-treated group, reflecting an exacerbation of symptoms with drug withdrawal (p less than 0.05 and less than 0.028, respectively). When the symptom scores were analyzed separately for gastrointestinal manifestations of disease (diarrhea, abdominal pain, nausea, and vomiting), cromolyn sodium treatment was significantly beneficial relative to placebo (p less than 0.02), whereas the benefit for nongastrointestinal manifestations did not reach statistical significance.
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PMID:Cromolyn sodium in the management of systemic mastocytosis. 211 Jan 98

To asses the efficacy of ketotifen (Zaditen; Sandoz Pharmaceuticals, Basel, Switzerland) for the treatment of pediatric mastocytosis, eight children who exhibited symptoms as a result of mastocytosis were enrolled in a 12-week, double-blind, placebo-controlled, crossover trial of ketotifen versus hydroxyzine (Atarax; Roerig, New York, N.Y.). Efficacy of each drug was assessed by daily symptom scores and plasma- and 24-hour urine-histamine levels. After completion of the study, symptom scores revealed that seven of the eight children exhibited a greater reduction in symptoms while they were receiving hydroxyzine (p less than 0.05). The symptoms most likely to improve with treatment with hydroxyzine were flushing and abdominal pain. Analysis of plasma- and 24-hour urine-histamine levels at the beginning and end of each trial period of each drug revealed no significant differences (p greater than 0.20). Changes in 24-hour urine-histamine levels, but not plasma-histamine levels, correlated with changes in symptom scores. We conclude that ketotifen offers no advantage over hydroxyzine in the treatment of pediatric mastocytosis.
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PMID:A double-blind, placebo-controlled, crossover trial of ketotifen versus hydroxyzine in the treatment of pediatric mastocytosis. 232 20

Mastocytosis is a disease characterized by an increase in the number of tissue mast cells and a concomitant increase in mast cell-derived mediators. To demonstrate the spectrum of skin disease in mastocytosis in the pediatric population, five children with mastocytosis and complaints of urticaria (4/5), bullae/vesicles (3/5), abdominal pain (3/5), flushing (2/5), headache (1/5), and bone pain (1/5) are reviewed. Confirmation of the diagnosis of cutaneous mastocytosis was obtained by histologic examination of a biopsy of lesional skin; however, mast cell numbers in lesional skin did not correlate with plasma histamine levels or the extent of cutaneous involvement. Mastocytosis is a diagnosis that must be recognized in the differential diagnosis of pediatric urticarial diseases.
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PMID:Mastocytosis in infants and children: recognition of patterns of skin disease. 292 86

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

Most clinical signs and symptoms of systemic mastocytosis (SM) are attributed to histamine release. We report here a 5-year-old male child with SM, who suffered from the age of 4 months from disseminated skin lesions, vomiting, diarrhoea, abdominal pain, flushing, tachycardia, hypotension, somnolence, and transient blindness, triggered by heat and egg ingestion. Oral disodium cromoglycate (DSCG) or placebo were started in a single blind trial at a dose of 100 mg/kg/day in four divided doses. The child was studied for 21 months during the administration of three courses of DSCG, each of 6 months' duration, interspersed with three 1-month courses of placebo. During treatment with DSCG all the systemic manifestations improved, and the histaminaemia decreased. During the placebo periods the symptoms, signs, and histaminaemia recurred.
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PMID:Systemic mastocytosis in a 5-year-old child: successful treatment with disodium cromoglycate. 642 16

A 75-year-old woman with known systemic mastocytosis presented with abdominal pain, ascites, and bile duct thickening on computed tomography and ultrasonography. A liver biopsy specimen showed infiltration with mast cells. Endoscopic retrograde cholangiography showed ductal changes compatible with those found in primary sclerosing cholangitis. Brush cytology of the intrahepatic bile ducts confirmed mast cell infiltration. Systemic mastocytosis can infiltrate the biliary system, producing a cholangiopathy radiographically similar to primary sclerosing cholangitis.
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PMID:Mast cell cholangiopathy: another cause of sclerosing cholangitis. 755 53


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