Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:3.4.21.4 (trypsin)
42,187 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A newborn infant with ante-natally ultrasonically diagnosed small bowel obstruction was found to have two isolated jejunal atresias at laparotomy. Assay of immuno-reactive trypsin enabled an early diagnosis of cystic fibrosis to be made, later confirmed by an elevated sweat sodium.
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PMID:Diagnosis of cystic fibrosis, by assay of immuno-reactive trypsin in a newborn infant with jejuno-ileal atresias. 372 8

In order to define basic biliary defects not related to steatorrhoea in cystic fibrosis, we studied 12 control and 18 cystic fibrosis subjects, with a wide range of pancreatic function. Duodenal aspirates were collected over three consecutive 20 minute periods, during continuous intravenous infusion of cholecystokinin and secretin using a marker perfusion technique, and analysed for pancreatic enzyme output (colipase, lipase, trypsin), bile acid output and concentration, and biliary lipids. Cystic fibrosis patients, at all levels of pancreatic function, had significantly reduced total bile acid output (mumol/kg/h) with delayed appearance of the bile acid peak, compared with control subjects. Actual duodenal bile acid concentrations were significantly higher in cystic fibrosis subjects than in controls, however, probably because of the markedly reduced water output shown in these patients. The lithogenic index was not raised in cystic fibrosis patients at any level of pancreatic function. The reduced bile acid output and the delayed peak appearance probably reflect a defect in gall bladder responsiveness which is independent of pancreatic function and steatorrhoea. Whether this defect is related to gall bladder filling or a defective peptide hormone response awaits further study.
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PMID:Bile acid secretion in cystic fibrosis: evidence for a defect unrelated to fat malabsorption. 375 17

We evaluated serum cationic trypsinogen as a marker of exocrine pancreatic function in children without cystic fibrosis. The ability of this test to determine steatorrhoea of pancreatic origin, and its relationship to a wide range of exocrine pancreatic function were assessed. Serum trypsinogen was measured in 32 children with steatorrhoea, 10 with pancreatic and 22 with non-pancreatic causes. In patients with pancreatic steatorrhoea, serum cationic trypsinogen was 4.9 +/- 4.9 micrograms/l (mean +/- SD), significantly below values in patients with non-pancreatic steatorrhoea (47.0 +/- 22.1 micrograms/l, p less than 0.001) and 50 control subjects (31.4 +/- 7.4 micrograms/l, p less than 0.001). Serum cationic trypsinogen values in patients with pancreatic steatorrhoea all fell below the lower limit of our control range and below all values for patients with non-pancreatic steatorrhoea. Serum cationic trypsinogen was also evaluated against pancreatic trypsin output in 47 patients (range 0.2-17.0 yr who underwent a hormonal pancreatic stimulation test. In 17 patients, serum cationic trypsinogen was low (less than -2SD or less than 16.6 micrograms/l), and associated with greatly impaired pancreatic trypsin output, ranging from 0-8% of mean normal trypsin output. Five of these 17 patients did not have steatorrhoea. In 30 patients with normal or raised serum cationic trypsinogen (greater than or equal to 16.6 micrograms/l), pancreatic trypsin output ranged from 15-183% of mean normal values. In conclusion, low serum cationic trypsinogen suggests severely impaired exocrine pancreatic function, with sensitivity extending above the steatorrhoeic threshold. In the presence of steatorrhoea, low serum cationic trypsinogen indicates a pancreatic aetiology. Normal serum cationic trypsinogen, however, does not exclude impaired pancreatic function, above the steatorrhoeic threshold.
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PMID:Serum immunoreactive cationic trypsinogen: a useful indicator of severe exocrine dysfunction in the paediatric patient without cystic fibrosis. 379 18

We evaluated the bentiromide test by analyzing para-aminobenzoic acid (PABA) in plasma and urine (a) for the identification of patients with complete pancreatic insufficiency and (b) as an alternative to the secretin-cholecystokinin test. Nine control subjects, 18 patients with cystic fibrosis, and 4 patients with Shwachman's syndrome were studied. Based upon the secretin-cholecystokinin test, pancreatic function was judged to be less than 0.1% of normal in 7 patients with cystic fibrosis and malabsorption and between 0.7% and 90% of control values in 11 patients with cystic fibrosis and 4 patients with Shwachman's syndrome without malabsorption. The bentiromide test was performed in two stages: first with bentiromide alone, then with equimolar free PABA. After ingestion of free PABA, the plasma profile and urinary excretion of PABA were comparable in controls, patients with cystic fibrosis, and patients with Shwachman's syndrome. Thirty minutes after oral bentiromide, plasma PABA values in patients with and without malabsorption were significantly lower than in the control group. From 60 to 180 min after ingestion, plasma PABA levels in patients without malabsorption were no different from controls; whereas levels in patients with malabsorption were significantly lower than in controls and in those without malabsorption, reaching the highest significance at 90 min. Similar results were obtained when the urinary excretion of PABA was considered. Only the 90-min plasma test reliably detected cystic fibrosis patients with steatorrhea, however. Duodenal colipase output was highly correlated with both the 90-min plasma test and the urinary excretion of PABA, with similar results for lipase and trypsin output. Reliable detection of pancreatic dysfunction, nevertheless, was not obtained even with the plasma test, in cystic fibrosis patients with greater than 5%-10% of the mean normal enzyme output. In patients with Shwachman's syndrome, none of whom had malabsorption, the plasma and urinary test failed to detect pancreatic dysfunction even with enzyme output as low as 1% of normal.
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PMID:Bentiromide test for assessing pancreatic dysfunction using analysis of para-aminobenzoic acid in plasma and urine. Studies in cystic fibrosis and Shwachman's syndrome. 387 4

We performed pancreatic function tests on sixty-five cystic fibrosis (CF), and eleven control children. The technique used continuous IV infusion of cholecystokinin and secretin, with duodenal juice collection over a 90 min period, and was made quantitative by continuous duodenal infusion and distal collection of an unabsorbable marker (bromosulphthalein). Some CF patients had near normal pancreatic enzyme outputs, some had impaired but measurable levels, but most (79%) had almost absent trypsin secretion. CF children with better pancreatic function, were younger and more likely to be male. All controls showed a large increase in bicarbonate concentration and secretion rate per kilogram body weight during the test, but most children with CF (96.5%) did not. Because two of our CF patients had water and bicarbonate secretion within the control range, this finding does not exclude the diagnosis of CF. Sodium, potassium and chloride ion secretion in CF patients was lower than controls but overlap occurred. We found a linear correlation between acinar and tubular secretion in CF patients which indicates that there is probably not a primary genetic defect in pancreatic bicarbonate secretion in CF.
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PMID:Abnormal pancreatic electrolyte secretion in cystic fibrosis. Reliability as a diagnostic marker. 395 70

To study pancreatic protein and water secretion in 28 patients with cystic fibrosis and 21 controls matched for pancreatic acinar function as defined by trypsin secretion, we used a quantitative-marker perfusion technique and continuous intravenous secretin-pancreozymin stimulation. Regardless of the level of pancreatic acinar function, secretions from the patients contained significantly higher concentrations of protein than those from the controls. Total protein output and albumin:protein ratios were not increased in secretions from the patients, but their fluid secretion was significantly decreased at any level of pancreatic function. A significant linear correlation was found between protein and volume secretion in the patients (r = 0.86, P less than 0.001), most of whom had a fluid output of less than 4.2 ml per kilogram of body weight per hour. No such relation was found in the control subjects, whose flow was always above 4.2 ml per kilogram per hour. We conclude that fluid secretion in patients with cystic fibrosis may be a rate-limiting factor in protein output and that a limited flow of hyperconcentrated protein secretions may predispose to protein precipitation and ductal obstruction in the pancreas.
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PMID:Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. 396 86

Peripheral membrane glycopeptides isolated from skin fibroblasts of patients with cystic fibrosis (CF) had an altered fucosylation when compared to age, race, and sex-matched controls. Glycopeptides obtained by trypsin treatment of cultured cells, metabolically labeled with L-[3H]fucose, were purified on a Sephadex G-50 column. The neutral monosaccharide composition of the glycopeptides was determined by gas-liquid chromatography of the alditol acetate derivatives. The fucose content of the CF membrane glycopeptides and the molar ratio of fucose to the other monosaccharides were increased when compared with those of controls. The mean fucose content was 2.3 +/- 1.7 (SD) and 0.3 +/- 0.05 (SD) nmol per 10(6) cells for CF control glycopeptides, respectively. Moreover, the specific activity of incorporated L-[3H]fucose was markedly decreased in the CF glycopeptides, 355 +/- 183 (SD) and 2707 +/- 839 (SD) cpm per nmol of fucose for the CF and controls, respectively. The membrane glycopeptides were further purified and characterized by affinity chromatography on immobilized lectins. The decreased specific activity of incorporated fucose was present in CF glycopeptides which bound to immobilized lentil lectin before and after Pronase degradation. Moreover, the CF glycopeptides bound more tightly to lentil lectin. Several classes of glycopeptides bind to lentil lectin and all contain Fuc alpha 1----6GlcNAc at the core. The binding characteristics of the Pronase-digested membrane glycopeptides for immobilized concanavalin A, lentil leukoagglutinating phytohemagglutinin, erythroagglutinating phytohemagglutinin, and serotonin provided information on the structure of the altered CF glycopeptides. Most of these radioactive glycopeptides were of the biantennary type containing at least one sialic acid residue and all containing fucosyl residues at the asparagine core.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Altered fucosylation of membrane glycoproteins from cystic fibrosis fibroblasts. 400 Jul 63

Serum immunoreactive trypsin (IRT) and pancreatic lipase have been measured in 59 patients with cystic fibrosis (age 1 month-27 years). Follow-up values were obtained from 49 patients. Their serum enzyme levels were compared to those of 120 healthy children of all age groups. Faecal fat excretion was determined in selected patients (n = 23) to elucidate the relationship between serum enzyme levels and pancreatic exocrine function. In cystic fibrosis IRT and lipase showed a very similar age-correlated pattern: in infancy levels were markedly elevated. During the following years the concentrations of both enzymes decreased rapidly and were found to be far below the normal range after the 10th year of life. Elevated enzyme levels in infancy as well as low levels in all age groups coincided with steatorrhea. Older patients (11-27 years) without severe pancreatic insufficiency however, had IRT and lipase levels in or above the normal range. In healthy children there was no age dependency of IRT levels, whereas in the first 12 months of life lipase levels were significantly lower than in later childhood.
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PMID:Serum immunoreactive trypsin and pancreatic lipase in cystic fibrosis. 404 29

Chronic reserpine treatment of adult rats results in the accumulation of pancreatic enzymes and reduction of their discharge. These changes are reminiscent of those in cystic fibrosis. Since the majority of cystic fibrosis patients have their pancreatic dysfunction manifested in childhood, we studied chronic reserpine treatment in rat pups. Four-day-old rat pups were given reserpine (50 micrograms/kg intraperitoneally) or vehicle daily until sacrifice. The reserpine group showed significant decreases in body weights at 14 and 21 days of age. Pancreatic weights were also decreased but were of normal weight or increased when normalized against body weights. At 14 and 21 days of age, pancreatic concentrations of amylase, lipase, and trypsinogen showed no difference between reserpine and control pups. At both ages, pancreatic contents of all three enzymes were generally less in the treated pups, but were found to be similar when corrected for body weights. Hydrocortisone treatment of 14-day-old pups caused precocious accumulation of pancreatic enzymes in both reserpine and control groups. Intestinal contents of lipase, trypsin, and amylase were decreased in the reserpine pups at 14 days of age and reached a more significant level at 21 days of age; these data suggest a decrease in the secretion of pancreatic enzymes. Dispersed acini from 14-day-old pups showed a reduced capacity to release amylase as stimulated by carbachol or the octapeptide of cholecystokinin. The results suggested that chronic reserpine treatment of pups in the suckling period did not cause significant disturbance of the developmental accumulation of pancreatic enzymes. A definite inhibition of exocrine secretion was found with reserpine treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Effect of chronic reserpine treatment on the pancreases of neonatal rats. 608 31

The activity of Ca-ATPase (Ca2+,Mg2+-ATPase, ATP phosphohydrolase, EC 3.6.1.3) was measured in erythrocyte membrane preparations from 37 cystic fibrosis patients, 27 with pancreatic insufficiency and 10 with pancreatic sufficiency, and from 24 healthy controls. The mean maximal calcium-stimulated specific activities, in the absence and presence of purified calmodulin, of the pancreatic sufficient patients (34.3 +/- 4.2 and 75.9 +/- 6.9 nmol/min/mg) was indistinguishable from that of controls (35.8 +/- 2.6 and 84.3 +/- 4.7 nmol/min/mg), while both activities of patients with pancreatic insufficiency were significantly decreased (28.9 +/- 1.3, p less than 0.02; 65.2 +/- 3.0, p less than 0.001) compared to the control group. Similarly, the mean erythrocyte membrane (Na + K)ATPase activity was decreased only for those patients with a history of steatorrhea and who clinically required pancreatic enzyme therapy and had low immunoreactive trypsin levels (10.6 +/- 0.8 versus control, 13.4 +/- 1.1, and pancreatic sufficient patients, 13.3 +/- 1.4 nmol/min/mg; p less than 0.025). No correlation was found between any of the ATPase activities and the clinical scores of the patients, suggesting the lack of significant contribution of general clinical status to the activities of those cation transporters.
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PMID:Calcium-ATPase activity in cystic fibrosis erythrocyte membranes: decreased activity in patients with pancreatic insufficiency. 609 Oct 22


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