Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We examine critically the biochemical methods capable of detecting and monitoring the end-organ disease processes in patients with cystic fibrosis. Although the diagnosis of cystic fibrosis is never justified on the basis of the sweat salt test alone, the original filter-paper technic (Gibson-Cooke, Pediatrics 23:545-549, 1959) for determining Na+ and Cl- concentrations in sweat remains the most discriminating method. We discuss the contributions for neonatal screening of the so-called cystic fibrosis protein, associated decreased enzymic activities in the homo- and heterozygous state, and immunoreactive trypsin. Because evidence of either intestinal malabsorption or a pancreatic lesion must be sought, we review the use and interpretation of some tests of pancreatic dysfunction (meconium albumin, duodenal juice components, serum pancreatic isoamylase, and trypsinogen), both in establishing and in confirming the diagnosis of cystic fibrosis.
 ...
PMID:Cystic fibrosis--its biochemical detection. 635 39

Two trypsin assay methods for the estimation of this enzyme in duodenal fluid from children have been compared. Assay results for a fluorometric method based on the use of N-carbobenzoxy-diglycyl-L-arginyl-2-naphthylamide hydrochloride (GANA) as the trypsin substrate were found to correlate well (r = 0.91, P less than 0.001) with those obtained with a much less sensitive titrimetric assay which used benzoylarginine ethylester hydrochloride (BAEE) as substrate. The higher sensitivity of the fluorometric assay has allowed accurate determination of trypsin activity in 10 microliter aliquots of duodenal fluid. This low volume requirement makes the assay suitable for studies on infants of all ages and conserves duodenal fluid for use in other investigations often warranted during the assessment of childhood malabsorption. The fluorometric assay has also been used to monitor the separation of enteropeptidase from trypsin(ogen) by chromatography on Sephacryl S-200 in samples of duodenal fluid from two children. Different proteolytic pathway deficiencies were confirmed in these children.
 ...
PMID:Fluorometric microassay of trypsin and enteropeptidase in children--comparison with a titrimetic assay. 639 83

Patients with cystic fibrosis have been found to have abnormal serum concentrations of immunoreactive trypsin and abnormal activities of pancreatic isoamylase. A study was undertaken to discover whether activity of pancreatic lipase is also altered in cystic fibrosis. Serum from 23 patients with cystic fibrosis was assayed for immunoreactive trypsin and pancreatic lipase. Median serum pancreatic lipase activity was significantly lower in patients with cystic fibrosis than in controls, as was immunoreactive trypsin concentration (p less than 0.0001). Some patients had supranormal lipase concentrations but these were not always associated with absence of malabsorption. Serum pancreatic lipase activity is considerably changed in cystic fibrosis.
 ...
PMID:Serum pancreatic lipase activity in cystic fibrosis. 640 37

The therapeutic efficacy of a pH-sensitive enteric coated pancreatic enzyme preparation was compared with conventional pancreatic enzyme preparations in 6 adult patients with exocrine pancreatic insufficiency. Fecal fat excretion and postprandial duodenal recovery of orally ingested pancreatic enzymes were evaluated after ingestion of each preparation. Fecal fat excretion decreased significantly (p less than 0.005) on treatment with pH-sensitive and conventional pancreatic enzyme preparations. Postprandial concentration and delivery of trypsin and lipase in samples aspirated from duodenojejunal junction were higher after ingestion of conventional pancreatic enzyme preparation as compared to the pH-sensitive enteric coated preparation. The difference, however, did not reach statistical significance. Our observations suggest that the pH-sensitive enteric coated pancreatic enzyme preparation is only as effective as conventional pancreatic enzyme preparations in controlling fat malabsorption in patients with exocrine pancreatic insufficiency. Failure of pH-sensitive enteric coated preparation to deliver greater quantities of pancreatic enzymes at duodenojejunal junction is most likely related to the impaired release of enzymes from microspheres due to low intraluminal pH in the upper small intestine in pancreatic insufficiency.
 ...
PMID:Comparative evaluation of the therapeutic efficacy of a pH-sensitive enteric coated pancreatic enzyme preparation with conventional pancreatic enzyme therapy in the treatment of exocrine pancreatic insufficiency. 654 46

The purpose of this study was to determine whether or not nutrients in the colon influence pancreatic and biliary secretion in humans. In six healthy subjects, similar caloric loads (117 cals) of oleic acid, essential amino acids, and glucose in isomolar (280 mosmol/L), similar pH (7.4) solutions were infused into the right colon at 10 ml/min through a colonic tube passed by mouth. A background of submaximal pancreatic and biliary secretion was maintained by continuous intravenous infusion of the octapeptide of cholecystokinin. Biliary and pancreatic secretions were quantitated using the gastroduodenal intubation perfusion technique. Among the three nutrients tested, only oleic acid in the colon decreased pancreatic enzymes and bicarbonate outputs. The mean trypsin output decreased from 26.3 +/- 2.6 kU/h to 12.3 +/- 1.9 kU/h (46% +/- 6% of control), while the lipase output decreased from 62 +/- 6.6 kU/h to 36 +/- 5.6 kU/h (58% +/- 6% of control). Similarly, the output of bicarbonate in the duodenal aspirate decreased from 31 +/- 7.2 mEq/h to 16.3 +/- 3.1 mEq/h (61% +/- 5% of control). Intracolonic perfusion of essential amino acids or glucose had no effect on pancreatic enzymes and bicarbonate secretion. In contrast, all three nutrients in the colon inhibited biliary secretion. The mean output of bilirubin decreased from 56 +/- 6 mg/h to 19 +/- 2 mg/h (35% +/- 5% of control) during intracolonic perfusion of oleic acid. Essential amino acids lowered the output of bilirubin from 54 +/- 12 mg/h to 31 +/- 8.6 mg/h (65% +/- 6% of control), whereas glucose lowered it from 53 +/- 12 mg/h to 22 +/- 4 mg/h (45% +/- 5% of control). This differing response of pancreatic and biliary output to intracolonic perfusion of nutrients suggests differential sensitivity of the pancreas and gallbladder to these inhibitory influences. In malabsorption states, unabsorbed nutrients in the colon may inhibit pancreatic and biliary secretion, further contributing to the loss of nutrients from the gastrointestinal tract.
 ...
PMID:Colonic inhibition of pancreatic and biliary secretion. 682 21

Some patients with cystic fibrosis (CF) have malabsorption of fat and protein in spite of large amounts of supplemental pancreatic enzymes. This is partly due to acid inactivation of exogenous pancreatic enzymes in the stomach. The effect of cimetidine on gastric function and exogenous pancreatic enzymes was assessed by a marker perfusion technique in 4 CF children in a double-blind controlled fashion. Gastric acid secretion was higher in CF patients than in controls (P less than 0.005) and was reduced significantly by oral cimetidine (P less than 0.02). Rapid inactivation of exogenous trypsin and lipase occurred when gastric pH fell to less than 4.5. There was no loss of enzyme activity during treatment with cimetidine when gastric pH remained above 5.5. Activity of lipase and trypsin in the jejunum improved in all subjects. Fat and nitrogen absorption assessed by a balance technique during the study period showed a small improvement in fat absorption while on cimetidine. We conclude that some CF patients have a high meal-stimulated gastric acid output which causes inactivation of trypsin and lipase. Cimetidine was effective in reducing acid secretion in such patients and led to small improvements in fat absorption.
 ...
PMID:The effect of cimetidine on meal-stimulated gastric function and exogenous pancreatic enzymes in cystic fibrosis. 692 76

Aflatoxicosis, ochratoxicosis, and T-2 toxicosis were produced by feeding diets containing graded concentration of the appropriate toxin to broiler chicks from hatching unit 3 weeks of age. Aflatoxin, even at levels not growth inhibitory, produced a malabsorption syndrome characterized by steatorrhea, hypocarotenoidemia, and decreased concentrations of bile salts and pancreatic lipase, trypsin, amylase, and RNase. The T-2 toxin at concentrations higher than required to inhibit growth produced a mild malabsorption syndrome characterized by steatorrhea and decreased levels of pancreatic lipase, trypsin, amylase, and RNase. The only suggestion of malabsorption during ochratoxicosis was a severe hypocarotenoidemia. The following observations indicated a lack of correlation between lipid malabsorption and hypocarotenoidemia. The T-2 toxicosis exhibited lipid malabsorption in the absence of hypocarotenoidemia, ochratoxicosis exhibited hypocarotenoidemia in the absence of lipid malabsorption, and aflatoxicosis exhibited both symptoms. These findings imply that carotenoids are physiologically active compounds with specific metabolic processes and are not inert substances swept along with lipids as is commonly assumed from the ability to grow apparently healthy birds free of carotenoids. The current findings also indicate that great specificities exist in mycotoxicoses despite superficial similarities.
 ...
PMID:Comparison of ochratoxin, aflatoxin, and T-2 toxin for their effects on selected parameters related to digestion and evidence for specific metabolism of carotenoids in chickens. 713 18

Previous data from this laboratory have shown good correlation between plasma cationic trypsin(ogen) and levels of pancreatic function in older cystic fibrosis (CF) patients with and without malabsorption. In the present study a radioimmunoassay for human anionic trypsin(ogen) has been employed in the assessment of pancreatic function in older patients with CF. Immunoreactive anionic trypsin(ogen) levels correlated poorly with pancreatic function due to an apparent elevation of this enzyme in many older CF patients with malabsorption (CF + M). When plasma from three older CF + M patients is examined for the molecular size of the apparent immunoreactive material detected, no free anionic trypsinogen is observed. Instead, a broad peak of apparent immunoreactive material appears in the gammaglobulin region. However, only free anionic trypsinogen could be detected in plasma from two CF patients without malabsorption (CF - M), who had not received pancreatic enzyme supplements. It appears possible that a human plasma immunoglobulin G (IgG) to porcine pancreatic enzyme in the CF + M patients might interfere in the assay by binding TLCK--anionic trypsin tracer. It is unclear why such an effect does not appear to occur in the cationic trypsin(ogen) assay. The results of the current study suggest that, for assessment of pancreatic insufficiency in CF, the radioimmunoassay for cationic trypsin(ogen) is more useful than the presently available radioimmunoassay for the anionic form.
 ...
PMID:Plasma immunoreactive anionic pancreatic trypsin in cystic fibrosis. 718 46

Serum immunoreactive trypsin (IRT) concentrations were measured blind in sera from 33 patients with cystic fibrosis and in 32 age-matched controls. Thirty-two patients had abnormal trypsin concentrations. In 30 of these patients the level of IRT was very low whereas in the other two (the youngest of the group) it was extremely high. All 30 patients with low IRT concentrations had clinical or biochemical evidence of malabsorption. Serum IRT concentration may thus be a useful diagnostic test for cystic fibrosis. In view of the recent observation that neonates with cystic fibrosis consistently have raised IRT concentrations, and the fact that the youngest two patients in our series also had markedly increased IRT, it would appear that the initial elevation of IRT reflects early damage to the pancreas with release of enzyme/zymogen; this is thereafter superseded by a progressive destruction of the pancreas resulting in low IRT concentrations in adults with cystic fibrosis.
 ...
PMID:Serum immunoreactive trypsin in cystic fibrosis. 729 84

Several reports suggest a relationship between bone marrow transplantation and pancreatic damage. The authors describe two patients with pancreatic insufficiency after bone marrow transplantation. The first patient had weight loss arising from steatorrhea secondary to severe pancreatic insufficiency (lipase output result <3% of normal) due to pancreatic atrophy. The second patient had steatorrhea secondary to intestinal bacterial overgrowth, but moderate pancreatic insufficiency was present (results of trypsin and lipase outputs were 50 and 30% of normal). Before the diagnosis of pancreatic insufficiency, both patients had acute graft vs host disease and the second patient had chronic graft vs host disease. We conclude that graft vs host disease after bone marrow transplantation may damage the pancreas. Patients with persistent malabsorption after bone marrow transplantation should be tested for exocrine pancreatic insufficiency.
 ...
PMID:Exocrine pancreatic insufficiency after allogeneic bone marrow transplantation. 764 72


<< Previous 1 2 3 4 5 6 Next >>