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)

Patients with cystic fibrosis (C.F.) showed raised serum levels of alpha-fetoprotein (AFP). A moderate but significant increase in serum AFP was present in their parents and some siblings. There was no correlation between the clinical severity of the disease and serum AFP concentration. Samples from control groups with gluten-induced malabsorption and bronchiectasis had normal levels. Persistent synthesis of AFP may be an associated marker of C.F. genes, and estimation of serum AFP might help in detecting heterozygote carriers in families at risk.
 ...
PMID:Serum alpha - fetoprotein levels in patients with cystic fibrosis and their parents and siblings. 4 79

Cystic fibrosis as a specific disease entity has been known to be associated with malnutrition for almost half a century. The importance of the malnutrition in the disease process remains unknown, as does much information about specific nutritional deficiencies in CF. Supplements for children with CF should include extra energy as fat or carbohydrate, a form of linoleic acid that can be absorbed, hydrolyzed protein, fat-soluble vitamins with vitamins A and E in a water emulsion, vitamin B12, probably B vitamins and vitamin C, and trace minerals. Routine measurements of nutritional status, particularly in children with growth failure, should be made at regular intervals and should include a three-day diet record and a simultaneous 72-hour stool fat determination. If fat malabsorption is not controlled by pancreatic enzymes, the use of antacids or cimetidine should be considered. The true role of nutrition in patients with CF will not be known until the appropriate studies are completed.
 ...
PMID:Cystic fibrosis and malnutrition. 38 15

Agarose gel electrophoresis (at pH 8.6) was used for qualitative determination of pancreatic enzymes in duodenal juice. The various enzymes were identified by staining techniques with specific chromogenic substrates, by quantitative determination of enzymes in eluates of gel slices, and by immunoelectrophoresis. The various protein bands corresponded to the following enzymes (from the anode to the cathode): chymotrypsin, trypsin, carboxypeptidase A, chymotrypsin, amylase (around the slit), lipase, elastase, and trypsin. The method was applied to a study of exocrine pancreatic function in 10 adults and 83 children suspected of having malabsorption. The duodenal juice, also analyzed for trypsin and amylase content, was collected in fasting condition and after a test meal of water. In patients with normal pancreatic function, all the enzyme bands were present and easy to recognize. In 87 patients carboxypeptidase A was present as two bands in 68 (80%), anodal trypsin as two bands in 39 (45%), and cathodal trypsin as two bands in 85 (97%). Electrophoresis of duodenal juice gave as much information from the fasting sample as after the test meal. Six children with pancreatic insufficiency (cystic fibrosis and Shwachmar's syndrome) had no or only faintly stained enzyme bands and a strongly stained albumin-containing band most anodally. The method is simple, rapid, and useful in routine work. The combination of this qualitative test with a quantitative one (e.g. trypsin determination) provides good information about exocrine pancreatic function.
 ...
PMID:Agarose gel electrophoresis of duodenal juice in normal condition and in children with malabsorption. 43 37

Malabsorption of bile acids is known to occur in patients with pancreatic insufficiency particularly when due to cystic fibrosis. Abnormal biliary secretion or intraluminal acidic precipitation of bile acids could contribute to the steatorrhea of pancreatic insufficiency. To measure bile acid outputs and duodenal concentrations of bile salts and lipids simultaneously, we performed intestinal intubation and perfusion studies during feeding of a solid test meal in 6 healthy controls and 8 adult patients with advanced acquired exocrine pancreatic insufficiency. The effects of various treatment regimens were also investigated. Postprandial bile acid secretion was similar in all treatment groups. However, significant (P less than 0.05) reductions in micellar concentrations of bile acids and fatty acids were observed in untreated pancreatic insufficiency. These abnormalities were directly related to pH-induced precipitation of bile acids and were corrected only by the addition of cimetidine to standard pancreatin therapy. Thus, in pancreatic insufficiency, treatment with pancreatin plus cimetidine enhances fat digestion and absorption by reducing both acid-peptic inactivation of lipase and acidic precipitation of bile acids.
 ...
PMID:Reduced intraluminal bile acid concentrations and fat maldigestion in pancreatic insufficiency: correction by treatment. 44 41

A test meal for assessing the intraluminal phase of absorption in childhood has been validated. 132 test meals were administered to 110 patients aged 2 weeks to 18 years (mean age 4.3 years). 10 children with suspected malabsorption, who were proven to be normal after extensive investigation, constituted the control group. The activities of pancreatic enzymes, and the total and individual bile salt concentrations are presented for the control subjects, and pancreatic enzyme levels in this group are compared with those seen in children with pancreatic insufficiency (cystic fibrosis). The test meal has been designed so that it can be administered to children with suspected gluten, cows' milk, or disaccharide intolerance. The control data provided a basis for the interpretation of information obtained from the application of such a test meal to the clinical investigation of children with suspected malabsorption.
 ...
PMID:Test meal for assessing intraluminal phase of absorption in childhood. 59 27

31 children with cystic fibrosis (CF) subdivided into three groups of age were studied before and after treatment with lyophilized pancreas and compared to 27 controls. CF was characterized by lower phospholipid, cholesterol and polyunsaturated fatty acid plasma concentrations and reduced coefficient of fat absorption. Treatment tended towards normalization of these values. With age polyunsaturated fatty acids increased in normals but not in CF. The significant correlations existing between fat absorption coefficient and polyunsaturated fatty acid percentage in neutral lipids of CF children suggests the use of the latter percentage as index of the stage of malabsorption.
 ...
PMID:Influence of lyophilized total pancreas on plasma lipids and on fat absorption in cystic fibrosis. 61 69

A retrospective analysis of oral D-xylose tolerance tests in 435 pediatric patients was performed. A significant difference was found between 126 normal subjects and 47 untreated children with celiac disease one hour after load. The one-hour value was found to be more reliable than was fecal fat analysis in screening children for celiac disease. Sex, age, weight, and body surface did not influence the results of the one-hour value in the control subjects. Forty-eight children with cystic fibrosis had one-hour xylose levels within the normal range, but the means at 90, 120, and 180 minutes after load exceeded significantly (P less than 0.01) those of the controls. In a nonceliac group of 63 children with abnormally low xylose levels, almost all were found to have clinical conditions compatible with upper small bowel mucosal impairment. This study shows that a single estimation of xylose in blood one hour after load is a reliable index of small bowel mucosal function. It is a good screening test for celiac disease and small bowel disorders producing malabsorption in children.
 ...
PMID:One-hour blood xylose test: a reliable index of small bowel function. 64 21

43 gastroenterologically healthy infants and children as well as 45 patients with different malabsorption and maldigestion syndromes were investigated during the basic secretion and after injection of secretin and pancreozymin in order to establish the total quantity and also the distribution of the secreted bile acids. The total concentration and quantity were determined enzymatically; column and thinlayer chromatography were utilized to separate the bile into 30 different bile acids. While the total quantity of bile acids was found to be independent of age, the compounding of bile changed during the first years of life. Patients with coeliac disease reacted to injection of peptide hormones by producing a larger volume of secretion than did the control group. Despite the increased secretion there was at the same time a significantly higher concentration of bile acids in the duodenal juice. In this group the analysis of the bile acid distribution indicated no abnormality but striking changes were found in patients with cystic fibrosis, biliary atresia, M. Wilson, and in children after subtotal resection of the small bowel.
 ...
PMID:[Bile acids in the intestinal juice of infants and children with malabsorption and maldigestion syndromes]. 70 May 80

Patients with cystic fibrosis have fat malabsorption, providing an experimental model for evaluation of the hypothesis that a low-fat intake may prevent atherosclerosis. We studied the frequency and extent of aortic precursor lesions (fatty streaks, early fibromusculoelastic lesions, late fibromusculoelastic lesions) found at autopsy in this disease as well as in other patients with debilitating disorders but with no apparent impairment of fat absorption. Fatty streaks were less common in the cystic fibrosis group, as were the late fibromusculoelastic lesions. There was no significant difference in the frequency, length, or thickness of the early fibromusculoelastic lesions. The findings suggest that fat may be responsible for progression but not initiation of the fibromusculoelastic precursor lesions, and support the concept that early restriction of dietary fat may prevent, delay, or otherwise modify atherosclerosis in the adult.
 ...
PMID:Preatherosclerotic aortic lesions in cystic fibrosis. 75 18

Vitamin D and mineral metabolism were studied in 21 adolescents and young adults with cystic fibrosis and the results were compared to those in 21 matched controls. All CF patients had been maintained on standard multivitamin supplements in combination with pancreatic enzyme replacement. Despite this supplementation, relative to control subjects the CF patients had a 36% reduction in serum 25-hydroxyvitamin D concentration, a slight but significant reduction in serum calcium concentration, evidence of calcium malabsorption with secondary hyperparathyroidism, and a 14% decrease in bone mass measured by the photon absorption technique. Currently accepted modes of pancreatic enzyme replacement and vitamin D supplementation are often inadequate to maintain normal mineral homeostasis in CF patients; additional measurements may be required to reduce the risk of clinically significant osteopenia concomitant with prolonged survival in CF.
 ...
PMID:Reduced serum 25-hydroxyvitamin D concentration and disordered mineral metabolism in patients with cystic fibrosis. 75 19


1 2 3 4 5 6 7 8 9 10 Next >>