Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0432222 (SEM)
 47,337 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Using radioimmunoassay, immunoreactive SP (iSP) has been measured in the plasma of 162 hospital patients with various disorders and in the plasma of 67 pregnant women. In pregnancy, iSP was undetectable in 40% women as compared to 12% in other hospital patients. The mean iSP plasma level in pregnancy was 37.8 +/- 4.6 (SEM) as compared to 77.1 +/- 4.9 (SEM) pg/ml in other hospital patients. The results support earlier observations based on bioassay, suggesting that the blood of pregnant women contains higher concentrations of a SP-inactivating factor. Of the hospital patients, elevated levels of iSP were found in patients with chronic leukaemia, in one patient with a basaloid carcinoma of the anus, and in one patient with toxic liver damage and pancreatic insufficiency. No correlation was found between thyroid function and iSP plasma levels. ISP plasma levels in various gastrointestinal disorders were similar to those found in normal subjects.
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PMID:Substance P plasma levels in pregnancy and in various clinical disorders. 47 37

To evaluate the impact of early pancreatic insufficiency on growth and nutritional status in cystic fibrosis, we studied 49 infants identified by a newborn screening program. Pancreatic insufficiency, determined by increased 72-hour fecal fat excretion, was present in 59% (23/39) of infants at diagnosis (7.0 +/- 0.8 weeks; mean +/- SEM). Before initiation of pancreatic enzyme replacement, growth and nutritional status of pancreatic-insufficient (n = 16) and pancreatic-sufficient (n = 13) infants were compared. Pancreatic-insufficient infants gained less weight from birth to diagnosis (13.4 +/- 3.4 vs 22.3 +/- 4.0 gm/day; p = 0.05), had decreased triceps skin-fold thicknesses (4.5 +/- 0.3 vs 6.1 +/- 0.4 mm; p less than 0.005), and had lower blood urea nitrogen (3.07 +/- 0.42 vs 4.62 +/- 0.65 mg/dl; p = 0.02) and albumin (2.99 +/- 0.14 vs 3.54 +/- 0.14 gm/dl; p less than 0.01) levels despite higher gross calorie (154 +/- 8 vs 116 +/- 13 kcal/kg per day; p less than 0.01) and protein intakes (2.81 +/- 0.21 vs 2.14 +/- 0.33 gm/kg per day; p = 0.03). Fecal nitrogen loss was correlated with fat loss (r = 0.79; p less than 0.001). Fat malabsorption was present in 79% (30/38) and 92% (33/36) of infants tested at 6 months and 12 months of age, respectively, indicating that pancreatic insufficiency persists and increases in frequency throughout infancy. We conclude that pancreatic insufficiency is prevalent in young infants with cystic fibrosis and has a significant impact on growth and nutrition.
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PMID:Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis. 155 90

The effects of exocrine pancreatic insufficiency on the small intestinal mucosa were examined in dogs following pancreatic duct ligation. There were no significant changes either in villus architecture or enterocyte height after duct ligation, but numbers of bacteria in duodenal juice increased then subsequently decreased following treatment with exogenous pancreatic enzymes. Pancreatic insufficiency resulted in a considerable increase in the proportion of microvillar membrane proteins of molecular mass over 200 kDa from 3.3 +/- 4 per cent (mean +/- SEM) to 13.6 +/- 7.2 per cent, and this decreased to 6.9 +/- 5.2 per cent following pancreatic enzyme supplementation. However, anticipated increases in activities of maltase and sucrase were not observed following duct ligation, and there was a reduction in lactase activity which was reversed by pancreatic supplementation. Activities of marker enzymes for the other subcellular organelles showed relatively minor or no changes throughout the study. These findings are consistent with a specific role for pancreatic enzymes in the post-translational processing of intestinal microvillar membrane proteins, and suggest that reduced degradation of brush border proteins in the absence of pancreatic secretions may be masked by quantitative and qualitative changes in the intestinal microflora.
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PMID:Biochemical changes in the jejunal mucosa of dogs with exocrine pancreatic insufficiency following pancreatic duct ligation. 259 94

To investigate the role of energy expenditure in the altered energy balance in cystic fibrosis (CF), total energy expenditure (TEE) was measured by the doubly-labelled water method in 9 clinically well CF infants (body weight 7.3-10.9 kg) without chronic lung disease. CF infants had 25% higher rates of energy expenditure when compared with data derived from measurements of TEE obtained by the same method in 16 healthy infants, matched for age and body weight. Mean TEE (SEM) for CF was 950 (38) kcal, vs 876 (72) kcal for controls matched for age and 758 (46) kcal for controls matched for weight. Although subclinical disease activity cannot be excluded as a determinant of the excess TEE, the possibility of an energy-requiring basic defect is suggested, because further analysis indicated that factors other than body weight, degree of underweight, presence of pancreatic insufficiency, or presence of lung disease were important. Increased TEE may contribute to undernutrition in CF, even in the absence of chronic lung disease.
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PMID:Increased energy expenditure in young children with cystic fibrosis. 289 57

Many approaches have been proposed to differentiate between steatorrhea due to pancreatic insufficiency and intestinal disease. Bo-Linn and Fordtran recently suggested that fecal fat concentration (FFC) is a useful screening test for this distinction. Our aim was to validate their result in a large group of patients. Fecal fat concentrations were calculated for 613 fecal fat tests in 538 patients. Included were 88 patients with pancreatic steatorrhea (13 pancreatic carcinoma, 6 cystic fibrosis, and 69 chronic pancreatitis) and 525 with nonpancreatic steatorrhea. The mean FFC of patients with pancreatic disease (15.0 +/- 1.9 g%, mean +/- SEM) was significantly higher than that of patients with other diseases causing malabsorption (8.9 +/- 0.3 g%, p less than 0.001). Forty-two percent of patients with pancreatic steatorrhea had an FFC below 10 g%. The overlapping of the FFC of steatorrhea due to pancreatic disease and that produced by celiac disease, gastric resection, and other conditions suggests that this approach does not differentiate between pancreatic and intestinal steatorrhea.
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PMID:Fecal fat concentration in the differential diagnosis of steatorrhea. 291 27

Dietary supplementation with fish oils high in the omega-3 fatty acids, eicosapentaenoic acid and docosahexaenoic acid, may have an antiinflammatory effect. We determined whether patients with cystic fibrosis (CF) could incorporate omega-3 fatty acids into their plasma and cell membrane phospholipids without adverse effects. In this double-blind study, 12 patients with pancreatic insufficiency who have CF (mean age, 12.2 +/- 5.4 (SD) years) and 13 subjects without CF (mean age, 13.4 +/- 6.3 (SD) years) were randomly assigned to ingest 8 gm daily of either encapsulated fish oil (3.2 gm of eicosapentaenoic acid and 2.2 gm of docosahexaenoic acid daily) or olive oil ethyl esters for 6 weeks. Two of seven and two of five patients with CF who received fish and olive oils, respectively, and one of eight and none of five subjects without CF discontinued taking the capsules before 6 weeks because of eructation or diarrhea. Significant incorporation of omega-3 fatty acids into plasma and erythrocyte membrane phospholipids was observed in subjects with and those without CF randomly assigned to the fish oil treatment. For example, in subjects randomly assigned to receive fish oil, the eicosapentaenoic acid/arachidonic acid ratio in plasma increased 9.8-fold, from 0.04 +/- 0.02 (mean +/- SEM) to 0.39 +/- 0.11 (p = 0.02), in the patients with CF (n = 7) and 23.0-fold, from 0.04 +/- 0.01 to 0.92 +/- 0.17 (p = 0.001), in the subjects without CF (n = 8) who received fish oil (p = 0.02, patients with CF vs subjects without CF at 6 weeks). No clinically or statistically significant changes from baseline were observed in platelet aggregation or levels of vitamin E or A in subjects who received fish oil. Future studies are indicated to determine whether omega-3 fatty acid enrichment provides a clinically beneficial antiinflammatory effect in patients with CF.
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PMID:Oral absorption of omega-3 fatty acids in patients with cystic fibrosis who have pancreatic insufficiency and in healthy control subjects. 807 70

The magnitude of complex carbohydrate malabsorption in exocrine pancreatic insufficiency has not been well quantified in the past. The quantity of carbohydrate malabsorbed after a rice starch (100 g) meal in 20 patients with chronic pancreatitis (n = 10) or pancreatic cancer (n = 10) was therefore estimated. Patients had a three day stool fat collection (80 g/24 hour fat intake), a lactulose (20 g), and a rice flour (100 g) breath hydrogen test. Normal controls (n = 29) had a postprandial H2 increase < or = 14 ppm and malabsorbed (mean (SEM)) 1.12 (0.44) (range 0-11.10) g of the 100 g of carbohydrate ingested. Patients malabsorbed significantly more carbohydrate (11.36 (2.23) (range 8.90-32.60) g, F1.47 = 29.92, p < 0.001). The number of patients with fat (> 7 g, n = 8) or carbohydrate (increase in H2 > or = 20 ppm, n = 10) malabsorption was not different (chi 2 = 0.10, p = 0.75). There was a significant correlation between faecal fat and amount of malabsorbed carbohydrate (r = 0.60, F1.17 = 9.70, p = 0.006) and faecal fat and stool wet weight (r = 0.57, F1.18 = 8.67, p < 0.009), but not between stool wet weight and amount of malabsorbed carbohydrate (r = 0.28, F1.17 = 1.45, p = 0.25). Although patients with exocrine pancreatic insufficiency malabsorb 10%-30% of the ingested complex carbohydrate, the main determinant of stool wet weight could be faecal fat.
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PMID:Complex carbohydrate malabsorption in exocrine pancreatic insufficiency. 834 88

We describe a tubeless test of exocrine pancreatic function based on a new dual isotope technique, using N-benzoyl-L-tyrosyl-p-aminobenzoic acid (NBT-PABA) as a substrate for intestinal chymotrypsin activity and the stable isotope, 13C-PABA as marker. Gas chromatography-mass spectrometry (GC-MS) was used for the quantification of PABA and 13C-PABA in blood. The method involves hydrolysis, extractions, separation by HPLC, and methyl ester formation of the test substances before GC-MS analysis. The test is precise and shows good separation of healthy volunteers from patients with pancreatic insufficiency. The PABA/13C-PABA ratios in serum after 1.5 h were 2.64 +/- 0.14 (mean +/- SEM) in 10 healthy volunteers and 1.26 +/- 0.22 in 10 patients with exocrine pancreatic insufficiency. We present a sensitive and specific assay, which is free of analytical interference and radiation hazards and, additionally, it illuminates extrapancreatic pharmacokinetic conditions. This test can eliminate the need for duodenal intubation, which makes it very acceptable to the patients.
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PMID:Determination of the exocrine pancreatic function with the NBT-PABA test using a novel dual isotope technique and gas chromatography-mass spectrometry. 920 Feb 75

The case records of 11 patients with cystic fibrosis (CF) who had 13 completed pregnancies between 1975 and 1995 were retrospectively reviewed to assess: (1) the changes in spirometry and body mass index (BMI) during pregnancy; and (2) maternal and neonatal complications and outcomes. Prepregnancy the mean age of the group was 24 (range 17-27) years. Two patients were exsmokers, 7 had pancreatic insufficiency and 7 had chest X-ray evidence of bronchiectasis. None of the patients had diabetes mellitus but 3 developed gestational diabetes. The mean +/- SEM (% predicted) forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) prepregnancy were 2.3 +/- 1.0 (83%) litres and 3.0 +/- 0.9 (85%) litres respectively. Five patients had normal spirometry (FEV1 and FVC >80% predicted) prior to 6 pregnancies. The mean body mass index (kg/height(m)2) for the group was 20.5 +/- 2.0. There was a significant decline in spirometry during pregnancy (FEV1 15.5 +/- 6.6% p<0.01; FVC 14.0 +/- 8.3% p<0.5). However, FVC but not FEV1 recovered to prepregnancy values by 12 months postpartum. There was a significant increase in both weight (7.1 kg) and BMI (2.6 kg/height(m)2) at the time of delivery compared with prepregnancy (p=0.0004). However, postpregnancy both weight and BMI had returned to their prepregnancy values (p<0.2). Mothers with an FEV1>80% had less decline in FEV1 related to pregnancy, better outcomes, fewer operative and instrumental deliveries, fewer preterm infants and fewer neonatal complications. Suggestions for the planning and management of pregnancy in women with CF are discussed.
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PMID:Cystic fibrosis and pregnancy. 965 56

Many patients with chronic pancreatitis (CP) complain of several types of food intolerance despite elimination of fat and alcohol. Since there are no data on serum immunoglobulin E (IgE) concentrations in CP, IgE concentrations in serum were detected in 97 persons with CP and 50 controls. IgE was analyzed by the use of a highly sensitive fluoro-enzyme-immunoassay. In CP, a significantly raised IgE level (mean +/- SEM; 286.1 +/- 49 KU/L; p < 0.0001) was detected compared with controls (65.2 +/- 13 KU/L). CP-patients without alcohol consumption and normal exocrine pancreatic function were found to have only slightly elevated serum IgE values (120.2 +/- 54 KU/L), whereas patients with exocrine insufficiency treated with enzyme supplementation showed an IgE level of 153.7 +/- 51 and exocrine insufficient patients without treatment of 261.0 +/- 173 KU/L (p = 0.01). IgE levels were far more elevated in the corresponding groups with continued alcohol consumption (> 25 g/day). Alcohol consuming patients with CP and normal pancreatic function had a mean serum IgE of 295.0 +/- 114 KU/L, while patients with alcohol consumption and sufficiently treated exocrine pancreatic insufficiency showed a serum IgE of 393.7 +/- 147 KU/L (p = 0.03). Non-enzyme supplemented patients with CP and exocrine pancreatic insufficiency were characterized by approximately 10-fold increased serum IgE (1080.0 +/- 313 KU/L; p = 0.001). Non-allergic, alcohol consuming patients with CP have significantly increased serum IgE values. Since patients without alcohol consumption and normal pancreatic function or sufficiently treated exocrine insufficiency showed clearly lower IgE values than non-compliant patients with manifest exocrine pancreatic insufficiency, these results are compatible with the assumption that a reduced rate of antigen digestion in exocrine pancreatic insufficiency may lead to an increased intestinal antigen load, stimulating an abnormal humoral immune response with IgE production. Alcohol may further contribute to this by damaging the mucosal barrier.
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PMID:[Stimulation of immunoglobulin E formation in chronic pancreatitis by alcohol drinking and exocrine pancreatic insufficiency]. 1136 75


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