PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “CYSTIC FIBROSIS OF PANCREAS”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,459 records · Page 81Linked to original sources
PubMed1998

Mild cystic fibrosis mutations in Southern Sweden with special reference to S549I and T338I.

In this study of cystic fibrosis (CF) gene mutations in Southern Sweden we found missense mutations in 12 out of 110 patients. These patients, as a group, differed from deltaF508 homozygotes by a higher frequency of pancreatic sufficiency and an older age at diagnosis as has been indicated in previous studies. In addition, lung function (vital capacity (VC) and forced expiratory volume in 1 s (FEV1)) tended to be better although the difference did not reach statistical significance (p = 0.13 for FEV1). For two mutations, S549I and T338I, our results differed from earlier reports. In our experience, S549I confers a milder phenotype and T338I a more severe one than previously reported. We conclude that each mutation should be treated separately when trying to correlate genotype with phenotype.

Adolescent↗
PubMed2004

Evaluation of the 13C-triolein breath test for fat malabsorption in adult patients with cystic fibrosis.

BACKGROUND AND AIMS: A simple non-invasive test not requiring the use of radioactive isotopes is required to assess fat malabsorption in adult cystic fibrosis (CF) patients. Breath tests using substrates labeled with 13C meet these conditions. The 14C-triolein breath test is sensitive and specific for measuring fat malabsorption, but involves radiation exposure. The aim of this study was to examine the utility of a test using a 13C label and to determine whether pancreatic replacement therapy would return the test to the values of a normal control group. METHODS: 13CO2 recovery was assessed after an overnight fast in six adult patients with CF, both with and without pancreatic enzyme replacement therapy (PERT) in the usual dose for a light snack, in a randomized order, on different days. Studies were also performed in eight healthy volunteers after oral ingestion. Subjects drank 50 mL of a liquid meal mixed with 200 microL 13C-triolein and breath samples were collected by blowing through a straw into collection tubes every 30 min for 6 h. The effect of gastric emptying was assessed by comparison of oral ingestion with intraduodenal infusion. Intra-individual variability was assessed in nine volunteers by repeating the breath test after drinking the test meal on a separate day. RESULTS: Compared with healthy subjects there was virtually no recovery of 13CO2 in CF patients without PERT. The median (interquartile range) cumulative percentage dose recovery (cPDR) at 6 h was 3% (0-8) in CF patients compared with 28% (22-41) in healthy controls (P < 0.01). Fat absorption was normalized (37%) (36-43) after ingestion of PERT. Gastric emptying delayed the peak in 13CO2 recovery, but there was no difference in the cPDR at 6 h. There was no difference in recovery between days 1 and 2. CONCLUSIONS: The 13C-triolein breath test is a simple and reproducible method to measure fat malabsorption. The test provides a screening technique for fat malabsorption in adult CF patients and may be useful for monitoring the lowest effective dose of PERT.

Administration, Oral↗
PubMed1984

Effect of intrajejunal acidity on aqueous phase bile acid and lipid concentrations in pancreatic steatorrhoea due to cystic fibrosis.

We have investigated whether jejunal hyperacidity leads to bile acid precipitation and thus limits lipid solubilisation in patients with pancreatic steatorrhoea. Jejunal contents from 12 adults with steatorrhoea due to cystic fibrosis were aspirated for three hours after a liquid test meal, and pooled according to their pH. Thirty eight per cent of the total aspirate was collected at pH less than 5 in cystic fibrosis, compared with 18% in healthy controls (p less than 0.05). Forty six per cent of the bile acids were precipitated at pH less than 5, compared with 15% at pH greater than 6 (p less than 0.01), leading to reduced aqueous phase bile acid concentration at low pH (4.7 mmol/l at pH less than 5 vs 12.5 mmol/l at pH greater than 6, p less than 0.01). Aqueous phase lipid concentrations were reduced at low pH (5.6 mmol/l at pH less than 5 vs 10.2 mmol/l at pH greater than 6, p less than 0.01). Lipolysis and total fatty acid concentrations were greatly reduced and did not vary with pH. We therefore conclude that jejunal hyperacidity leads to bile acid precipitation in pancreatic steatorrhoea due to cystic fibrosis, and imposes a further limitation on lipid solubilisation over that of lipase deficiency.

Adult↗
PubMed1985

Effect of cimetidine on enzyme inactivation, bile acid precipitation, and lipid solubilisation in pancreatic steatorrhoea due to cystic fibrosis.

In pancreatic steatorrhoea, both pH-dependent bile acid precipitation and enzyme inactivation may limit the efficacy of pancreatic enzyme supplements and both may be preventable by addition of cimetidine. To separate these effects we compared postprandial jejunal aspirate from eight adults with steatorrhoea due to cystic fibrosis on three randomised treatment regimens (pancreatin, cimetidine, and both together). We also compared the results with those of previous studies of patients on no treatment, and of healthy subjects. On pancreatin 60% of the test meal entered the jejunum at pH less than 5 compared with 17% in health. Lipase concentration and lipolysis increased over the values on no treatment (14.2 vs 4.4 U/l, p less than 0.01; 16% vs 11%, p less than 0.02) but bile acid precipitation was not reduced (38% vs 27%, NS), and aqueous-phase lipid concentration decreased (6.7 vs 8.6 mM/l, p less than 0.05). On cimetidine, bile acid precipitation fell (19% vs 38%, p less than 0.05); although lipase concentration and lipolysis were lower than on pancreatin (4.8 U/l vs 14.2 U/l, p less than 0.01; 9% vs 16%, p less than 0.01) lipid solubilisation increased (8.8 vs 6.7 mM/l, p less than 0.05). On the combination, there was a marked improvement (p less than 0.02) in lipid solubilisation (18.3 mM/l), reflecting the improvement both in lipase (38.4 U/l) and lipolysis (24%), and in bile acid precipitation (5.6%). We conclude that the efficacy of pancreatin is limited by pH-dependent bile acid precipitation in addition to enzyme inactivation. The action of cimetidine in improving the efficacy of pancreatin depends on prevention of both these effects.

Adolescent↗
PubMed1996

Health perceptions and treatment adherence in adults with cystic fibrosis.

BACKGROUND: Clinical and demographic variables are poor predictors of treatment adherence. This study therefore examined the relationships between the patients' perception of their cystic fibrosis and their reported adherence to physiotherapy, exercise, pancreatic enzyme and vitamin therapies. METHODS: Sixty adults with cystic fibrosis completed the Health Perception Scale, Health Locus of Control Scale, and the Manchester Adult Cystic Fibrosis Compliance Questionnaire. RESULTS: Reported adherence to physiotherapy, exercise, pancreatic enzyme and vitamin therapies was not influenced by patients' perceptions of their past, current and future disease severity, or their perceived susceptibility to recurrent infections. The greater their level of worry regarding their disease, the more likely they were to adhere to their physiotherapy (p < 0.002), pancreatic enzyme (p < 0.001) and vitamin (p < 0.001) regimens. No difference between the exercise adherent and non-adherent groups emerged regarding their level of worry. Compared with patients who believed that they were in control of their health, those who believed that their cystic fibrosis was controlled by others (family and health professionals) reported greater levels of adherence to their physiotherapy (p < 0.001), pancreatic enzyme (p < 0.001) and vitamin (p < 0.001) therapies. In contrast, patients who adhered to their exercise regimen believed that they were in control of their disease to a greater extent than those who did not (p < 0.003). CONCLUSIONS: Worrying about cystic fibrosis and the perception of having little personal control over the disease facilitated treatment adherence. It is clear that patients perceive exercise therapy differently from other forms of treatment.

Adolescent↗
PubMed1995

CPX, a selective A1-adenosine-receptor antagonist, regulates intracellular pH in cystic fibrosis cells.

The selective A1-adenosine-receptor antagonist, 8-cyclopentyl-1,3-dipropylxanthine (CPX), has been reported to activate Cl- efflux from cystic fibrosis cells, such as pancreatic CFPAC-1 and lung IB3 cells bearing the cystic fibrosis transmembrane regulator(delta F508) mutation, but has little effect on the same process in cells repaired by transfection with wild-type cystic fibrosis transmembrane regulator (O. Eidelman, C. Guay-Broder, P. J. M. van Galen, K. A. Jacobson, C. Fox, R. J. Turner, Z. I. Cabantchik, and H. B. Pollard. Proc. Natl. Acad. Sci. USA 89: 5562-5566, 1992). We report here that CPX downregulates Na+/H+ exchange activity in CFPAC-1 cells but has a much smaller effect on cells repaired with the wild-type gene. CPX also mildly decreases resting intracellular pH. In CFPAC-1 cells, this downregulation is dependent on the presence of adenosine, since pretreatment of the cells with adenosine deaminase blocks the CPX effect. We also show that, by contrast, CPX action on these cells does not lead to alterations in intracellular free Ca2+ concentration. We conclude that CPX affects pH regulation in CFPAC-1 cells, probably by antagonizing the tonic action of endogenous adenosine.

Calcium↗
PubMed1984

Carbohydrate tolerance in cystic fibrosis is closely linked to pancreatic exocrine function.

We evaluated carbohydrate tolerance in nine thin cystic fibrosis (CF) patients and in six controls, measuring responsiveness to the following insulinotropic secretagogues: oral glucose, IV glucose, and IV tolbutamide. Glucose responses segregated patients into two groups: Group I with normal carbohydrate tolerance associated with normal to slightly increased insulin responses, and Group II with impaired carbohydrate tolerance associated with insulinopenia. This latter group included one patient with frank diabetes. The CF patients demonstrated a significant positive correlation between insulin secretion, in response to each secretagogue, and pancreatic exocrine function as measured by serum pancreatic amylase isoenzyme concentration. Pancreatic alpha-cell function, as reflected by basal plasma glucagon concentrations, also correlated well with exocrine function in the CF patients, excluding the diabetic individual. The enteroinsular axis of the CF group was intact as reflected by normal plasma gastric inhibitory polypeptide concentrations in Group I and by elevated levels, basally and in response to oral glucose, in the insulinopenic Group II patients. Furthermore, those patients with impaired tolerance demonstrated a greater magnitude of insulinopenia compared to controls following IV glucose and possibly IV tolbutamide, than following oral glucose. Thus, these data suggest that loss of carbohydrate tolerance in patients with CF, like that seen with classical chronic pancreatitis, 1) parallels the loss of exocrine function, 2) is associated with appropriate enteroinsular signaling, and 3) can be detected earlier or more easily following testing with direct IV secretagogues than following oral glucose stimulation.

Adult↗
PubMed2004

Lung function decline in cystic fibrosis patients and timing for lung transplantation referral.

STUDY OBJECTIVES: To determine risk factors associated with an accelerated decline in lung function in cystic fibrosis (CF), and whether longitudinal changes in FEV(1) would be a better predictor of the need for referral for lung transplantation than any single value for FEV(1.) DESIGN: The rate of decline in pulmonary function was determined by standard linear regression from each patient's calendar year's best percentage of predicted FEV(1) (%FEV(1)) over at least 4 years, and patients were classified into three cohorts based on their rate of decline. Differences between groups in age, weight-for-age z score, gender, genotype, pancreatic status, diabetes, and the presence of various lung microbial isolates were analyzed. A subset of 30 patients referred for lung transplantation were further analyzed, and a prediction model for lung transplantation referral was created using the patient's rate of decline in lung function, the mean waiting time for donor organs, and the average level of lung function of patients prior to lung transplantation. PATIENTS: One hundred fifty-three patients with CF followed up at the Washington University Adult Cystic Fibrosis Center. RESULTS: Younger age, malnutrition, and concurrent infection with both Pseudomonas aeruginosa and Staphylococcus aureus were significant (p < 0.05) risk factors for rapidly declining lung function. Among patients with rapidly declining lung function, referral for lung transplantation would have occurred 8.4 months earlier than actual referral age (p < 0.05) if the prediction model had been used, possibly resulting in additional patient salvage in several cases. CONCLUSIONS: Rate of decline in lung function should be routinely evaluated in patients with CF, and a prediction model utilizing the rate of decline in %FEV(1), and the median regional waiting period for donor lungs for patients with CF may assist in the timing of referral for lung transplantation and more rapidly declining lung function.

Adult↗
PubMedDate not supplied

Small intestinal brush border enzymes in cystic fibrosis.

The study concerns the maltase, saccharase, lactase and alkaline phosphatase activity in small intestinal biopsy specimens from 61 consecutively admitted, untreated, Caucasian cystic fibrosis patients. A group of 319 age matched controls admitted during the same time period for undefined gastrointestinal or nutritional disorders acted as the controls. In order to eliminate morphological damage as a confounding factor, the enzyme activities were studied in small intestinal biopsy specimens having both normal stereomicroscopic and histological features. It was shown that neither maltase nor saccharase activity was different in the two groups, in contrast to lactase and alkaline phophatase activity, that was significantly lower in cystic fibrosis patients. The differences could not be explained by the nutritional status as judged by the body mass index. Lactase activity is known to be easily affected by numerous enteropathies. As the information on alkaline phosphatase activity is limited, the low activity is discussed in more detail. Taking into account the literature data, the low alkaline phosphatase activity is tentatively attributed either to enhanced release from the brush border or to the faulty handling of alkaline phophatase protein in the post-golgi compartments secondary to the accumulation of incorrectly glycosylated CFTR in the same cell structures.

Alkaline Phosphatase↗
PubMed2004

[Dynamics of markers of blood inflammation against the background of antibacterial therapy in children with mucoviscidosis, with the various regimens of intake of pancreatic enzymes].

The results of the studies show that patients with mucoviscidosis diagnosed for the first time who do not receive any adequate replacement pancreatic therapy should not be prescribed high doses of pancreatic enzymes along with antibacterial therapy when they are hospitalized on account of exacerbation of the bronchopulmonary process. Sharply increased lipid absorption can cause an aggravation of oxidative stress and impair the patient's state instead of improving it. In our opinion, such patients should be prescribed delayed adequate pancreatic therapy with immediate introduction of high doses of antioxidants (vitamins E, A, C, beta-carotene, etc.). There is no doubt that it is necessary to conduct further research into the problem for a greater number of patients.

Adolescent↗
PubMed2005

Genetic factors in pancreatitis.

The understanding of pathogenesis of acute and chronic pancreatitis has benefited from the progress made in genetic investigations. The discoveries of the gain of function mutations of cationic trypsinogen gene (PRSS1) and the loss of function mutations of pancreatic secretory trypsin inhibitor (SPINK 1) or other potential defects in genes that regulate pancreatic secretory function or modulate inflammatory response to pancreatic injury has changed our current concepts on the pathogenesis of pancreatitis. Genetic factors play an important role in the susceptibility to pancreatic injury, severity and evolution of inflammatory process, leading in some cases to chronic inflammation and/or fibrosis. Acute pancreatitis is viewed as an event and chronic pancreatitis as a process, sequentially linked, reflecting a complex interaction between genetic and environmental factors.

Carrier Proteins↗
PubMed1986

Serum immunoreactive pancreatic lipase and cationic trypsinogen for the assessment of exocrine pancreatic function in older patients with cystic fibrosis.

Indirect and qualitative tests of pancreatic function are commonly used to screen patients with cystic fibrosis for pancreatic insufficiency. In an attempt to develop a more quantitative assessment, we compared the usefulness of measuring serum pancreatic lipase using a newly developed enzyme-linked immunosorbent immunoassay with that of cationic trypsinogen using a radioimmunoassay in the assessment of exocrine pancreatic function in patients with cystic fibrosis. Previously, we have shown neither lipase nor trypsinogen to be of use in assessing pancreatic function prior to 5 years of age because the majority of patients with cystic fibrosis in early infancy have elevated serum levels regardless of pancreatic function. Therefore, we studied 77 patients with cystic fibrosis older than 5 years of age, 41 with steatorrhea and 36 without steatorrhea. In addition, 28 of 77 patients consented to undergo a quantitative pancreatic stimulation test. There was a significant difference between the steatorrheic and nonsteatorrheic patients with the steatorrheic group having lower lipase and trypsinogen values than the nonsteatorrheic group (P less than .001). Sensitivities and specificities in detecting steatorrhea were 95% and 86%, respectively, for lipase and 93% and 92%, respectively, for trypsinogen. No correlations were found between the serum levels of lipase and trypsinogen and their respective duodenal concentrations because of abnormally high serum levels of both enzymes found in some nonsteatorrheic patients. We conclude from this study that both serum lipase and trypsinogen levels accurately detect steatorrhea in patients with cystic fibrosis who are older than 5 years but are imprecise indicators of specific pancreatic exocrine function above the level needed for normal fat absorption.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1979

Cystic fibrosis in adults: an autopsy study.

An annotated catalog of clinical and pathologic observations in an adult population with CF has been presented. The spectrum of disease is broad, and the diagnosis usually requires the demonstration of abnormalities in multiple systems. Tissues of each germ layer have been found involved in CF. Correlation of clinical and pathologic observations suggests that manifestations are essentially episodic and focal. In this population, the most characteristic abnormalities recognized by the pathologist are focal biliary cirrhosis, distinctive obstructive lesions of the male genital tract, prolonged staphylococcal or pseudomonas colonization of respiratory secretions, and obstructive bronchopulmonary disease. Some form of pancreatic atrophy is usually present.

Adult↗