Pancreatic Function In Cystic Fibrosis Research Articles

Secretin-stimulated MRI assessment of exocrine pancreatic function in cystic fibrosis and healthy volunteers

Secretin-stimulated MRI assessment of exocrine pancreatic function in cystic fibrosis and healthy volunteers

237 A new endoscopic short test for exocrine pancreatic function in cystic fibrosis

237 A new endoscopic short test for exocrine pancreatic function in cystic fibrosis

239 Parenchymal and functional ultrasonographic imaging of the pancreas in cystic fibrosis correlates with exocrine pancreatic function

237 A new endoscopic short test for exocrine pancreatic function in cystic fibrosis T. Engjom1,2, B.N. Laerum3, F. Erchinger2,4, E. Tjora5,6, O.H. Gilja1,2,7, G. Dimcevski1,2. 1Haukeland University Hospital, Section for Gastroenterology, Bergen, Norway; 2University of Bergen, Institute of Medicine, Bergen, Norway; 3Haukeland University Hospital, Department of Thoracic Medicine, Bergen, Norway; 4Voss Hospital, Department of Medicine, Voss, Norway; 5Haukeland University Hospital, Department of Paediatry, Bergen, Norway; 6University of Bergen, Institute for Clinical Medicine, Bergen, Norway; 7Haukeland University Hospital, National Centre for Ultrasound in Gastroenterology, Bergen, Norway

238 Faecal calprotectin is increased in CF patients with pancreatic insufficiency

237 A new endoscopic short test for exocrine pancreatic function in cystic fibrosis T. Engjom1,2, B.N. Laerum3, F. Erchinger2,4, E. Tjora5,6, O.H. Gilja1,2,7, G. Dimcevski1,2. 1Haukeland University Hospital, Section for Gastroenterology, Bergen, Norway; 2University of Bergen, Institute of Medicine, Bergen, Norway; 3Haukeland University Hospital, Department of Thoracic Medicine, Bergen, Norway; 4Voss Hospital, Department of Medicine, Voss, Norway; 5Haukeland University Hospital, Department of Paediatry, Bergen, Norway; 6University of Bergen, Institute for Clinical Medicine, Bergen, Norway; 7Haukeland University Hospital, National Centre for Ultrasound in Gastroenterology, Bergen, Norway

Evaluating pancreatic function in cystic fibrosis

Evaluating pancreatic function in cystic fibrosis

Faecal elastase-1 test is superior to faecal lipase test in the assessment of exocrine pancreatic function in cystic fibrosis

Faecal elastase-1 test is superior to faecal lipase test in the assessment of exocrine pancreatic function in cystic fibrosis

Comparison of fecal lipase test and fecal elastase-1 test in the assessment of exocrine pancreatic function in cystic fibrosis

Comparison of fecal lipase test and fecal elastase-1 test in the assessment of exocrine pancreatic function in cystic fibrosis

Fecal elastase-1 cut-off levels in the assessment of exocrine pancreatic function in cystic fibrosis

Background: Fecal elastase-1 (E1) test is a sensitive and specific indirect test. However, there are few data on the best cut-off level in the assessment of exocrine pancreatic function in cystic fibrosis (CF). Material and methods: In 725 CF patients and 243 healthy subjects (HS) from Greece, Russia, Poland and the United Kingdom, E1 concentrations were measured. The best cut-off levels for the discrimination between CF and HS (for whole group as well as for individual countries) were calculated. Results: The best cut-off level for the differentiation between CF pancreatic insufficiency and normal pancreatic function in HS was found to be 184 μg/g of feces. However, some inter-country differences were stated. E1 concentrations in the UK subgroup were significantly lower than those found in Polish and Russian CF patients. E1 concentrations in Greek patients were significantly higher than in the other countries. However, E1 concentrations in ΔF508 homozygotes were very similar in all studied subgroups. In conclusion: In clinical practice, instead of a single best cut-off level for the E1 test, we suggest using a range of values (160–200 μg/g). The presence of different best cut-off levels within countries is a practical consequence of the different distribution of pancreatic function.

Analysis of exocrine pancreatic function in cystic fibrosis: one mild CFTR mutation does not exclude pancreatic insufficiency.

Cystic fibrosis (CF) is the most common cause of exocrine pancreatic insufficiency in childhood. The aim of the present study is to evaluate the correlation between genotype and exocrine pancreatic insufficiency in CF patients. The special emphasis was put on the analysis of mild CFTR mutations. The study comprised 394 CF patients and 105 healthy subjects (HS). Elastase-1 concentrations were measured in all subjects. Severe pancreatic insufficiency was associated with the presence of two CFTR gene mutations (DeltaF508, N1303K, CFTR dele 2,3 (21kb), G542X, 1717-1G-A, R533X, W1282X, 621GT, 2183AAG, R560T, 2184insA and DeltaI507, G551D, 895T) and mild insufficiency with the presence of at least one mutation (R117H, 3171insC, A155P2, 138insL, 296 + 1G-A, E92GK, E217G, 2789 + 5G-A. 3849 + 1kbC-T/3849 + 1kbC-T) genotype resulted in high elastase-1-values. However, in case of patients with genotype DeltaF508/3849 + 10kbC-T, 1717-1GA/3849 + 10kbC-T as well as with DeltaF508/R334W, both high and low elastase-1 concentrations were found. Low E1 values were found in a patient with DeltaF508/R347P genotype. Patients who carry two 'severe' mutations develop pancreatic insufficiency, whereas those who carry at least one 'mild' usually remain pancreatic sufficient. However, the presence of one mild mutation does not exclude pancreatic insufficiency.

Fecal elastase‐1 is decreased in villous atrophy regardless of the underlying disease

Fecal elastase-1 (E1) is a sensitive and reliable test in the assessment of exocrine pancreatic function in cystic fibrosis (CF). In patients with celiac disease (CD), different E1 values have been reported. E1 levels in other malabsorption conditions are unknown. Therefore, the aim of the present study was to evaluate E1 concentrations in various malabsorption syndromes. The study was carried out in 54 patients, selected from patients referred with suspicion of CF, who had been diagnosed as celiac disease (CD; n = 16), secondary malabsorption syndrome (SMS, giardiasis- or cow milk-related enteropathy; n = 18) and food allergy (FA; n = 20). 70 age-matched healthy children (HC) and 131 cystic fibrosis (CF) patients served as control groups. In CD and SMS patients, a gluten-free diet was introduced. In addition, SMS patients were treated appropriately to underlying disease. In all subjects, E1 concentrations were measured. In CD and SMS patients, E1 concentrations were repeatedly measured after one year of the treatment. With a cut-off level of 200 microg g-1, abnormal E1 concentrations were found in 87.2% of the CF group and in 56.2% and 50.0% of the CD and SMS subgroups, respectively. In none of FA patients, were E1 values below the normal range. After mucosal recovery, E1 concentrations in patients with CD and SMS increased, suggesting that villous atrophy can diminish exocrine pancreatic secretion. In 18 out of 19 CD and SMS patients with abnormal E1 concentrations, monitored for at least 12 months of a gluten-free diet, abnormal E1 concentrations increased above the cut-off value to normal range. Two out of the 54 referred patients were finally diagnosed as having CF, one with stable low E1 levels and the second with finally normal values. The exocrine pancreatic function is decreased in villous atrophy regardless of underlying disease. The specificity of the fecal elastase-1 test in the differentiation between 'primary' exocrine pancreatic insufficiency and intestinal malabsorption with mucosal atrophy is low. After mucosal regeneration, fecal elastase-1 specificity is high.

Analysis of exocrine pancreatic function in cystic fibrosis in relation to the genotype

Analysis of exocrine pancreatic function in cystic fibrosis in relation to the genotype

Fluorescein dilaurate test of exocrine pancreatic function in cystic fibrosis.

Fluorescein dilaurate test of exocrine pancreatic function in cystic fibrosis.

Fluorescein dilaurate test of exocrine pancreatic function in cystic fibrosis.

The fluorescein dilaurate test, a non-invasive test of exocrine pancreatic function, was carried out on 21 children with cystic fibrosis and pancreatic exocrine insufficiency, and 12 healthy siblings. The test clearly discriminated between the patients with cystic fibrosis and severe exocrine pancreatic insufficiency and the normal control subjects.

Evaluation of Tests of Exocrine and Endocrine Pancreatic in Function in Older Patients with Cystic Fibrosis

In order to determine the value of noninvasive tests in the analysis of pancreatic function in cystic fibrosis, 14 older cystic fibrosis patients were studied by a set of noninvasive tests of exocrine and endocrine pancreatic function. The tests, comprising trypsin, total amylase, pancreatic isoamylase, lipase, pancreatic polypeptide (PP), glucose and insulin in fasting serum, PP, glucose and insulin in postprandial serum, and p-aminobenzoic acid (PABA) excretion in urine, were compared to fecal fat excretion after discontinuation of pancreatic enzyme supplementation. Eleven of the 14 patients were found to have a fecal fat excretion of more than 7 g/day. Serum levels of trypsin, pancreatic isoamylase and lipase, and the urinary excretion of PABA showed significant negative correlations with fecal fat excretion. Endocrine pancreatic function was abnormal in the majority of patients with fibrocystic disease. Although serum trypsin, postprandial PP, and urinary PABA excretion were the most sensitive tests for severe exocrine pancreatic insufficiency, the differences in sensitivity were rather modest. Therefore, the type of test to be selected for clinical use is mainly dependent upon factors as accessibility, simplicity, patient's acceptability, and costs.

Familial concordance of pancreatic function in cystic fibrosis

Patients with cystic fibrosis have great variability in the clinical severity of lung disease and digestive problems. Ten to fifteen percent have sufficient endogenous production of pancreatic enzymes to allow normal growth and nutrition, without the need for oral pancreatic enzyme replacement therapy. Endogenous pancreatic enzyme secretion in these patients ranges from normal levels to only 1% to 2% of normal values, 1 indicating that a relatively low level of pancreatic enzyme secretion may be sufficient to avoid steatorrhea. Patients with CF and pancreatic sufficiency have less severe lung disease and a better overall prognosis than the 85% to 90% of patients who have pancreatic insufficiency requiring supplemental enzymes for digestion) With the recent development of relatively simple measures of pancreatic function, based on the level of pancreatic enzymes in serum, it has become easier to characterize the pancreatic function of CF patients? Clinical observation suggests that CF siblings in the same family tend to have a similar degree of pancreatic dysfunction. Because the patients with pancreatic sufficiency have a better prognosis, and because any familial trend may be related to variation of the CF gene, we examined the results of pancreatic function testing in families with more than one CF patient, to assess the degree of concordance within families. Close concordance within families may be of importance in genetic counseling and in understanding the pathogenesis of the disease.

Altered PABA pharmacokinetics in cystic fibrosis. Implications for bentiromide test.

The bentiromide test has been proposed as a useful noninvasive method for assessing exocrine pancreatic function in cystic fibrosis (CF) patients. Following oral administration, this peptide is selectively cleaved by pancreatic chymotrypsin liberating PABA which is passively absorbed. Recent studies have suggested that PABA measured in plasma is superior to the more established method of estimating urinary recovery of this marker. However, in using the plasma test in CF patients, one makes the assumption that the PABA marker has similar distribution and elimination patterns in normal and CF subjects. Since many drugs display altered pharmacokinetics in CF patients, we studied the disposition of PABA following ingestion of free PABA in six controls (age 19-28 years) and 18 CF patients (13-18 years; seven steatorrheic and 11 nonsteatorrheic). Elimination of T1/2 of PABA was significantly shorter in CF patients (58 +/- 21 min) compared to controls (93.5 +/- 28) (P less than 0.005). PABA clearance was similar in the control and CF patients (2.99 +/- 1.21 and 3.27 +/- 1.02 ml/min/kg, respectively). PABA distribution volume was smaller, although not significantly so, than in the controls (268 +/- 107 vs 376 +/- 140 ml/kg). Good correlation was found between PABA distribution volume and T1/2 (r = 0.51 P less than 0.02). Our simulation data suggest that altered pharmacokinetics of PABA in CF patients would cause their PABA levels to be 7% lower than controls at 90 min, 18% at 120 min, 29% at 150 min, and 38% at 180 min.(ABSTRACT TRUNCATED AT 250 WORDS)