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PubMed2002

Lung infections associated with cystic fibrosis.

While originally characterized as a collection of related syndromes, cystic fibrosis (CF) is now recognized as a single disease whose diverse symptoms stem from the wide tissue distribution of the gene product that is defective in CF, the ion channel and regulator, cystic fibrosis transmembrane conductance regulator (CFTR). Defective CFTR protein impacts the function of the pancreas and alters the consistency of mucosal secretions. The latter of these effects probably plays an important role in the defective resistance of CF patients to many pathogens. As the modalities of CF research have changed over the decades from empirical histological studies to include biophysical measurements of CFTR function, the clinical management of this disease has similarly evolved to effectively address the ever-changing spectrum of CF-related infectious diseases. These factors have led to the successful management of many CF-related infections with the notable exception of chronic lung infection with the gram-negative bacterium Pseudomonas aeruginosa. The virulence of P. aeruginosa stems from multiple bacterial attributes, including antibiotic resistance, the ability to utilize quorum-sensing signals to form biofilms, the destructive potential of a multitude of its microbial toxins, and the ability to acquire a mucoid phenotype, which renders this microbe resistant to both the innate and acquired immunologic defenses of the host.

Bacterial Infections↗
PubMed2001

Uroguanylin and guanylate cyclase C in the human pancreas: expression and mutuality of ligand/receptor localization as indicators of intercellular paracrine signaling pathways.

The intestinal peptide hormone uroguanylin regulates electrolyte/fluid transport in the gastrointestinal epithelium by binding to its receptor, guanylate cyclase C (GC-C), and thus specifically coupling to activation of cystic fibrosis transmembrane conductance regulator (CFTR). Since CFTR is crucially involved in pancreatic electrolyte secretion, we investigated the human pancreas for expression and cell-specific localization of uroguanylin and guanylate cyclase C as potential regulatory components of pancreatic electrolyte secretion. RT-PCR analyses with specific primers revealed that uroguanylin and GC-C are expressed in the human pancreas (and in the duodenum, used as positive control); at the translational level, western blotting analyses with peptide- and region-specific antibodies identified the presence of 12.5 kDa uroguanylin and 130 kDa GC-C in both human pancreatic and intestinal extracts. At the cellular level, uroguanylin and GC-C immunoreactivities were absent from the islets of Langerhans but were exclusively confined to the exocrine parenchyma. Hence, uroguanylin was localized to the centroacinar cells typical of the pancreas, and also to epithelial cells of the intercalated, intralobular and interlobular ducts where the peptide was primarily concentrated adluminally to the apical portion of the respective cells. Coincidently, correlative studies localized the GC-C receptor to the epithelial cells of the ductal network, where it was confined exclusively to the apical cell membrane that evidently represents the functionally relevant target membrane domain for the regulatory peptide. In view of the fact that CFTR is highly expressed in pancreatic ductal cells where uroguanylin and its receptor are also localized, we assume that uroguanylin, an intrinsic pancreatic peptide, is involved in the regulation of electrolyte/water secretion in the ductal system via GC-C and CFTR. The particular cellular expression of uroguanylin in duct cells and the localization of GC-C to the duct cell apical membrane domain predict a novel route of intercellular signaling and luminal activation of GC-C via the pancreatic juice.

Duodenum↗
PubMed1984

Cystic fibrosis serum pancreatic amylase. Useful discriminator of exocrine function.

To develop a simple test for pancreatic exocrine function in patients with cystic fibrosis, we compared serum pancreatic amylase isoenzyme (P isoamylase) activity with the more complex standard tests of pancreatic function. Twenty-seven patients with cystic fibrosis, newborn to 46 years of age, were studied. All patients over 17 months old with evidence of pancreatic exocrine insufficiency, as manifested by abnormal duodenal secretions and/or abnormal 72-hour fecal fat absorption, had serum P isoamylase activity below the age-matched normal range; patients with adequate pancreatic function (aged 2 to 46 years) had P isoamylase activity in or above the normal range. Although both normal neonates and neonates with cystic fibrosis have very low levels of serum P isoamylase activity, in patients over 1 1/2 years of age serum P isoamylase activity may serve as a simple and useful discriminator of pancreatic exocrine function in patients with cystic fibrosis.

Amylases↗
PubMed1998

Effect of pancreatic enzymes on zinc absorption in cystic fibrosis.

BACKGROUND: The trace metal zinc has a wide range of important physiologic roles. Indirect evidence suggests that fat malabsorption is associated with malabsorption of zinc. The objective of this study was to evaluate the effect of pancreatic enzyme replacement on zinc absorption in children and adolescents with cystic fibrosis. METHODS: Subjects were four boys and four girls ranging in age from 7 to 17 years of age. All were pancreatic insufficient. Stable isotope labels, 70Zn and 67Zn, were administered orally in divided doses on consecutive days with meals. Meals were identical on the first 2 study days. Subjects were randomized to have pancreatic enzyme replacement withheld on the first or second day. All fecal samples were collected quantitatively for 10 days after label administration and were analyzed individually for total zinc and isotopic enrichment using atomic absorption spectrophotometry and fast atom bombardment mass spectrometry, respectively. Fractional absorption of zinc was calculated from cumulative fecal excretion of unabsorbed label. RESULTS: Fractional absorption while receiving enzymes was 0.50 +/- 0.29 versus 0.38 +/- 0.24 while not taking enzymes (p = 0.05). CONCLUSIONS: These results indicate that fractional absorption of zinc is impaired by pancreatic insufficiency in patients with cystic fibrosis, and is improved by exocrine pancreatic enzyme replacement.

Adolescent↗
PubMed1991

Analysis of pancreatic serum markers in patients with cystic fibrosis.

The occurrence of increased serum concentrations of pancreatic tumor markers CA 19-9, CA 50 and elastase 1 in cystic fibrosis (CF) prompted us to investigate the pancreatic markers DU-PAN-2 and TATI. DU-PAN-2 was measured in serum samples of 48 CF patients, 42 pediatric control patients, and 51 parents of CF children by a competition radioimmunoassay (RIA). A commercially available RIA test kit was used to measure TATI serum levels of 38 CF patients and 40 control patients. Nineteen percent of CF patients, 0% of the control group, and 6% of the parents exceeded the DU-PAN-2 threshold value of 300 U/ml. TATI concentrations were increased (greater than 20 micrograms/L) in 24% of CF patients and in 17% of control patients. The sensitivity-specificity curve of DU-PAN-2 was similar to those of CA 50 and elastase 1 but less discriminating than that of CA 19-9. However, the sensitivity-specificity profile of CA 19-9 can only be marginally enhanced in a range of low specificity by simultaneously determining elastase 1 and/or DU-PAN-2 antigens. TATI does not provide any diagnostic use in CF.

Adult↗
PubMed2005

Chronic pancreatitis.

PURPOSE OF REVIEW: As in our previous reviews, we endeavor to review important new observations in chronic pancreatitis made in the past year. We included articles, including review articles, only if they contained new observations or readdressed old questions and provided new insights into old and new concepts. RECENT FINDINGS: Important observations include the following: (1) Strong association between cystic fibrosis transmembrane regulator dysfunction/mutations and 'recurrent acute pancreatitis', particularly in patients with pancreas divisum (2) Pancreas divisum may be incidental finding in recurrent acute pancreatitis (3) Smoking increases risk of chronic pancreatitis (4) Coxsackie B virus may increase severity of alcoholic chronic pancreatitis (5) CD4+ T cells and an immune reaction against amylase may play a role in pathogenesis of autoimmune pancreatitis (6) 2-(18F)-Fluro-2-deoxy-D-glucose positron emission tomography might be useful to detect pancreatic cancer in chronic pancreatitis patients at risk for developing pancreatic cancer, but contrast-enhanced Doppler ultrasound or endosonography may be as sensitive and better than contrast enhanced computed tomography (7) Superiority of surgery vs endotherapy for long term pain relief and weight gain in painful chronic pancreatitis (8) Early treatment of pain and malabsorption may improve life quality (9) Antifibrogenesis and fibrolytic agents as potential therapies. SUMMARY: Ongoing basic and clinical research this past year has further characterized genetic, molecular and clinical aspects of chronic pancreatitis. The advent of predictable and lasting treatments of chronic pancreatitis is most likely to appear on the wings of carefully conducted studies targeting genetic and molecular mechanisms of chronic pancreatitis, particularly pancreatic fibrogenesis.

Global Health↗
PubMed1995

Presence of MRP8 and MRP14 in pancreatic cell lines: differential expression and localization in CFPAC-1 cells.

A complex of two calcium binding proteins, MRP8 [also called cystic fibrosis (CF) antigen] and MRP14, proteins known to be expressed in cells of myeloid origin, has been shown to be present in higher amounts in the serum of CF patients and heterozygotes compared with normal subjects. We demonstrated here for the first time, by dot-blot analysis and immunocytochemistry, the expression and the presence of these S100 calcium binding proteins in the pancreatic cell lines CAPAN-1 and CFPAC-1, the latter provided from a patient with CF. Moreover, using immunocytochemical methods, we showed that the localization of MRP8 and MRP14 on the plasma membrane seems to be restricted to the cells expressing a cystic fibrosis transmembrane conductance regulator (CFTR) wild-type protein such as CAPAN-1 cells and CFPAC-1 cells transfected with a plasmid containing the nonmutated CFTR gene (CFPAC-PLJ-CFTR-6 cells). In CFPAC-1 cells, immunoreactivity remains in the cytoplasm throughout the stationary phase. We also showed an increased level of the mRNAs of the two proteins in the CFPAC-1 cells compared with those transfected with the nonmutated CFTR. The demonstration of a difference in the cellular localization of these two proteins and in their mRNA levels in the cell line of CF origin leads us to assume the existence of a possible correlation in the expression of the MRPs with that of the CFTR protein.

Antigens, Differentiation↗