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At least 1,423 records · Page 79Linked to original sources
PubMed1988

Fatty acids in cystic fibrosis in response to a marathon race.

Lipid metabolism was studied in three cystic fibrosis (CF) patients (18 years old) and three healthy controls (35-40 years old) who completed the New York Marathon 1984. Lipase was not detectable in plasma from two of the CF patients, but in one CF patient with a functioning pancreas and in the three controls it increased during and after the run. Cholesterol concentrations were lower in all patients than in the controls. The HDL/total cholesterol ratios increased in all runners throughout the observation period, substantially more in the patients than in the controls. Triglycerides and glycerol values were similar in the two groups. The relative distribution of fatty acids in total plasma, plasma free fatty acid fractions, and total red blood cell membranes, at rest, were similar to those earlier reported both for the CF patients with pancreatic insufficiency and for the controls. Lower relative concentrations of linoleic acid (C18:2n6) and higher relative concentrations of C14:0, C16:0, C16:1, C18:3n6, and C20:3n6 were found in the CF specimens. During and after the race, linoleic acid concentration increased and the other fatty acids decreased, giving a more normalized pattern in the patients. The fatty acid pattern of the CF patient with a functioning pancreas behaved more like the healthy controls. The fatty acid pattern of the red cell membrane did not change during or after the run. Although the observations are few, it is encouraging that both the HDL/total cholesterol ratio and the fatty acid pattern improve in response to prolonged aerobic exercise.

Adolescent↗
PubMed1997

MUCLIN expression in the cystic fibrosis transmembrane conductance regulator knockout mouse.

BACKGROUND & AIMS: Cystic fibrosis is characterized by increased secretion of glycoconjugates with altered carbohydrate composition, but no specific gene products that show these changes have been identified. The aim of this study was to use a recently described sulfated mucin-like glycoprotein (MUCLIN: formerly called gp300) as a model glycoconjugate to study such changes in the gastrointestinal system in the cystic fibrosis transmembrane conductance regulator (CFTR) knockout mouse (cftrm1Unc). METHODS: Western and Northern blots were used to determine the tissue levels of MUCLIN and its messenger RNA (mRNA) in normal and CFTR knockout mice. Immunocytochemistry was used to determine the localization of MUCLIN. RESULTS: MUCLIN is expressed in the normal mouse intestinal tract, pancreas, and gallbladder. In CFTR knockout mice, MUCLIN shows increased expression at both mRNA and protein levels in pancreas and duodenum, but not in the gallbladder. In the duodenum, MUCLIN was localized intracellularly in crypt enterocytes and on the luminal surface, and luminal surface labeling was dramatically increased in the CFTR knockout mouse. In the CFTR knockout mouse duodenum and gallbladder, MUCLIN showed retarded electrophoretic migration indicating altered posttranslational processing. CONCLUSIONS: MUCLIN shows increased expression and possibly altered posttranslational processing in the CFTR knockout mouse and will serve as a good model for understanding changes in the composition of mucous secretions in patients with this disease.

Animals↗
PubMed2001

Chronic pancreatitis: diagnosis, classification, and new genetic developments.

The utilization of recent advances in molecular and genomic technologies and progress in pancreatic imaging techniques provided remarkable insight into genetic, environmental, immunologic, and pathobiological factors leading to chronic pancreatitis. Translation of these advances into clinical practice demands a reassessment of current approaches to diagnosis, classification, and staging. We conclude that an adequate pancreatic biopsy must be the gold standard against which all diagnostic approaches are judged. Although computed tomography remains the initial test of choice for the diagnosis of chronic pancreatitis, the roles of endoscopic retrograde pancreatography, endoscopic ultrasonography, and magnetic resonance imaging are considered. Once chronic pancreatitis is diagnosed, proper classification becomes important. Major predisposing risk factors to chronic pancreatitis may be categorized as either (1) toxic-metabolic, (2) idiopathic, (3) genetic, (4) autoimmune, (5) recurrent and severe acute pancreatitis, or (6) obstructive (TIGAR-O system). After classification, staging of pancreatic function, injury, and fibrosis becomes the next major concern. Further research is needed to determine the clinical and natural history of chronic pancreatitis developing in the context of various risk factors. New methods are needed for early diagnosis of chronic pancreatitis, and new therapies are needed to determine whether interventions will delay or prevent the progression of the irreversible damage characterizing end-stage chronic pancreatitis.

Chronic Disease↗
PubMed1998

Polycystic kidney and liver disease in cats.

This paper reviews 27 cases of polycystic disease of the kidneys and/or liver in cats. The multiple cysts in the kidneys were rounded in all but one case, as described in adult polycystic kidney disease in humans. In 68% of the cats presented with polycystic kidneys, there were also cystic changes of the liver (uni- or multilocular cysts and/or congenital hepatic fibrosis (CHF)). In 1 cat polycystic changes of kidneys and liver were accompanied by cysts in the pancreas. In 5 cases there was severe pancreas fibrosis. Twenty-one of the 27 cats were Persian or Persian-crossbred.

Animals↗
PubMed2003

Association between serum oncofetal antigens CA 19-9 and CA 125 and clinical status in patients with cystic fibrosis.

UNLABELLED: In cystic fibrosis (CF), mucus plugging in the airways and in the gastrointestinal tract leads to severe morbidity and mortality. The mucin-associated antigens CA 19-9 and CA 125 are markers of gastrointestinal malignancy, and CA 19-9 has also been reported in association with pulmonary function in CF. AIM: To test whether these antigens might serve as markers for the severity of pulmonary and gastrointestinal disease in CF. METHODS: In 99 patients, aged 1 to 48 y, serum levels of CA 19-9 and CA 125 were measured by RIA and ELISA and related to clinical data. RESULTS: Patients with severe mutations had significantly increased serum levels of CA 125, indicating an association with a more severe CF phenotype. This was further supported by the association with lung function, chronic pulmonary colonization of Pseudomonas aeruginosa and pancreatic insufficiency. CA 19-9 was also shown to be associated with lung function and Ps. aeruginosa colonization. No gastrointestinal malignancy was found in our patients despite very high values of CA 19-9 in some patients. During a 5-y follow-up, the very high serum levels of CA 19-9 decreased along with improved general condition of the patients. CONCLUSION: Increased serum levels of CA 125 in CF patients were associated with severe cystic fibrosis transmembrane conductance regulator mutations and a severe phenotype. Both antigens were associated with pseudomonas colonization and lung function and CA 125 also with pancreatic insufficiency. The estimates of CA 19-9 are hampered by the influence of the Lewis histo-blood group system on the synthesis of CA 19-9.

Adolescent↗
PubMed1997

Spectrum of CFTR mutations in Argentine cystic fibrosis patients.

The identification of different mutations which cause cystic fibrosis (CF) in Argentine patients has been performed. Initially, 10 of the most commonly mutated loci in 228 independent chromosomes were analyzed. Each allele was detected by PCR amplification of DNA samples either directly on polyacrylamide gels, by restriction enzyme digestion and agarose gels electrophoresis, or by hybridization with allele specific oligonucleotides. The delta F508 mutation was found in 57% of the alleles. The frequencies of the other CF mutations were as follows: G542X 3.9%, W1282X 3.1%, N1303K 1.7%, 1717 1-G-->A 0.9%, R553X 0.4%, R1162X 0.4%, whereas G551D, delta I507 and S549N were not found. This direct mutation analysis enabled the detection of 155/228 CF alleles (67%). Of the remaining 73 unidentified CF alleles, 22 were investigated for the 27 exons by DGGE and 9 rare mutations were identified. The incidence of the main CF mutations analyzed was similar to that of the South European population and markedly different from other Latin American countries. The mutation data presented here may be useful for designing DNA testing strategies for CF in Argentina.

Argentina↗
PubMed1996

Phenotypic abnormalities in long-term surviving cystic fibrosis mice.

Mouse models for cystic fibrosis (CF) with no CFTR function (Cftr-/-) have the disadvantage that most animals die of intestinal obstruction shortly after weaning. The objective of this research was to extend the lifespan of CF mice and characterize their phenotype. Weanlings were placed on a nutrient liquid diet, and histologic and functional aspects of organs implicated in the disease were subsequently examined. Approximately 90% of Cftr-/- mice survived to 60 d, the majority beyond 100 d. Cftr-/- mice were underweight and had markedly abnormal intestinal histology. The intestinal epithelia did not respond to challenges with agents that raised intracellular cAMP, consistent with the absence of functional CFTR. No lesions or functional abnormalities were evident in the lungs. Liquid-fed Cftr-/- mice were infertile, although some males weaned to a solid diet were fertile before they died. Thus, we have succeeded in using dietary means to prolong the lives of Cftr-/- mice.

Animals↗
PubMed1999

Synergistic effects of cAMP- and calcium-mediated amylase secretion in isolated pancreatic acini from cystic fibrosis mice.

We evaluated pancreatic enzyme secretory response to secretagogues (cAMP- and Ca2+-mediating) involved in exocytosis and in chloride channel activation in an exon 10 knockout cystic fibrosis (CF) mouse model. Experiments were performed in isolated pancreatic acini from liquid-fed Cftr-/- mice (5-6 wk of age) and age-matched Cftr+/+ controls fed a solid or liquid diet. BrcAMP and forskolin alone induced higher amylase secretion (% initial amylase content) in the Cftr+/+ acini than carbachol (p < 0.05). Carbachol and BrcAMP or BrcAMP and forskolin, given in combination, produced additive effects on enzyme secretion in the Cftr+/+ acini. Ca2+- and cAMP-mediated amylase secretion in isolated pancreatic acini from the Cftr-/- mice was no different to that observed in the age- and diet-matched Cftr+/+ animals. However, Cftr-/- pancreatic acini showed a significantly greater amylase response to the combination of BrcAMP and carbachol than the sum of the individual responses in separate experiments (p < 0.05). The amylase response was not different in acini from solid-fed or liquid-fed Cftr+/+ controls. In summary, this study suggests that cystic fibrosis transmembrane conductance regulator is not essential for enzyme secretion as evidenced by no reduction in cAMP-mediated amylase secretion in Cftr-/- mice. The results in Cftr+/+ acini suggest pancreatic enzyme secretion is mediated via multiple intracellular pathways acting in parallel and probably converge at a distal step in the secretory process. However, Cftr-/- pancreatic acini exhibited a synergistic secretory response following stimulation by BrcAMP plus carbachol. The enhanced secretory response may partially contribute to the development of pancreatic dysfunction in CF patients by facilitating occlusion of digestive enzymes in the secretory canaliculus of the pancreatic acini.

8-Bromo Cyclic Adenosine Monophosphate↗
PubMed2000

Parallel secretion of pancreatic phospholipase A(2), phospholipase A(1), lipase, and colipase in children with exocrine pancreatic dysfunction.

The cosecretion of pancreatic lipase and colipase are important in normal fat digestion. As adsorption of phosphatidylcholine to the lipid substrate interferes with lipase activity, hydrolysis to lysophosphatidylcholine with subsequent desorption is also essential for fat digestion. There are some data regarding the secretion of pancreatic phospholipases in normal adults but none in children or patients with pancreatic disease. In the present study, we aimed a) to develop an accurate fast assay method to measure phospholipase A(2) and b) to determine the secretion rate of pancreatic phospholipase A(2) and whether it is cosecreted with lipase and colipase in children with exocrine pancreatic dysfunction. Nine male patients aged 0.5 to 16 y (seven with cystic fibrosis, two with malabsorption) underwent pancreatic stimulation tests. Their colipase and lipase secretion rates were measured by titrimetric methods and phospholipase A(2) and A(1) by phosphorus magnetic resonance spectroscopy ((31)P NMR). It was found that the phospholipases, colipase, and lipase were absent in the two patients with pancreatic insufficiency. In patients with normal absorption, there were marked inter-and intrasubject variations of lipase, colipase, and phospholipase secretion rates that were consistent with the degree of exocrine pancreatic dysfunction. However, in the three 20-min stimulation periods of the pancreatic function test, pancreatic phospholipase is cosecreted with lipase and colipase, and average colipase and phospholipase A(2) secretion rates follow a similar or parallel pattern. These findings are consistent with the important role of pancreatic phospholipases in intestinal phospholipid hydrolysis leading to the desorption of phospholipids from the lipid substrate and enhancing lipid hydrolysis and phospholipid absorption.

Adolescent↗
PubMed1992

Genetic determination of exocrine pancreatic function in cystic fibrosis.

We showed elsewhere that the pancreatic function status of cystic fibrosis (CF) patients could be correlated to mutations in the CF transmembrane conductance regulator (CFTR) gene. Although the majority of CF mutations--including the most common, delta F508--strongly correlated with pancreatic insufficiency (PI), approximately 10% of the mutant alleles may confer pancreatic sufficiency (PS). To extend this observation, genomic DNA of 538 CF patients with well-documented pancreatic function status were analyzed for a series of known mutations in their CFTR genes. Only 20 of the 25 mutations tested were found in this population. They accounted for 84% of the CF chromosomes, with delta F508 being the most frequent (71%), and the other mutations accounted for less than 5% each. A total of 30 different, complete genotypes could be determined in 394 (73%) of the patients. The data showed that each genotype was associated only with PI or only with PS, but not with both. This result is thus consistent with the hypothesis that PI and PS in CF are predisposed by the genotype at the CFTR locus; the PS phenotype occurs in patients who have one or two mild CFTR mutations, such as R117H, R334W, R347P, A455E, and P574H, whereas the PI phenotype occurs in patients with two severe alleles, such as delta F508, delta I507, Q493X, G542X, R553X, W1282X, 621 + 1G----T, 1717-1G----A, 556delA, 3659delC, I148T, G480C, V520F, G551D, and R560T.

Alleles↗
PubMed1983

The diffuse endocrine system in cystic fibrosis.

The pathology, aetiology and clinical features of cystic fibrosis (CF) are briefly reviewed. The diffuse endocrine system (DES) may be involved in either a primary or secondary manner. Carbohydrate intolerance is common and the release of islet hormones is deficient, as is the release of the intestinal hormone gastric inhibitory polypeptide (GIP). The post-prandial responses of insulin and GIP can be improved by substituting an elemental meal, suggesting that malabsorption and local intestinal factors could be causative in the deficient responses. Vasoactive intestinal polypeptide-like immunoreactive (VIP-LI) cell numbers are increased in CF and an intimate association with mucous cells is noted suggesting a paracrine relationship. These findings could have implications in the diagnosis, management and aetiology of CF.

Atrophy↗