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Results for “CYSTIC FIBROSIS OF PANCREAS”

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At least 865 records · Page 48Linked to original sources
PubMed2001

F508 amino acid deletion mutation of CFTR gene in Korean lung cancer patients.

Mutations of the transmembrane conductance regulator (CFTR) gene in cystic fibrosis lead to dysfunction of the lung, pancreas, and sweat glands, etc. To investigate the possibility of the relationship between lung cancer and the mutations of CFTR gene, we determined amino acid sequences using reverse transcription-polymerase chain reaction (RT-PCR) and DNA sequencing. In this study, the deletion mutation of 508th amino acid in one of nine lung caner patients was found confirming that CFTR gene mutation exists in a Korean lung cancer patient.

Adult↗
PubMed1981

Serum immunoreactive trypsin in cystic fibrosis.

Serum immunoreactive trypsin (IRT) concentrations were measured blind in sera from 33 patients with cystic fibrosis and in 32 age-matched controls. Thirty-two patients had abnormal trypsin concentrations. In 30 of these patients the level of IRT was very low whereas in the other two (the youngest of the group) it was extremely high. All 30 patients with low IRT concentrations had clinical or biochemical evidence of malabsorption. Serum IRT concentration may thus be a useful diagnostic test for cystic fibrosis. In view of the recent observation that neonates with cystic fibrosis consistently have raised IRT concentrations, and the fact that the youngest two patients in our series also had markedly increased IRT, it would appear that the initial elevation of IRT reflects early damage to the pancreas with release of enzyme/zymogen; this is thereafter superseded by a progressive destruction of the pancreas resulting in low IRT concentrations in adults with cystic fibrosis.

Adolescent↗
PubMed1975

Insulin release in cystic fibrosis.

Early insulin response to rapid intravenous injection of glucose was studied in 7 cases of cystic fibrosis aged 8 months to 9 1/2 years. Plasma insulin was measured with a radioimmunological method. Blood glucose values were determined and the glucose disappearance rate (kG) calculated. In all children except the youngest one the early insulin response values were low compared with normal children. The kG-values were normal and correlated neither to the duration of clinical symptoms, nor to the patients' actual clinical condition measured by means of the Shwachman score. The explanation of the decreased insulin response is probably the progressive fibrosis of the pancreas. This may also explain the reported increased incidence of diabetes mellitus in cystic fibrosis. Comparison is made with the condition in pancreatic fibrosis in rabbits, produced through duct ligation.

Blood Glucose↗
PubMed1991

Renal-hepatic-pancreatic dysplasia and its variants.

A family is reported, in which two pregnancies resulted in the birth of a female fetus with multiple congenital anomalies, including renal cystic dyplasia, pancreatic fibrosis with dilated pancreatic duct, and some anomalies of the face and genitalila. The pathology of the second fetus was revealed by prenatal ultrasonographic examination. In the relevant literature 20 additional cases of renal-hepatic-pancreatic dysplasia (RHPD) could be found. It is demonstrated that cystic renal dysplasia associated with pancreatic fibrosis or cystofibrosis (with normal liver) as well as cystic renal dysplasia associated with hepatic fibrosis (with normal pancreas) should be considered as incomplete RHPD variants. In 6 cases out of 22, the infants had some features of the apolysplenia complex, including situs inversus and/or heart defects. The association of RHPD and apolysplenia has been proved to be an autosomal recessively inherited syndrome. Most probably RHPD without apolysplenia represents a separate entity with autosomal recessive mode of inheritance as well.

Chromosome Aberrations↗
PubMed2002

Cystic fibrosis presenting as acute pancreatitis and obstructive azoospermia in a young adult male with a novel mutation in the CFTR gene.

Cystic fibrosis is rare in the Asian population, and is often associated with consanguinity and rare genotypes. We report on a 23-year-old Asian man from a consanguineous pedigree referred to the regional cystic fibrosis unit after a diagnosis of congenital bilateral absence of the vas deferens during investigations for infertility. A detailed history revealed several previous episodes of acute pancreatitis. Full diagnostic appraisal showed homozygosity for a novel cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation, but normal sweat test and nasal potential difference studies. An endoscopic retrograde cholangiopancreatogram (ERCP) showed chronic pancreatitis with bulky side branches. The vas deferens and the pancreas appeared exquisitely sensitive to mild CFTR dysfunction. Patients with cystic fibrosis and unexplained upper abdominal pain should be screened for pancreatitis, and consideration should be given to screening patients with idiopathic pancreatitis for mutations in the CFTR gene.

Acute Disease↗
PubMed2005

Ethanol administration to cystic fibrosis knockout mice results in increased fatty acid ethyl ester production.

BACKGROUND: Fatty acid ethyl esters (FAEE) are nonoxidative ethanol metabolites shown to produce toxic effects in the liver and pancreas in vivo and in vitro. Because alcohol-induced chronic pancreatitis is associated with mutations in the gene responsible for cystic fibrosis (CFTR), we hypothesized that CFTR dysfunction leads to increased levels of these toxic nonoxidative ethanol metabolites following alcohol administration. METHODS: Cystic fibrosis (CF) and wild-type (WT) mice were injected intraperitoneally with 1, 2, or 3 g/kg of 50% ethanol. Mice were sacrificed and the liver and pancreas removed for FAEE analysis. RESULTS: The mean FAEE concentration (pmol/g) detected in the liver of cftr mice following injection with 2 g/kg of ethanol was significantly greater than the amount detected in WT (p < 0.005). A similar trend in FAEE concentration was seen in the pancreas, but the difference was not statistically different. In both the liver and pancreas, analysis of individual FAEE species demonstrated a selective increase in ethyl oleate. CONCLUSION: These data show an association between CFTR dysfunction and qualitative and quantitative changes in FAEE in liver and pancreas upon ethanol exposure.

Animals↗
PubMed1987

Impairment of pancreatic acinar function by reserpine in vivo and in vitro.

Chronic reserpine treatment of animals, an experimental model for cystic fibrosis (CF), results in generalized exocrinopathy, impaired pancreatic secretion, and decreased pancreatic content of amylase. The mechanisms of altered acinar function and decreased amylase content in both CF and the reserpine-treated rat are unknown. To examine this alteration, the rate of [3H]phenylalanine (phe) incorporation into cellular protein was determined in pancreatic acinar cells after reserpine treatment of rats in vivo (7 d) and of cells in vitro (1 to 24 h). Acinar cells isolated from control, chronic reserpine-treated, and pair-fed rats were incubated in vitro with 0, 30, 50, or 100 microM reserpine. Reserpine treatment in vitro for 24 h of acinar cells from control rats significantly decreased amylase activity (20 to 70%), an effect similar to that of reserpine treatment in vivo. In vivo, reserpine treatment decreased [3H]phe incorporation (disintegrations per minute per milligram protein) 56% in freshly isolated cells, but did not alter intracellular specific activity (disintegrations per minute per nanomole phe, SA) of [3H]phe. Reserpine treatment (30 and 50 microM) in vitro for 1 h also decreased [3H]phe incorporation by freshly isolated cells from control (53 to 85%) and pair-fed (40 to 68%) rats. Reserpine treatment for 24 h in vitro significantly decreased [3H]phe incorporation by cells from control (82 and 98%), pair-fed (80 and 95%), and chronic reserpine-treated (90 and 97%) rats as compared with cells from respective in vivo treatments cultured with no reserpine. In vitro reserpine treatment also decreased the intracellular SA of [3H]phe in freshly isolated cells from control (14 and 36%) and pair-fed (35 and 39%) rats and in cultured cells from control (11 and 86%), pair-fed (60 and 88%), and chronic reserpine-treated (49 and 76%) rats. However, these alterations of SA by reserpine did not account for the decreased incorporation of [3H]phe into acinar protein, which remained significantly lower (70 to 88%) when expressed as total phe incorporation. These results suggest that reserpine acts directly on acinar cells to alter function and that the ability of the pancreas to synthesize digestive enzymes may be impaired in this model of cystic fibrosis.

Amylases↗
PubMed1982

Origin and levels of trypsin in amniotic fluid throughout pregnancy.

Trypsin is present in midtrimester amniotic fluid with a mean level of 350 +/- 260 ng/ml, but has virtually disappeared by term. The trypsin originates in fetal pancreas and it is suggested that its mode of entry into the gestation sac is by passage of meconium which ceases as a physiological event by 16-18 weeks. The finding could form the basis of an antenatal test for the presence of cystic fibrosis in the fetus.

Amniotic Fluid↗
PubMed1989

A competition enzyme-linked immunosorbent assay (ELISA) for the measurement of pancreatic GP-2 glycoprotein.

A competition enzyme-linked immunosorbent assay has been developed for the quantitative detection of soluble and membrane-bound GP-2, a glycoprotein which is confined to the exocrine pancreas. Zymogen granule membranes fixed to microtiter plates with poly-L-lysine were used as the source of antigen. Detergent 3-[(3-cholamidopropyl)dimethylammonio]-1-propanesulfonate (CHAPS] was added to the assay in order to reveal all the antigens, more particularly in membranous samples. Presence of detergent at concentrations as high as 0.5% did not interfere with any particular steps of the ELISA. This competition ELISA can detect 10 ng of GP-2 and will be useful for measuring soluble as well as membrane GP-2 in order to elucidate its role in the secretory process of the pancreas as well as in certain pathologies such as cystic fibrosis.

Animals↗
PubMed1986

Diagnosis of exocrine pancreatic insufficiency in cystic fibrosis by use of fluorescein dilaurate test.

In the fluorescein dilaurate test fluorescein dilaurate is cleaved by the pancreas specific cholesterol ester hydrolase activity and the liberated fluorescein is absorbed and excreted in the urine. Fluorescein recovery is a reflection of exocrine pancreatic function. The test was evaluated in 14 patients with cystic fibrosis and 16 healthy volunteers. The test was well tolerated by patients, was easy to perform, and gave significantly lower values in the patients suffering from cystic fibrosis. The result of the pancreolauryl test was also correlated with the result of the faecal chymotrypsin test in 11 of the patients suffering from cystic fibrosis. A positive correlation was found between the two test results. The test is a practical and reliable index of pancreatic exocrine function and may have a useful role as a screening procedure.

Adolescent↗
PubMed1987

Lack of pancreatic [3H]estradiol-binding activity in cystic fibrosis.

A major characteristic of cystic fibrosis (CF) is the progressive degeneration of acinar cells in the pancreas. It is now well established that the normal pancreas contains an abundant amount of an [3H]estradiol-binding protein. Although the physiological function of this protein is unknown, available evidence suggests that it modulates resting secretion from acinar cells. Analysis of pancreatic autopsy samples from 13 patients who had CF demonstrated a high degree of correlation between loss of acinar cells and loss of [3H]estradiol-binding activity. Autopsy samples taken from individuals unaffected by CF contained large amounts of the [3H]estradiol-binding protein that were significantly correlated with the tissue content of amylase. This biological model demonstrates that the [3H]estradiol-binding protein in pancreas is localized primarily within acinar cells. Based on the presumed regulatory role this protein has on pancreatic secretion, an hypothesis is offered to account for acinar cell degeneration in individuals suffering from cystic fibrosis.

Adolescent↗