cholecystokinin and Cystic-Fibrosis

Topics: Amino Acid Metabolism, Inborn Errors; Amylases; Child, Preschool; Cholecystokinin; Cystic Fibrosis; Dicloxacillin; Humans; Infant; Infant, Newborn; Kidney Diseases; Lipase; Lipid Metabolism, Inborn Errors; Pancreas; Pancreatic Cyst; Pancreatic Diseases; Pancreatic Extracts; Prognosis; Secretin; Triglycerides; Trypsinogen; Uric Acid

Topics: Adrenal Cortex Hormones; Adrenocorticotropic Hormone; Bile Acids and Salts; Cholecystokinin; Cystic Fibrosis; Enzymes; Gastric Juice; Gastrointestinal Hormones; Humans; Lipoprotein Lipase; Pancreas; Pancreatic Juice; Pancreatitis; Phospholipases; Research; Salts; Secretin; Vitamin B 12

The secretin-cholecystokinin (CCK) test is the gold standard in the evaluation of exocrine pancreatic insufficiency. Because of its invasive character, it is of limited value in cystic fibrosis (CF) patients, especially in those with severe respiratory disease. The aim of the study was to evaluate the sensitivity of fecal elastase-1 in relation to the secretin-CCK test and quantitative fecal fat excretion in CF patients.. The study comprised 28 patients (11 females and 17 males) aged 4 to 20 years. In all patients the secretin-CCK test and determination of fecal elastase-1 concentration (with enzyme-linked immunosorbent assay) and fecal fat excretion were performed.. The range of fecal elastase-1 was from undetectable to 485 microg/g (mean, 84.6+/-119.9 microg/g) and of fecal fat excretion from 1.0 to 55.1 g/day (mean, 15.0+/-12.2 g/day). On the basis of the results of the secretin-CCK test (and fecal fat analysis) exocrine pancreatic insufficiency was divided into three subgroups: mild (I), moderate (II), and severe (III). Four patients were classified in subgroup I, 4 in II and 20 in III. Fecal elastase (elastase-1) results were 332.0+/-124.9 microg/g in subgroup I, 96.9+/-45.7 microg/g in subgroup II, and 32.1+/-41.2 microg/g in subgroup III. The fecal elastase-1 sensitivity with a cut-off point of 200 microg/g was 89.3% for all patients, 100% for patients in subgroups II and III, but only 25.0% for patients in subgroup I; the specificity was 96.4%. Linear regression analysis showed a statistically significant correlation between fecal elastase (elastase-1) and duodenal volume, bicarbonate, amylase, lipase, and trypsin secretion (in all cases P < 0.001).. Measurement of fecal elastase-1 is simple and very useful for assessing the exocrine pancreatic function in CF patients. Elastase is highly specific in severe and moderate exocrine pancreatic insufficiency, but it is rather unspecific for milder forms of pancreatic insufficiency.

Topics: Adolescent; Adult; Child; Child, Preschool; Cholecystokinin; Clinical Enzyme Tests; Cystic Fibrosis; Exocrine Pancreatic Insufficiency; Feces; Female; Humans; Male; Pancreatic Elastase; Secretin; Sensitivity and Specificity

Regulation of pancreatic exocrine secretion is controlled by vagovagal reflexes and hormones. A negative feedback control mechanism exists between the intraduodenal protease concentration and pancreatic enzyme secretion. In man cholecystokinin (CCK) is the major regulator of postprandial pancreatic enzyme secretion. There is a 50% reduction of meal-stimulated secretion by the specific CCK receptor antagonist loxiglumide, whereas atropine completely blocks postprandial secretion. Neurotensin is released postprandially by nerval reflexes and fat. It has been claimed that both hormones are increased in patients with pancreatic insufficiency.. We investigated CCK and neurotensin levels in patients with cystic fibrosis and pancreatic insufficiency. In 35 patients (2-24 years old) with cystic fibrosis with steatorrhea and in 15 patients (1.5-24 years old) with cystic fibrosis without pancreatic insufficiency pre- and post-prandial CCK and neurotensin plasma levels were measured 3 days after pancreatic enzyme therapy had been withdrawn. Nine patients (3-14 years old) who had no complaint of abdominal disease served as controls.. Basal and postprandial CCK plasma levels did not differ statistically in the three groups, whereas basal and postprandial neurotensin levels were significantly increased in the cystic fibrosis groups. The severity of the disease had no effect on the neurotensin levels.. Cystic fibrosis patients with severe pancreatic insufficiency did not have increased CCK plasma levels, suggesting that a CCK-mediated feedback mechanism of pancreatic enzyme secretion does not operate in our patients. In contrast, basal and postprandial neurotensin plasma levels were significantly increased in patients with cystic fibrosis but were independent of the severity of the pancreatic insufficiency.

Topics: Adolescent; Case-Control Studies; Child; Cholecystokinin; Cystic Fibrosis; Exocrine Pancreatic Insufficiency; Feedback; Female; Humans; Male; Neurotensin; Pancreas; Radioimmunoassay

Pancreatic function can only be determined exactly via the pancreozymin-secretin test. We conducted this test in two versions: (1) under conditions of continuous perfusion with the possibility of volume correction and (2) as a simple tubing. We compared the results of 86 tubings with the results of 87 examinations under perfusion. For that purpose all patients were classified into four groups: group a) with 46 and 10 examinations, respectively, in patients suffering from cholestasis in early infancy, group b) with 7 and 12 examinations, respectively, in older patients with liver diseases, group c) with 8 and 17 examinations, respectively, in patients suffering from cystic fibrosis or Shwachman's syndrome and group d) with 25 and 48 examinations, respectively, in children with normal pancreatic function. Both examination methods nearly identical mean values of the enzyme activities in all four patient groups. However, mean variations were found to be higher in case of tubing. Therefore the lower limits (x - 2s) of this test were defined at a lower level than those of the tests under perfusion.

Topics: Amylases; Child; Child, Preschool; Cholecystokinin; Cystic Fibrosis; Diagnosis, Differential; Exocrine Pancreatic Insufficiency; Humans; Infant; Infant, Newborn; Intestinal Secretions; Jaundice, Neonatal; Lipase; Liver Diseases; Pancreatic Function Tests; Reference Values; Secretin; Trypsin

Topics: Anesthesia, Intravenous; Child; Cholecystokinin; Cystic Fibrosis; Humans; Pancreatic Function Tests; Propofol; Secretin

1. In patients with cystic fibrosis, abnormalities in plasma cholecystokinin level and gall-bladder emptying may contribute to the development of maldigestion and gall-stones. 2. Therefore, we have measured plasma cholecystokinin levels and gall-bladder volumes before and after ingestion of a standard breakfast in eight adult patients with cystic fibrosis and in eight normal control subjects. 3. In the patients with cystic fibrosis basal (2.8 +/- 0.4 pmol/l; P less than 0.05, t-test) and maximum post-prandial (5.7 +/- 0.5 pmol/l; P less than 0.05, t-test) plasma cholecystokinin levels were significantly higher than those in the control subjects (1.9 +/- 0.1 pmol/l, and 4.5 +/- 0.2 pmol/l, respectively). On the other hand, integrated plasma cholecystokinin secretion in response to the meal was similar (t-test, P = 0.4 versus control subjects). The increased plasma cholecystokinin levels in the patients with cystic fibrosis were accompanied by reduced gallbladder volumes in both the basal (7.8 +/- 2.1 cm3 versus 20.9 +/- 2.3 cm3 in control subjects; P less than 0.005, t-test) and the post-prandial state (2.2 +/- 1.0 cm3 versus 4.8 +/- 0.8 cm3 in control subjects; P = 0.06, t-test). Gall-bladder emptying in the patients with cystic fibrosis was well preserved (70 +/- 7% versus 78 +/- 9% in control subjects; P = 0.4, t-test). 4. In comparison with normal control subjects, patients with cystic fibrosis have an increased basal plasma cholecystokinin level and a reduced gall-bladder volume, whereas post-prandial gall-bladder emptying and plasma cholecystokinin secretion are not significantly different.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Adult; Cholecystokinin; Cystic Fibrosis; Female; Gallbladder; Humans; Male

A 3.5-month-old white boy was born with meconium ileus, peritonitis, and jejunal atresia from cystic fibrosis. He subsequently developed unrelenting and severe extrahepatic biliary obstruction as demonstrated by liver biopsy showing periportal inflammation, cholestasis, and fibrosis. Surgical exploration confirmed the diagnosis of extrahepatic biliary obstruction by severely inspissated bile. A cholecystostomy tube was left in place. The cholestasis remained unresponsive to conservative medical therapy. The obstruction was relieved by hydrostatic infusion of 2% N-acetylcysteine into the biliary tree over a 6-day period. The child also received concurrently four i.v. injections of synthetic cholecystokinin. This therapeutic modality was thought to be both safe and effective.

Topics: Acetylcysteine; Cholecystokinin; Cholestasis; Cystic Fibrosis; Humans; Infant; Injections, Intravenous; Liver; Male; Radiography; Therapeutic Irrigation

We performed pancreatic function tests on sixty-five cystic fibrosis (CF), and eleven control children. The technique used continuous IV infusion of cholecystokinin and secretin, with duodenal juice collection over a 90 min period, and was made quantitative by continuous duodenal infusion and distal collection of an unabsorbable marker (bromosulphthalein). Some CF patients had near normal pancreatic enzyme outputs, some had impaired but measurable levels, but most (79%) had almost absent trypsin secretion. CF children with better pancreatic function, were younger and more likely to be male. All controls showed a large increase in bicarbonate concentration and secretion rate per kilogram body weight during the test, but most children with CF (96.5%) did not. Because two of our CF patients had water and bicarbonate secretion within the control range, this finding does not exclude the diagnosis of CF. Sodium, potassium and chloride ion secretion in CF patients was lower than controls but overlap occurred. We found a linear correlation between acinar and tubular secretion in CF patients which indicates that there is probably not a primary genetic defect in pancreatic bicarbonate secretion in CF.

Topics: Adolescent; Bicarbonates; Child; Child, Preschool; Cholecystokinin; Cystic Fibrosis; Duodenum; Electrolytes; Female; Humans; Lipid Metabolism; Male; Pancreas; Pancreatic Function Tests; Secretin

Topics: Cholecystokinin; Cystic Fibrosis; Humans; Sincalide

Topics: 4-Aminobenzoic Acid; Aminobenzoates; Child; Child, Preschool; Cholecystokinin; Chymotrypsin; Cystic Fibrosis; Humans; Pancreas; Secretin

To study pancreatic protein and water secretion in 28 patients with cystic fibrosis and 21 controls matched for pancreatic acinar function as defined by trypsin secretion, we used a quantitative-marker perfusion technique and continuous intravenous secretin-pancreozymin stimulation. Regardless of the level of pancreatic acinar function, secretions from the patients contained significantly higher concentrations of protein than those from the controls. Total protein output and albumin:protein ratios were not increased in secretions from the patients, but their fluid secretion was significantly decreased at any level of pancreatic function. A significant linear correlation was found between protein and volume secretion in the patients (r = 0.86, P less than 0.001), most of whom had a fluid output of less than 4.2 ml per kilogram of body weight per hour. No such relation was found in the control subjects, whose flow was always above 4.2 ml per kilogram per hour. We conclude that fluid secretion in patients with cystic fibrosis may be a rate-limiting factor in protein output and that a limited flow of hyperconcentrated protein secretions may predispose to protein precipitation and ductal obstruction in the pancreas.

Topics: Adolescent; Adult; Albumins; Body Water; Child; Child, Preschool; Cholecystokinin; Cystic Fibrosis; Humans; Infant; Pancreas; Proteins; Secretin; Trypsin

More than 80% of patients with cystic fibrosis have poor pancreatic function, and have large daily faecal bile acid losses. This has been postulated to lower luminal bile acid concentrations and adversely affect fat absorption. We studied, for the first time, quantitative individual conjugated duodenal bile acid secretion rates into the duodenum during cholecystokinin/secretin infusion in 55 cystic fibrosis patients and six controls, using a quantitative non-absorbable marker technique. We were able to show adequate duodenal total bile acid concentrations and normal secretion rates in these children. The bile acid secretion pattern in cystic fibrosis patients showed a marked increase in bile acid concentration during cholecystokinin/secretin infusion, to levels which were above the critical micellar concentration indicating that the gall bladder is a functional organ in this disease. The subsequent fall in secretion rate was similar to controls. We have documented a significantly raised glycine/taurine bile acid conjugation ration in duodenal juice from cystic fibrosis patients and suggest that the combined effects of lowered ileal pH and increased glycine conjugated proportion of bile acids may cause precipitation of bile acids leading to decreased fat absorption and large faecal bile acid losses. To further investigate bile acid secretion in children with cystic fibrosis, we modified the high performance thin layer chromatography/densitometry method to enable measurement of individual glycine and taurine conjugates in serum. In comparing cystic fibrosis patients and controls, we were able to determine a group of 18 (36%) with bile acid evidence of liver damage who also showed reduced bile acid secretion into the duodenum. We were unable to study changes in serum bile acids during cholecystokinin/secretin infusion because of the high level of bile acid contamination in Boots Secretin. Some patients showed raised fasting serum bile acid concentrations more than two years before changes in conventional liver function tests or clinically evident liver disease. We have shown fasting serum bile acids to be a sensitive measure of liver dysfunction in cystic fibrosis and postulate that raised proportions of glycine conjugated bile acids may be responsible for the high incidence of liver disease in cystic fibrosis.

Topics: Adolescent; Adult; Bile Acids and Salts; Child; Child, Preschool; Cholecystokinin; Cystic Fibrosis; Duodenum; Fasting; Female; Humans; Infant; Intestinal Secretions; Male; Secretin

Topics: Child; Child, Preschool; Cholecystokinin; Cystic Fibrosis; Humans; Infant; Pancreas; Pancreatic Function Tests; Pancreatic Polypeptide

Ultrastructural and microvillous enzyme (MVE) histochemical studies of fetuses with cystic fibrosis (CF) and trisomies 13 and 18 identified features in CF which differed from the abnormalities in trisomies 13 and 18. The principal abnormalities in CF were in the tight (occluding) junctions and intracellular organelles, particularly the golgi and mitochondria, of the epithelial cells of the pancreas, respiratory system, intestine, and gallbladder. Abnormalities of amniotic fluid MVE levels in CF and trisomy 13 occur because of disruption of the pathways by which the MVE reach the amniotic fluid. Trisomy 18 shows hypoplasia and deficiency of epithelial cell microvilli. It is postulated that the basic defect in CF is due to the deficiency of an enzyme that cleaves the Arg-Asp peptide bond in cholecystokinin to produce the active octapeptide CCK-8, which normally stimulates exocrine secretion, especially in pancreas, gallbladder, and intestine, and potentiates the action of other gastrointestinal hormones.

Topics: Amniotic Fluid; Cholecystokinin; Chromosomes, Human, 13-15; Chromosomes, Human, 16-18; Congenital Abnormalities; Cystic Fibrosis; Female; Gallbladder; Humans; Intestines; Male; Microvilli; Pancreas; Peptide Hydrolases; Pregnancy; Prenatal Diagnosis; Respiratory System; Trisomy

Topics: Cholecystokinin; Cystic Fibrosis; Female; Fetus; Humans; Male; Pregnancy

Topics: Cholecystokinin; Cystic Fibrosis; Humans

Topics: Cholecystokinin; Cystic Fibrosis; Humans

It has been claimed that plasma cholecystokinin (CCK) concentrations are raised in patients with pancreatic insufficiency. We have measured plasma CCK concentrations in 32 patients with pancreatic insufficiency (22 alcoholic pancreatitis and 10 cystic fibrosis) and in 30 normal subjects by radioimmunoassays using antibodies with different specificities. Antibody 1703 binds to COOH-terminal forms of CCK containing at least 14 amino acid residues and does not cross-react with gastrins. Antibody T204 binds to all CCK-peptides containing the sulfated tyrosyl region and shows low cross-reactivity with sulfated gastrins but no binding to nonsulfated gastrins. Antibody 5135 binds to all COOH-terminal CCK-peptides and shows full cross-reactivity with gastrins. In patients with pancreatic insufficiency, plasma CCK concentrations (1.2 +/- 0.1 pmol/liter, antibody 1703; 2.0 +/- 0.2 pmol/liter, antibody T204; 12.5 +/- 1.4 pmol/liter, antibody 5135) were not significantly different from those in normal subjects (1.1 +/- 0.1 pmol/liter, antibody 1703; 2.2 +/- 0.3 pmol/liter, antibody T204; 10.5 +/- 0.9 pmol/liter, antibody 5135). Furthermore, plasma CCK concentrations in patients with pancreatic insufficiency due to alcoholic pancreatitis (1.2 +/- 0.1 pmol/liter, antibody 1703; 1.9 +/- 0.2 pmol/liter, antibody T204; 14.0 +/- 1.9 pmol/liter, antibody 5135) were not significantly different from those in patients with cystic fibrosis (1.2 +/- 0.2 pmol/liter, antibody 1703; 2.4 +/- 0.4 pmol/liter, antibody T204, 9.1 +/- 1.0 pmol/liter, antibody 5135). Cross-reactivity with gastrin accounted for almost all CCK-like-immunoreactivity measured with antibody 5135.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Adult; Aged; Alcoholism; Antibodies; Antibody Specificity; Binding Sites, Antibody; Cholecystokinin; Cross Reactions; Cystic Fibrosis; Diabetes Mellitus; Exocrine Pancreatic Insufficiency; Female; Gastrins; Humans; Male; Middle Aged; Pancreatitis; Radioimmunoassay

Responses of 11 gastrointestinal regulatory peptides to a standard test meal were assessed in 10 adult patients with cystic fibrosis. The basal plasma neurotensin was significantly elevated in patients with cystic fibrosis, being 31.5 +/- 6.1 pmol/L compared with a control value of 10.3 +/- 1.5 pmol/L (p less than 0.005). Plasma neurotensin remained elevated throughout the test period. Basal plasma enteroglucagon was similarly elevated, the patients with fibrocystic disease having levels of 51.3 +/- 4.6 pmol/L compared to controls with levels of 33.2 +/- 6.7 pmol/L (p less than 0.02). There was, however, no significant difference in postprandial levels of plasma enteroglucagon. Postprandial motilin was significantly elevated in the patients with cystic fibrosis; this elevation is in contrast with previous findings in children. Release of gastric inhibitory polypeptide was impaired, while release of cholecystokinin showed no significant difference in control values, although there was a tendency for delay. There was no significant postprandial rise of pancreatic polypeptide in the patients, whose levels were grossly lower than controls. Insulin showed a delayed response. No significant differences were observed between patients and controls in levels of gastrin, pancreatic glucagon, somatostatin, or vasoactive intestinal peptide. The elevation of plasma neurotensin and enteroglucagon in the basal state may reflect an adaptive response and may be part of the improved digestive function in adults compared with children with fibrocystic disease.

Topics: Adult; Cholecystokinin; Cystic Fibrosis; Female; Gastric Inhibitory Polypeptide; Gastrointestinal Hormones; Glucagon; Humans; Male; Neurotensin; Pancreatic Polypeptide

Topics: alpha-Amylases; Cholecystokinin; Cystic Fibrosis; Female; Heterozygote; Humans; Male; Pancreatin; Secretin; Trypsinogen

Duodenal fluids from control and cystic fibrosis (CF) patients were assayed for enterokinase (EK), trypsin and chymotrypsin activities. CF patients as a group were found to have higher basal EK activity in spite of low trypsin and chymotrypsin activities. In control patients, pancreozymin (CCK) injection led to increases in specific activities of trypsin and chymotrypsin and a decrease in EK but did not change the total EK activities. Secretin administration led to decreases in specific activities of trypsin and chymotrypsin compared to post-CCK levels. The total EK activities were greatly increased following secretin administration. Thus, secretin may have direct influence on the release of EK into the duodenum. CCK and secretin have no effect on the specific activities of trypsin, chymotrypsin and EK in CF patients. EK release in CF patients is either constitutive and therefore not affected by CCK and secretin or it has been fully induced by the low trypsin content and becomes unresponsive to further hormonal stimulation.

Topics: Adolescent; Child; Child, Preschool; Cholecystokinin; Chymotrypsin; Cystic Fibrosis; Duodenum; Endopeptidases; Enteropeptidase; Humans; Infant; Proteins; Secretin; Trypsin

We have studied the secretin-pancreozymin stimulation test of pancreatic function with particular regard to its usefulness in the diagnosis of cystic fibrosis patients without gastrointestinal symptoms. We have compared the test results in 38 control individuals, 14 patients with cystic fibrosis and five other patients with problems affecting pancreatic function. Of the cystic fibrosis patients tested, six had no gastrointestinal symptoms and were shown to have normal or raised output of pancreatic enzymes measured (lipase, trypsin, chymotrypsin, and amylase). There was a positive correlation between both volume and bicarbonate measurement versus body weight in all subjects, but this relationship was clearly different in the cystic fibrosis patients compared with others regardless of the cystic fibrosis patients' ability to secrete pancreatic enzymes. Among the cystic fibrosis patients, bicarbonate concentration tended to be higher in those able to secrete a significant amount of pancreatic enzymes. However, the actual output of bicarbonate measured in all cystic fibrosis patients (range 0.001-0.037 mmol/kg/45 min post-stimulation) was below that found in all control patients (range 0.104-0.516 mmol/kg/45 min post-stimulation). Therefore the secretin-pancreozymin test of pancreatic function appears to be useful in the diagnosis of cystic fibrosis even in those patients with adequate enzyme production.

Topics: Adolescent; Adult; Bicarbonates; Child; Child, Preschool; Cholecystokinin; Cystic Fibrosis; Female; Humans; Infant; Male; Pancreas; Pancreatic Juice; Secretin; Trypsin

Alterations in the pancreatic secretion of fluid and of enzymes in response to either pilocarpine (15 mg/kg) or an octapeptide of cholecystokinin (0.1 microgram/kg) have been found in rats that received daily injections of reserpine (0.5 mg/kg) for 7 days. During a 3-hr secretory period, significant reductions in the volume of pancreatic juice and in the total output of protein, amylase, and trypsin were observed in these animals. In the first hour of the secretory response, however, protease output was increased in the treated animals, particularly that of chymotrypsin, which was also increased in the longer secretory period following pilocarpine, but not cholecystokinin, stimulation. Zymogen granules isolated from the pancreas of the treated rats by differential centrifugation in a 0.3 M sucrose buffer had increased specific activities of the proteases when compared to those of untreated controls. Ultrastructurally, zymogen granules isolated from the pancreas of the treated rats showed changes in density, with bizonal and trizonal configurations being frequently observed, and had less distinct limiting membranes. In some, the membrane appeared broken at intervals, and there was granular material, presumably derived from the granule contents, lining the surface of the granule. It is concluded that pretreatment with reserpine inhibits fluid secretion and alters enzyme secretion in the rat exocrine pancreas. The latter effect is related to a nonparallel storage of amylase and proteases in the secretory granules induced by the drug treatment, probably through an action on protein synthesis or intracellular transport. An accumulation of proteases may lead to activation of these enzymes and to granule lysis. Inasmuch as the reserpine-treated rat has been proposed as an experimental model for cystic fibrosis, these findings are relevant in terms of possibly pathogenetic mechanisms in this disease.

Topics: Amylases; Animals; Cholecystokinin; Chymotrypsin; Cystic Fibrosis; Disease Models, Animal; Male; Pancreas; Pilocarpine; Rats; Reserpine; Trypsin

Cholesterol gallstones occur 600-1000 times more frequent in adults than in children. Seeking a possibility to explain this discrepancy, the molar concentrations of cholesterol, lecithin and bile acids as well as the bile acid pattern have been determined in duodenal juice, after an injection of 2 U/kg pancreocymin, of 33 children aged between 4 months and 14 years who were gastroenterologically healthy. The absolute values as well as the percentage composition were calculated. The lithoindices have been determined to be Li1=0.54 +/- 0.25 and Li2=1.06 +/- 0.50 according to the formulas of Thomas and Hofmann. Dependence on age in infancy and during childhood could be excluded. The main reason for the fact that hardly any gallstones occur among children, compared to the occurrence in adults seems to be the small concentration of cholesterol in the bile. Whereas there had been no deviations either of lithoindices or in the bile acid pattern in 10 patients with coeliac disease, 2 out of 6 children with mucoviscidosis and 3 out of 4 children with small bowel syndrome showed apparently increased lithoindices. Only in the last group a bile acid pattern was found which could reduce the higher risk of getting cholelithiasis.

Topics: Adolescent; Age Factors; Bile Acids and Salts; Celiac Disease; Child; Child, Preschool; Cholecystokinin; Cholelithiasis; Cholesterol; Cystic Fibrosis; Germany, West; Humans; Infant; Intestinal Secretions; Intestine, Small; Phosphatidylcholines

Topics: Bile; Child; Cholecystokinin; Cholestasis; Cyclic AMP; Cystic Fibrosis; Humans; Pancreatic Juice; Secretin

A diagnosis of cystic fibrosis was incorrectly made after false-positive sweat tests in 14 children. 13 of these children had been tested at hospitals where it seems likely that sweat tests were not done very often. All the children had normal sweat-electrolyte values when the test was repeated at a regional paediatric centre where approximately 250 sweat tests are done each year. In 5 cases, detailed testing of pancreatic function was normal. None of the children had typical chest disease and only 2 had gastrointestinal symptoms. In the absence of the typical clinical features of the disease a diagnosis of cystic fibrosis should be made with extreme caution and only after meticulous testing of both sweat electrolytes and pancreatic function.

Topics: Amylases; Bicarbonates; Child; Child, Preschool; Chlorides; Cholecystokinin; Cystic Fibrosis; Evaluation Studies as Topic; False Positive Reactions; Humans; Lipase; Pancreas; Secretin; Sodium; Sweat; Trypsin

Topics: Amylases; Animals; Cholecystokinin; Cystic Fibrosis; Disease Models, Animal; Electrolytes; Male; Organ Size; Pancreatic Juice; Proteins; Rats; Reserpine; Secretin; Time Factors

Ten adolescent and young adults with cystic fibrosis (CF) have had well-documented recurrent attacks of acute pancreatitis. The diagnosis of CF in each patient was delayed because they did not have pancreatic insufficiency. The diagnosis of CF was documented by the typical pulmonary involvement and elevated sweat sodium and chloride levels in all cases and a positive family history in six of the ten patients. Two patients were diagnosed as having acute pancreatitis before the diagnosis of CF was made, thus indicating that acute pancreatitis may be the presenting complaint in the young adult with CF. The diagnosis of acute pancreatitis was based on the presence of severe abdominal pain, usually with vomiting, tenderness in the mid-epigastrium, elevated serum and urinary amylase and serum lipase. Attacks were precipitated by fatty meals, alcohol ingestion; postcholecystectomy and tetracycline administration. In some patients no precipitating event could be elicited. Intravenous secretin-pancreozymin stimulation tests revealed a diminished bicarbonate secretion with little effect on the secretion of the zymogen enzymes. A mild attack of pancreatitis occurred after secretin-pancreozymin stimulation. The endocrine pancreatic function tested in four patients was normal as revealed by the glucose tolerance tests and determinations of serum insulin, growth hormone and free fatty acid. Transduodenal pancreatograms were performed in three patients; one showed a normal pancreatic duct, one showed duct obstruction and in the third patient a beady type of narrowing was found. The selenomethionine Se 75 uptake of the pancreas was noted only in the head of the pancreas. This suggests that loss of function occurs initially to a greater extent in the tail and body of the pancreas. Three patients died and showed characteristic lesions of CF.

Topics: Acute Disease; Adolescent; Adult; Amylases; Chlorides; Cholecystokinin; Chymotrypsin; Cystic Fibrosis; Female; Glucose Tolerance Test; Humans; Intubation, Gastrointestinal; Lipase; Magnesium; Male; Methionine; Pain; Pancreas; Pancreatitis; Potassium; Recurrence; Secretin; Selenium; Sodium; Trypsin

Topics: Age Factors; Body Height; Body Weight; Child; Cholecystokinin; Cystic Fibrosis; Female; Humans; Infant; Male; Pancreas; Pancreatic Diseases; Secretin

Topics: Adolescent; Bile Acids and Salts; Celiac Disease; Child; Child, Preschool; Cholecystokinin; Clinical Enzyme Tests; Cystic Fibrosis; Duodenum; Female; Gallbladder; Humans; Infant; Male; Pancreas; Pancreatic Diseases; Pancreatic Juice; Secretin; Stimulation, Chemical

Topics: Adolescent; Bicarbonates; Calcium; Child; Child, Preschool; Cholecystokinin; Cystic Fibrosis; Duodenum; Humans; Infant; Intestinal Secretions; Magnesium; Pancreas; Potassium; Proteins; Secretin; Sodium; Stimulation, Chemical; Water-Electrolyte Balance

Topics: Adolescent; Amylases; Bicarbonates; Child; Child, Preschool; Cholecystokinin; Chymotrypsin; Cystic Fibrosis; Feces; Female; Humans; Infant; Infant, Newborn; Intubation, Gastrointestinal; Lipase; Male; Pancreas; Pancreatic Juice; Secretin; Trypsin; Viscosity

Topics: Adolescent; Amylases; Carboxypeptidases; Child; Child, Preschool; Cholecystokinin; Cystic Fibrosis; Duodenum; Electrophoresis; Humans; Immunodiffusion; Immunoelectrophoresis; Infant; Methods; Pancreas; Pancreatic Diseases; Proteins; Secretin; Trypsin

Topics: Adolescent; Adult; Amylases; Bicarbonates; Carboxypeptidases; Child; Child, Preschool; Chlorides; Cholecystokinin; Chymotrypsin; Cystic Fibrosis; Female; Humans; Lipase; Male; Pancreatic Juice; Secretin; Trypsin

Topics: Adolescent; Age Factors; Amylases; Bicarbonates; Body Weight; Child; Child, Preschool; Cholecystokinin; Cystic Fibrosis; Duodenum; Exocrine Glands; Humans; Infant; Intubation, Gastrointestinal; Pancreas; Pancreatic Diseases; Pancreatic Juice; Secretin; Water

Topics: Amylases; Bicarbonates; Cholecystokinin; Cystic Fibrosis; Diarrhea; Gastrointestinal Hormones; Hemochromatosis; Humans; Liver Cirrhosis; Pancreas; Pancreatic Juice; Pancreatic Neoplasms; Pancreatitis; Physiology; Secretin

Topics: Amylases; Bicarbonates; Cholecystokinin; Cystic Fibrosis; Diarrhea; Gastrointestinal Hormones; Hemochromatosis; Humans; Liver Cirrhosis; Pancreas; Pancreatic Juice; Pancreatic Neoplasms; Pancreatitis; Pharmacology; Physiology; Secretin