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At least 199 records · Page 11Linked to original sources
PubMed2001

Idiopathic acute recurrent pancreatitis.

Acute recurrent pancreatitis (ARP) results most commonly from alcohol abuse or gallstone disease. Initial evaluation fails to detect the cause of ARP in 10-30% of patients, and as a result the diagnosis of "idiopathic" ARP is given. In these patients, a more extensive evaluation including specialized labs, ERCP, endoscopic ultrasound, or magnetic resonance cholangiopancreatography typically leads to a diagnosis of microlithiasis, sphincter of Oddi dysfunction, or pancreas divisum. Less commonly, hereditary pancreatitis, cystic fibrosis, a choledochocele, annular pancreas, an anomalous pancreatobiliary junction, pancreatobiliary tumors, or chronic pancreatitis are diagnosed. Determining the etiology is important, as it helps to direct therapy, limits further unnecessary evaluation, and may improve a patient's long term prognosis.

Acute Disease↗
PubMed1990

Independent genetic determinants of pancreatic and pulmonary status in cystic fibrosis.

The concordance of pancreatic and pulmonary status among siblings with cystic fibrosis, the cystic fibrosis genotype, and non-genetic factors were analysed in adult patients with cystic fibrosis. Genetic factors were more important than non-genetic factors in affecting the severity of pancreatic and lung disease. The genetic factors were independent of each other and did not invariably cosegregate with specific mutations at the cystic fibrosis locus.

Adult↗
PubMed1999

The duct cell in cystic fibrosis.

The pancreatic duct cell is central to the etiology of cystic fibrosis (CF) and is the site where pathology commences in utero. We have evaluated expression of the cystic fibrosis transmembrane conductance regulator gene (CFTR) through human development and shown it to be expressed from the early mid-trimester, with highest levels in the most distal portion of the developing duct system and in centroacinar cells. The precise cause of pancreatic destruction in CF is thought to be the obstruction of pancreatic ducts with inspissated secretions. We have shown that the MUC6 mucin is a significant component of the material that obstructs the ducts and that the MUC6 gene shows a very similar pattern of expression to that of CFTR in the developing pancreas. These observations provide a starting point for investigating how mutations in CFTR lead to obstruction of the pancreatic ducts in CF.

Cystic Fibrosis↗
PubMed1988

Effects of reserpine treatment on dietary adaptation of the rat exocrine pancreas.

Chronic reserpine treatment (500 micrograms/kg) of the rat results in generalized exocrinopathy, impaired pancreatic secretion, and decreased pancreatic amylase. These characteristics are similar to those in cystic fibrosis and are the basis for use of this experimental model for cystic fibrosis. Pancreatic enzymes adapt to diet, but it is not known whether chronic reserpine treatment affects this response. Due to the malnutrition induced by this treatment, another dose of reserpine was required that would alter pancreatic function but not induce malnutrition in order to evaluate dietary adaptation. Male rats (100-120 g) were injected subcutaneously daily for 7 days with 1) no injection (control); 2) 1.0 ml/kg vehicle or sham (pair fed-sham); or 3) reserpine: 500, 50, or 5 micrograms/kg. Food consumption was comparable among control and reserpine-treated (50 and 5 micrograms/kg) rats and significantly greater (200%) than pair fed-sham and 500 micrograms/kg reserpine-treated rats. Pancreatic amylase, however, was significantly lower in all reserpine-treated rats (500 micrograms/kg, 74%; 50 micrograms/kg, 56%; 5 micrograms/kg, 52%) than in control rats. To evaluate dietary adaptation, control and reserpine-treated (5 micrograms/kg) rats were fed high carbohydrate, high fat or high protein diets. Both groups adapted to these diets with the greatest amylase, lipase, and trypsin activities in high carbohydrate-, high fat-, and high protein-fed rats, respectively. Reserpine-treated rats fed high carbohydrate, however, had significantly lower (64%) amylase activity than high carbohydrate-fed control rats. Although reserpine-treated rats can adapt pancreatic enzymes to diet, the adaptation of amylase to carbohydrate is impaired.

Amylases↗
PubMed1985

Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis.

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.

Adolescent↗
PubMed1980

Fatty acid composition of erythrocyte phospholipids from patients with cystic fibrosis.

The phospholipid fatty acid composition of erythrocytes from patients with cystic fibrosis with pancreatic insufficiency, patients with cystic fibrosis with normal pancreatic enzyme function, and normal subjects was determined. Increased percentages of palmitoleic acid and total eicosatrienoic acid and a decreased percentage of linoleic acid were observed only in the cystic fibrosis patients with pancreatic insufficiency.

Adolescent↗
PubMed1987

C. L. Oakley lecture (1987). The pathogenesis of beta cell destruction in type I (insulin-dependent) diabetes mellitus.

In a study of pancreases from 75 patients who died at presentation of Type I diabetes there was selective destruction of beta cells associated with islet inflammation (insulitis). According to a recent hypothesis, aberrant expression of Class II major histocompatibility complex (MHC) products on a target cell may allow presentation of organ specific surface antigen(s) to potentially autoreactive T helper lymphocytes and thus lead to autoimmunity. Aberrant expression of Class II MHC was demonstrated immunohistochemically on beta cells in 21 out of 23 patients with recent onset diabetes. No such expression was seen on the other pancreatic endocrine cells. Ninety-four per cent of insulin-containing islets in these patients had marked hyperexpressions of Class I MHC affecting all endocrine cells in these islets. Insulin deficient islets were not thus affected. Both these abnormalities of MHC expression appeared to precede insulitis within a given islet and appeared to be unique to Type I diabetes, being absent in pancreases of patients with Type II diabetes, chronic pancreatitis, cystic fibrosis, graft-versus-host disease and Coxsackie B viral pancreatitis. The development of autoimmunity to beta cells in Type I diabetes may be a 'multistep' process in which abnormalities of MHC expression are crucial events.

Adult↗
PubMed1987

Aberrant expression of class II major histocompatibility complex molecules by B cells and hyperexpression of class I major histocompatibility complex molecules by insulin containing islets in type 1 (insulin-dependent) diabetes mellitus.

Twenty-three patients with recent onset Type 1 (insulin-dependent) diabetes in whom residual insulin secreting B cells were present and 12 patients with disease of more prolonged duration (maximum 9 years), 8 of whom had residual B cells, were studied. Aberrant expression of Class II major histocompatibility complex molecules was demonstrated immunohistochemically on insulin secreting B cells in 21 out of 23 patients with recent onset disease and 6 of the patients with more prolonged disease. No such expression was seen on glucagon secreting A cells or somatostatin secreting D cells. Islets where there was marked hyperexpression of Class I major histocompatibility complex molecules on islet endocrine cells were seen in all cases in which residual B cells were present. Ninety-two per cent of insulin containing islets but only 1% of insulin deficient islets exhibited this phenomenon (p less than 0.001, Chi-squared test). There was evidence to suggest that both these abnormalities of major histocompatibility complex expression preceded insulitis within a given islet. They also appeared to be unique to Type 1 diabetes, being absent in pancreases of patients with Type 2 (non-insulin-dependent) diabetes, chronic pancreatitis, cystic fibrosis, graft-versus-host disease and Coxsackie B viral pancreatitis. The development of autoimmunity to B cells in Type 1 diabetes may be a "multistep" process in which abnormalities of major histocompatibility complex expression on islet endocrine cells are crucial events.

Adolescent↗
PubMedDate not supplied

[Exocrine pancreatic insufficiency. Organic and functional deficiencies].

The exocrine pancreas is a gland which secretes water, enzymes and electrolytes into the intestinal lunar. These enzymes for the normal digestion of food and a deficit, whether due to a seduced secretion (chronic pancreatitis, cystic fibrosis), or dysfunction in the chronology of their secretion (following truncal vagotomy) will cause malabsorption which often develops, in clinical terms, into malnutrition sometimes this functional deficit is secondary to other pathologies or surgical operations which alter the digestive tract physiology, preventing the correct combination of nutrients and pancreatic enzymes together with the biliary salts. The outcome is malabsorption, mainly of fast and fat soluble vitamins. As the onset is slow and forms part of a chronic, pathology, diagnosis is difficult, for which reason it is included in the general group of malabsorption due to pancreatic insufficiency or surgical sequelae, finally resulting in pancreatic failure or problems following surgery. The purpose of this publication is to review one by one all the situations in which there is an alteration in the function of the pancreatic enzymes, with emphasis on cases in which a defined malabsorption syndrome will result, and in which the prescription of exogenous pancreatic enzymes will imposiue the picture.

Chronic Disease↗
PubMed1995

Hepatobiliary disease in cystic fibrosis patients with pancreatic sufficiency.

Focal and multilobular biliary cirrhosis are considered pathognomonic of cystic fibrosis (CF) and almost invariably have been reported in patients with steatorrhea. In contrast, patients with pancreatic sufficiency and normal absorption are considered less likely to develop liver or biliary tract problems. The authors report three patients with CF and pancreatic sufficiency, presenting with recurrent abdominal pain (unrelated to pancreatitis). All had common bile duct disease, one with multilobular cirrhosis and portal hypertension. Pancreatic sufficiency was proven by quantitative pancreatic stimulation tests, 3-day fecal fat analyses, and serum pancreatic isoamylases. All three patients had mild lung disease. Two were homozygous for the common delta F508 mutation, and the other, a delta F508 compound heterozygote. Hepatobiliary structure and function were determined by serial hepatobiliary scintigraphy, percutaneous transhepatic cholecystography, and biochemical liver function tests. Patients 1 and 3 had mild hepatomegaly, normal liver biochemistry, and distal common bile duct strictures. Patient 2 had a firm nodular liver with splenomegaly, abnormal liver biochemistry, and a cholangiographic appearance of sclerosing cholangitis. All have undergone operative treatment for persistent abdominal pain. These cases confirm the occurrence of common bile duct pathology and liver disease in patients with CF and pancreatic sufficiency. They demonstrate that liver and biliary tract disease can occur independently of the underlying disease severity and the presence of steatorrhea. Further, they suggest that obstruction of the biliary tract may be an additional factor in the evolution of liver disease in CF.

Biliary Tract Diseases↗
PubMed1996

Cystic fibrosis: MR assessment of pancreatic damage.

PURPOSE: To evaluate magnetic resonance (MR) imaging for assessment of pancreatic damage in cystic fibrosis. MATERIALS AND METHODS: Twenty-seven patients with cystic fibrosis and 12 control subjects underwent T1-weighted imaging for visual assessment for pancreatic hyperintensity and mixed spin-echo-inversion-recovery imaging for quantitative measurement of T1. Pancreatic insufficiency, pulmonary status, and genotype were recorded. Statistical correlation was conducted. RESULTS: Four patterns of pancreatic involvement were noted: diffuse hyperintensity with a lobular pattern, diffuse homogeneous hyperintensity without residual lobular pattern, hyperintensity with focal areas of sparing, and no structural or signal intensity changes. Statistically significant reduction of the T1 was noted in 21 patients compared with that of controls. Statistically significant correlation between T1 shortening and pulmonary clinical-radiologic compromise and pancreatic insufficiency was found. CONCLUSION: MR imaging may offer further indication of pancreatic and, indirectly, pulmonary damage during the clinical course of cystic fibrosis. A pattern of fibrofatty infiltration of the pancreas, of undetermined clinical significance, is reported.

Adolescent↗
PubMed1977

Rickets in adult cystic fibrosis with myopathy, pancreatic insufficiency and proximal renal tubular dysfunction.

Rickets is reported in a 19 year old white man with cystic fibrosis in whom pancreatic and hepatic involvement was advanced. There was evidence of secondary hyperparathyroidism with proximal renal tubular acidosis, aminoaciduria, phosphaturia and hypophosphatemia. Treatment with oral pancreatic and parenteral vitamin D supplements led to full recovery of the rachitic syndrome and the proximal renal tubular dysfunction.

Adult↗
PubMed1998

Hereditary pancreatitis: report of a family from Turkey.

Chronic pancreatitis is a rare disease in children and is usually secondary to underlying diseases such as hereditary pancreatitis, cystic fibrosis, hyperlipidemia, prolonged malnutrition, gallstones or anomalies of the biliary-pancreatic duct system. Hereditary pancreatitis is a common cause of chronic pancreatitis in children but is often unrecognized until months or years later. We report here a family with hereditary pancreatitis in which four members are affected.

Adult↗
PubMed1976

Relationship between bile acid malabsorption and pancreatic insufficiency in cystic fibrosis.

Bile acid loss (mg/m2 24h) in the stools of 43 cystic fibrosis (CF) children with pancreatic insufficiency was 751-1 +/- 48-3, while that of six without clinical evidence of pancreatic disease (133-4 +/- 15-9) did not differ from values in 25 controls (109-8 +/- 9-8). There was a good correlation between the degree of bile acid (BA) and fat sequestration. Concomitant changes in bile acid and fat loss were observed in the one group of six patients studied on and off pancreatic enzymes as well as in a second group of seven children treated with pancreatic supplements and maintained on a normal diet followed by a low fat diet supplemented with medium chain triglycerides. Administration of NA bicarbonate led to a significant decrease in fat loss (15-8 +/- 2-7 leads to 10-3 +/- 1-9) without any simultaneous change in bile acid excretion (533-1 +/- 58-3 leads to 500-4 +/- 58-6). Qualitative bile acid patterns in controls, in infants after an ileal resection, and in patients with CF or with coeliac disease showed that the percentage of primary BA followed closely the total amount excreted except in situations where antibiotics were administered. The exact mechanism for the increased loss of BA in CF is unknown. It is found in all age groups and is related to the presence and degree of pancreatic insufficiency. The possibility that unhydrolysed triglycerides may interfere with the intestinal absorption of bile acid needs further confirmation.

Adolescent↗
PubMed1975

Electrophoretic separation, detection, and variation of amylase isoenzymes.

An electrophoretic technique for demonstrating amylase isoenzymes is described. After separation in an agarose gel containing a linear polyacrylamide polymer to reduce electroendosmotic flow, the amylase fractions are visualized by incubation with a commercially available dye-starch polymer (Phadebas Amylase Test). The technique detects amylase fractions with activities below 10 U/l. Some characteristic changes in such diseases as acute and chronic pancreatitis, cystic fibrosis of the pancreas, macroamylasemia and inherited variants as well as after maxillofacial surgery are mentioned.

Acute Disease↗