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Query: UMLS:C0149521 (chronic pancreatitis)
 7,199 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic pancreatitis (adults) and cystic fibrosis (children) are the most common diseases leading to exocrine pancreatic insufficiency that, when reduced to < 5% of normal function, is characterised by steatorrhoea. The pathogenesis of the former condition is outlined, and recent concepts are emphasized. Biochemical tests to detect pancreatic insufficiency and to identify pancreatic disease as the cause of steatorrhoea include: serum enzyme tests (lipase, amylase, trypsin); stool chymotrypsin; isotopic tests based upon the assimilation of [14C] lipids and starch or excretion of the isotope as breath CO2, as well as the dual-labelled Schilling test; oral function tests utilising substrates hydrolysed by pancreatic enzymes such as benzoyl tyrosyl-p-aminobenzoic acid and fluorescein dilaurate; and duodenal intubation studies following meal-induced or hormonal stimulation of the pancreas. The rationale for these tests and the cumulative clinical experience of their utility are reviewed. A recommended diagnostic strategy is briefly presented. The role of various biochemical procedures to evaluate the efficacy of pancreatic enzyme replacement therapy is also described.
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PMID:Biochemical tests in the diagnosis of chronic pancreatitis and in the evaluation of pancreatic insufficiency. 824 88

The authors consider the different strategies in long term enzyme replacement therapy in relation to the complex mechanics at the basis of pancreas exocrine insufficiency. This condition occurs in chronic pancreatitis and is present in Cystic Fibrosis, the most common potentially lethal inherited disorder of Caucasians. Pancreatic exocrine insufficiency occurs in the vast majority of cystic fibrosis affected children and is now becoming a frequent pathology in adults for the better life expectancy and the recent acknowledgements in this disease. The principal aims of research in enzyme replacement therapy have been directed at the formulation of products with high enzyme concentration, to the prevention of gastric acid inactivation of enzymes and to the better mixing of the preparations with meals. The authors consider all the different enzyme preparations from pancreatin powder to the 1st. generation of enteric coated tablets and examine the advantages of administering H-2 receptor antagonists or antacids and the possibility of stimulating bicarbonate secretion as an adjunct to pancreatic enzyme replacement therapy. Significant benefits in pancreatic insufficiency therapy have derived from the introduction of enteric coated microspheres which ensure a consistent level of enzymes to reach the duodenum mixed with the meal and which are resistant to gastric acid inactivation as well.
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PMID:[Pancreatic extract therapy in exocrine pancreatic insufficiency]. 828 85

NIDDM is a heterogeneous disease and subgroups of NIDDM include MODY (Maturity Onset Diabetes of the Young), Malnutrition-related diabetes (MRDM) and Fibrocalculus pancreatic diabetes (FCPD). Endocrine cell population is relatively unchanged in NIDDM: B-cells are reduced by up to 30% and A-cells increased by 10%. Islet amyloid is found in 96% of subjects occupying up to 80% of the islet associated with a reduction in B-cells. Amyloid formation is unlikely to cause diabetes but progressive accumulation increases the severity of the disease. Islet amyloid is formed from the islet amyloid polypeptide (IAPP), a normal constituent of B-cells, co-secreted with insulin. The causal factors for IAPP fibrillogenesis are unknown but abnormal synthesis or overproduction could be involved: stimulation of B-cell secretion in NIDDM by obesity, hyperglycaemia or suphonylurea therapy may promote amyloidosis and further aggravate islet pathology. A mutation of the glucokinase gene in MODY leads to diminished B-cell secretion but not amyloid formation. Diabetes and mutations of mitochondrial DNA is associated with poorly developed islet structure. Exocrine pancreatic size is reduced and there is evidence of sub-clinical chronic pancreatitis in NIDDM. In MRDM and FCPD, chronic pancreatitis and exocrine necrosis is associated with reduced insulin secretion. Unlike cystic fibrosis where islet amyloid is present in diabetic individuals, amyloid is absent from subjects with FCPD. Pathological changes in the exocrine and endocrine pancreas in NIDDM results from and contributes to the pathophysiology of insulin secretion in NIDDM.
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PMID:Pancreatic pathology in non-insulin dependent diabetes (NIDDM). 852 18

Disturbances of the intestinal integrity, reflected by an increased intestinal permeability, are reported in cystic fibrosis (CF). Controversy exists whether the increased intestinal permeability is due to CF itself or a consequence of the concomitant exocrine pancreatic insufficiency (PI). We measured intestinal permeability by the sugar absorption test in 32 PI patients: 20 CF-PI, 12 nonCF-PI with chronic pancreatitis, and 50 controls. In the sugar absorption test, the lactulose/mannitol ratio is measured in 5-h urine samples after oral ingestion of a solution of lactulose and mannitol, hyperosmolar by the addition of sucrose. The lactulose/mannitol ratio was increased in both CF-PI and nonCF-PI versus controls (p < 0.0001). In CF, the L/M ratio and permeability for lactulose and mannitol did not change by increasing pancreatic enzyme supplementation by 30-50% for 2 wk (p = 0.74, p = 0.97, p = 0.74, respectively) nor by decreasing the osmolarity of the test solution by 75% (p = 0.24, p = 0.10, p = 0.39, respectively). We conclude that an increased intestinal permeability in CF is probably a consequence of PI and is not related to the dose of pancreatic enzyme supplementation nor the osmolarity of the test solution. The increase is due to an increased permeability for lactulose which might point toward a defect in the tight junctions of the villi and/or crypts. The cause of the increased intestinal permeability in the presence of PI is still unclear. An increased intestinal permeability points toward an impaired functional integrity of the small bowel, which may contribute to gastrointestinal dysfunction in CF.
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PMID:Intestinal permeability in exocrine pancreatic insufficiency due to cystic fibrosis or chronic pancreatitis. 872 59

The spectrum of inherited and congenital disorders of the exocrine pancreas is described. Although functional disturbances of the exocrine pancreas are less frequent in childhood than in adult life, there is a wide spectrum of causes, many of which are genetic or congenital in origin. Rarely, a disturbance of pancreatic function may arise as a result of disordered embryogenesis. More frequently, however, inherited disorders affecting multiple organs give rise to pancreatic dysfunction. Among Caucasian children, cystic fibrosis (CF) is, by far, the most common inherited disorder of disturbed pancreatic function. Examples of rarer, inherited causes of pancreatic dysfunction include Shwachman-Diamond syndrome, Johanson-Blizzard syndrome, Pearson's syndrome, pancreatic agenesis, and isolated enzyme deficiencies. Hereditary pancreatitis and several recently recognized metabolic causes of chronic pancreatitis can also produce severe pancreatic exocrine dysfunction.
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PMID:Inherited and congenital disorders of the exocrine pancreas. 889 82

In children with pancreatic disease, computed tomography (CT) has a primary role in the evaluation of pancreatitis, trauma, and malignancy. At CT, pancreatic abnormalities may manifest as pancreatic enlargement (tumor, acute pancreatitis), pancreatic atrophy (cystic fibrosis, chronic pancreatitis), cystic lesions (pseudocysts, congenital simple cysts, autosomal dominant polycystic kidney disease, von Hippel-Lindau disease, cystic fibrosis, cystic neoplasms), or fatty replacement (cystic fibrosis, Shwachman-Diamond syndrome, history of steroid therapy, Cushing syndrome, Johanson-Blizzard syndrome, obesity). CT is the best modality for evaluation of pancreatitis, allowing detection of pancreatic abnormalities as well as abnormal extrapancreatic fluid collections. In children who have undergone blunt abdominal trauma, CT has been shown to be the best initial imaging study, being more sensitive than ultrasound for detection of pancreatic injury. In neoplastic conditions, CT demonstrates the extent of disease, enables characterization of the tissue components of the tumor, and allows accurate posttreatment follow-up. Although the various diseases of the pancreas may have overlapping appearances at CT, the correct diagnosis can often be made on the basis of the CT findings in combination with the clinical history, laboratory data, and the patient's age.
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PMID:Pancreatic disease in children and young adults: evaluation with CT. 974 14

Cystic fibrosis is by far the commonest cause of chronic pancreatitis in children, but pancreatitis itself is only rarely its presenting feature. In this paper an hypothesis for the development of the pancreatic lesions is presented. Impaired activation of pancreatic proteases in the small intestine is perceived as the pivotal problem that leads to continual feedback release of cholecystokinin, thus, in effect, causing a chronic hyperstimulation pancreatitis with intra-acinar activation of zymogens and, when bicarbonate secretion falls, precipitation of 'Reg' and other proteins in the duct system. This position contrasts with that in hereditary pancreatitis in which a mutation in the cationic trypsinogen gene leads to a form of trypsin that resists degradation by mesotrypsin and enzyme Y. A survey of the literature suggests that oxidant stress is a plausible contributor to pancreatic injury in both these diseases and in several other conditions linked with childhood pancreatitis.
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PMID:Paediatric and hereditary aspects of chronic pancreatitis. 983 35

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.
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PMID:Hereditary pancreatitis: report of a family from Turkey. 989 1

After a short introduction (chapter 1) methods of measuring gastrointestinal pH are described in chapter 2. The methods are divided into intubation techniques and tubeless methods, and the advantages and disadvantages are discussed. Measurements with pH-sensitive, radiotransmitting capsules are highlighted, and methodological problems with these capsules are described. Chapter 3 concerns the gastrointestinal pH profile of healthy subjects. The intraluminal pH is rapidly changed from highly acid in the stomach to about pH 6 in the duodenum. The pH gradually increases in the small intestine from pH 6 to about pH 7.4 in the terminal ileum. The pH drops to 5.7 in the caecum, but again gradually increases, reaching pH 6.7 in the rectum. The physiological background of these pH values is discussed. Chapter 4 describes the effect of gastrointestinal pH on bacterial flora, absorption of vitamins and electrolytes, and on the activity of digestive enzymes. The pH-profile in children is described in chapter 5. The profile is identical with that of adults, and it is therefore concluded that the release of a drug from pH-dependent, controlled-release preparations is also probably identical with that of adults. Chapter 6 describes the correlation between certain diseases and the gastrointestinal pH. A resection of the colon and the creation of an ileostomy do not affect the pH of the remaining gut. An ileocaecal resection shortens the small intestinal transit time, increases pH of the proximal colon, but does not change the pH-profile of the small intestine. Chronic pancreatitis and cystic fibrosis seem to decrease pH of the proximal small intestine. Very low colonic pH values have been observed in severe active ulcerative colitis and in Crohn's disease, but the background and clinical implication of this phenomenon are not clear. Chapter 7 describes the modulating effect of diet and drugs on gastrointestinal pH. Diet primarily has an effect on the colonic pH, whereas drugs might affect both small intestinal and colonic pH. The different effects are described. Finally, chapter 8 summarizes the present knowledge about gastrointestinal pH, and future investigations are proposed.
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PMID:Intraluminal pH of the human gastrointestinal tract. 1042 78

The leading causes of chronic pancreatitis are alcohol and idiopathic pancreatitis. The importance of genetic factors in chronic pancreatitis has been uncertain. Recently, however, it was learned that many patients with idiopathic chronic pancreatitis have mutations of the cystic fibrosis gene. This article reviews the evidence that links mutations of this gene to unexplained pancreatitis, and discusses the implications of this for the evaluation, pathogenesis, classification, and possible prevention of pancreatitis.
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PMID:Are mutations in the cystic fibrosis gene important in chronic pancreatitis? 1047 Mar 22


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