Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present a case of a 27-year-old female suffering from chronic calcifying pancreatitis with diabetes mellitus. Radiographic examinations and exocrine pancreatic function tests revealed considerable dilatation of pancreatic ducts with large intraductal calculi and exocrine pancreatic insufficiency, respectively. Recent literature indicates that a decrease in the activity of pancreatic stone protein (PSP), which inhibits CaCO3 crystal formation in pancreatic juice, is closely related to the development of chronic calcifying pancreatitis. The patient had no apparent cause or family history of pancreatitis. We therefore investigated the possibility that alterations in the PSP gene might explain the chronic pancreatitis seen in this patient. Six exons of the PSP gene amplified by polymerase chain reaction were directly sequenced, but there was no apparent base mutation observed. Furthermore, Southern blot analysis revealed neither rearrangement nor deletion of the PSP gene in the genomic DNA of this case. However, this genetic approach will be useful for future study of the etiology of hereditary pancreatitis.
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PMID:Idiopathic chronic calcifying pancreatitis with diabetes mellitus. Analysis of pancreatic stone protein gene. 848 98

Cystic fibrosis (CF), the most common lethal autosomal recessive disease in white populations, is characterized by dysfunctional chloride ion transport across epithelial surfaces. Although recurrent pulmonary infections and pulmonary insufficiency are the principal causes of morbidity and death, gastrointestinal symptoms commonly precede the pulmonary findings and may suggest the diagnosis in infants and young children. The protean gastrointestinal manifestations of CF result primarily from abnormally viscous luminal secretions within hollow viscera and the ducts of solid organs. Bowel obstruction may be present at birth due to meconium ileus or meconium plug syndrome. Complications of meconium ileus include volvulus, small bowel atresia, perforation, and meconium peritonitis with abdominal calcifications. Older children with CF may present with bowel obstruction due to distal intestinal obstruction syndrome or colonic stricture, and tenacious intestinal residue may serve as a lead point for intussusception or cause recurrent rectal prolapse. Radiologic studies often demonstrate thickened intestinal mucosal folds in older children and uncommonly show colonic pneumatosis, peptic esophageal stricture due to gastroesophageal reflux, and duodenal ulcer. Appendicitis due to inspissated secretions is uncommon. Obstruction of ducts and ductules produces exocrine pancreatic insufficiency, pancreatitis, cholestasis, cholelithiasis, and cirrhosis with portal hypertension. On imaging studies, the pancreas is commonly small and largely replaced by fat, sometimes displays calcifications, and is rarely replaced by macrocysts. Radiologic features of hepatobiliary disease include an enlarged radiolucent liver from steatosis, gallstones, a shrunken nodular liver, splenomegaly, and portosystemic collateral vessels. With the improved survival of CF patients, an increased risk for developing gastrointestinal carcinomas has been established, many occurring as early as the 3rd decade.
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PMID:Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation. 883 77

Cystic fibrosis is the most prevalent hereditary disease in the Caucasian race. It is a multisystemic alteration that affects the quality and quantitative properties of exocrine secretions. The pancreas develops a progressive atrophy causing steatorrhoea and nutritive deficiencies. Acute pancreatitis is an unusual complication. The pancreatic atrophy prevents the inflammatory response. Published series suggest that pancreatitis in 0.5%, including patients without pancreatic insufficiency. We present two cases with cystic fibrosis, with and without pancreatic insufficiency, who developed acute pancreatitis.
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PMID:[Acute pancreatitis in cystic fibrosis]. 896 66

The nature of the gastrointestinal injury following bone marrow transplantation and its clinical and nutritional sequelae are poorly defined. Prospective assessments of gastrointestinal function, nutritional status, and wellbeing were therefore carried out in 47 consecutive patients (28 males, 19 females; mean age 8.4 years) undergoing bone marrow transplant. 31 diarrhoeal episodes (median duration 9.5 days) occurred in 27 patients at a median of 10 days after transplantation. Ninety one per cent of episodes were associated with protein losing enteropathy. Protein losing enteropathy was more severe in graft-versus-host disease (GVHD) comparing with other causes. It led to a substantial fall in serum albumin and there was a negative correlation between faecal alpha 1-antitrypsin concentrations and serum albumin. Transient pancreatic insufficiency developed in 18 patients, and pancreatitis in one. Intestinal permeability was normal in 12 patients who had no diarrhoea during the conditioning treatments. Diarrhoeal patients had a significantly greater decrease in nutritional status and wellbeing than patients without diarrhoea. Gastrointestinal injury following bone marrow transplantation is thus complex. Severe protein losing enteropathy in this context suggests the presence of GVHD.
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PMID:Gastrointestinal and nutritional sequelae of bone marrow transplantation. 897 59

Chronic fibrosing pancreatitis in childhood is an uncommon condition of unknown etiology with a variety of clinical presentations, histopathologic features, and outcomes. The diagnosis is one of exclusion (of hereditary or secondary pancreatitis), which should include histological assessment. The histological features of this condition have been described, to our knowledge, in nine published cases. We report a case in a 13-year-old male, who presented with obstructive jaundice and subsequently had evidence of endocrine and exocrine pancreatic insufficiency, despite a surgical decompression of the pancreatic-biliary duct system.
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PMID:Chronic fibrosing pancreatitis in childhood: report of a case and literature review. 902 65

Pancreatitis induced by ligation of the pancreatic duct produces morphologic similarities to human pancreatitis. This model is easily performed in big animals, but it is very difficult to perform pancreatic duct ligation in small animals. Many experimental studies of pharmaceutical treatments for pancreatitis used pancreatic duct-ligation models, but it is also difficult to evaluate the efficacy of the drugs used, because the animals used are of different species with individual differences. To overcome these problems, we ligated the main pancreatic duct of the splenic lobe by a 5.0 absorbable suture by using a surgical microscope and left the gastroduodenal lobe intact in the same rats. This model produced damaged pancreatic tissue in one part and normal pancreatic tissue in another part of the pancreas in the same animals, biochemically and histologically. We evaluated the effect of a new protease inhibitor (ONO-3404) on this preliminary model and found this new protease inhibitor demonstrated a hypertrophic effect on the damaged pancreatic tissue and the normal pancreatic tissue in the same animals. This model is also useful to study pharmaceutic treatment for pancreatic insufficiency and to study chemically induced pancreatic carcinogenesis in the damaged pancreatic tissue and the normal pancreatic tissue in the same animals.
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PMID:A new model for pancreatitis. 954 68

Pancreatitis and pancreatic insufficiency are associated with both cystic fibrosis and alcoholism. The pathogenesis of alcoholic pancreatitis is unknown, but only a minority of alcoholics develop pancreatitis, and it has been suggested that a genetic predisposition may play a role in this disease. Two observations led to the hypothesis that this genetic predisposition could result from mutations in the cystic fibrosis gene. First, the prevalence of cystic fibrosis mutations in the Caucasian population (approximately 5%) is similar to the prevalence of pancreatitis among heavy drinkers. Second, in both diseases, pancreatic duct damage is a prominent feature and has been postulated to be the initial site of injury. Therefore, the aim of this study was to determine whether an increased frequency of mutations in the cystic fibrosis gene occurs in alcoholic pancreatitis. The 15 most common cystic fibrosis mutations in a Caucasian community were sought in 24 subjects with alcoholic pancreatitis. None were homozygous or heterozygous for these mutations. These findings suggest that cystic fibrosis mutations are not a major genetic factor predisposing to pancreatic injury in alcoholics.
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PMID:Cystic fibrosis genotypes and alcoholic pancreatitis. 964 47

The aim of this study was to document the natural history of chronic hereditary pancreatitis and to compare its evolution to that of chronic alcoholic pancreatitis. Twelve subjects with chronic hereditary pancreatitis were followed up for a mean duration of 15.8 years (range, 1-23) and compared to subjects with chronic alcoholic pancreatitis who were followed up from 1972 to 1980. The subjects with chronic hereditary pancreatitis, when compared to those with chronic alcoholic pancreatitis, were found to have an earlier onset of symptoms (10.5 vs. 46.0 years, p < 0.05); a significant delay in diagnosis (14.3 vs. 3 years); a similar prevalence of pancreatic calcification (58 vs. 57%); a similar amount of pancreatic insufficiency; both endocrine (50 vs. 70%) and exocrine 42 vs. 38%); and a higher prevalence of pseudocysts (33 vs. 10%, p < 0.05). Only one pancreatic adenocarcinoma was diagnosed in a patient with chronic alcoholic pancreatitis. Apart from the earlier onset and the delay in diagnosis, chronic hereditary pancreatitis has a natural history similar to that of chronic alcoholic pancreatitis. The disease is progressive with a high incidence of complications, but all subjects were alive after follow-up.
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PMID:The natural history of hereditary chronic pancreatitis: a study of 12 cases compared to chronic alcoholic pancreatitis. 978 40

Except for cystic fibrosis, which is the most frequent genetic disorder in the Caucasian population, diseases of the exocrine pancreas are relatively uncommon in children. However, they are many and varied in terms of their pathogenesis and clinical manifestation. They can be classified as: (1) congenital anatomical abnormalities, (2) congenital secretory insufficiencies, and (3) pancreatitis. In all of these diseases, when pancreatic insufficiency (whether partial or complete) is present, the nutritional status of the patients must be investigated regularly, and pancreatic enzymes as well as nutritional supplementations must be prescribed as soon as malnutrition is present, or even prophylactically. The preservation of good nutritional status is the guarantee of a better prognosis.
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PMID:Pancreatic diseases (excluding cystic fibrosis). 1007 7

In the majority of patients suffering from chronic pancreatitis an endocrine pancreatic insufficiency is correlated with exocrine dysfunction. The prevalence of impaired or diabetic glucose tolerance is 40-70%, half of these patients suffer from an insulin-dependent diabetes mellitus. In general the probability of endocrine insufficiency progressively increases within the ten years following diagnosis of chronic pancreatitis. Onset and severity of the endocrine dysfunction depend on parenchymal destruction of the pancreas but are also influenced by ongoing alcohol consumption. Pathological findings in the endocrine pancreas are a loss of B-cells with decrease in secretion of insulin but also a loss of B-cell responsiveness to glucose by impaired perisinusoidal diffusion. Disturbances of the enteroinsulinar axis with diminished levels of incretins due to an exocrine insufficiency are also discussed. In addition, an impaired A-cell function may be important, that is characterized by diminished levels of stimulated glucagon. Increased plasma levels of somatostatin were found, the source of which is unknown. The susceptibility to severe hypoglycemia in patients with diabetes mellitus secondary to chronic pancreatitis is higher than in Type I diabetics. This is mainly caused by the impaired glucagon secretion but also influenced by malnutrition and concomitant hepatic dysfunction due to the toxic affect of alcohol. Diagnostic procedures are the measurement of C-peptide-concentrations and profiles of blood glucose after fasting and stimulation with L-arginine or glucose. Especially in the beginning of the endocrine insufficiency the determination of basal levels of blood glucose or C-peptide are not useful. Unless treatment by diet is effective, the therapy of diabetes secondary to chronic pancreatitis should be done by insulin replacement. A certain degree of hyperglycemia may be tolerated due to the risk of hypoglycemia and the persistent alcohol consumption in these patients. Intensified insulin therapy should only be done in selected patients with good compliance. Long-term complications in patients with pancreatogenic diabetes are comparable to diabetes Type I and largely depend on the duration of the diabetes. Life expectancy is reduced, death in these patients is mainly due to persistent alcohol and nicotine abuse (cardiovascular disease, malignant tumors, etc.), in only a minority pancreatitis or diabetes (mainly hypoglycaemia) are relevant risk factors.
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PMID:[Secondary diabetes in chronic pancreatitis]. 1044 9


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