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)

Definite inherited defect in hereditary pancreatitis (HP) is not known. A new kindred with 3 definite and 6 suspected cases of HP was investigated for possible inherited abnormalities. No aminoaciduria (except for a slight rise in urinary histidine in one patient) and no hyperparathyroidism, hyperlipidemia, or chromosomal abnormality was present. An increase in serum IgM level of a polyclonal type was noted in 3 definitely affected sisters and also in 2 nonaffected members. Serum alpha-1-antitrypsin and serum trypsin inhibition were normal. However, very marked dilatation and ectasia of the pancreatic duct were found in the propositus. Reviewing the data from this family and previously described kindreds, it is postulated that the genetic abnormality in HP encompasses a wide variety of structural and anatomical defects in the sphincter of Oddi or the pancreatic ductal system. These predispose to intermittent obstruction of the duct with concomitant activation of enzymes and ductal metaplasia. In suspected cases an early effort should be made to outline the pancreatic duct as the defect may be amenable to surgery.
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PMID:Inherited defect in hereditary pancreatitis. 30 62

The most important diagnostic step in the management of patients with severe acute pancreatitis is the discrimination between acute interstitial and necrotizing pancreatitis. Measurement of C-reactive protein, lactic acid dehydrogenase, alpha-1-antitrypsin, and alpha-2-macroglobulin and contrast-enhanced CT are useful in detecting the necrotizing course of acute pancreatitis. C-reactive protein, lactic acid dehydrogenase, and contrast-enhanced CT offer detection rates of 85 per cent to more than 90 per cent for pancreatic necrosis. Surgical decision-making in necrotizing pancreatitis should be based on clinical, morphologic, and bacteriologic data. Patients with focal pancreatic necrosis, in general, respond well to medical treatment and do not need surgery. Extended (50 per cent or more) pancreatic necroses, infected necroses, and intrapancreatic parenchymal necroses plus extrapancreatic fatty tissue necroses are indicators for surgical management. The decision for the timing of operation in patients with proved necrotizing pancreatitis should be based on clinical criteria: the development of an acute surgical abdomen, generalized sepsis, shock, persisting or increasing organ dysfunction, or some combination thereof despite maximum intensive care treatment for at least 3 days. Major pancreatic resection for the treatment of necrotizing pancreatitis appears disadvantageous. Necrosectomy and continuous local lavage allow debridement of devitalized tissue and preservation of vital pancreatic tissue. Postoperative local lavage thus results in an atraumatic evacuation of necrotic tissue, the bacterial material, and biologically active substances. The hospital mortality rate of patients treated with necrosectomy and continuous local lavage (the Ulm protocol) is below 10 per cent. Nevertheless, controlled prospective clinical trials should be performed in order to bring more precision to our clinical decisions in respect to the role of surgery for this disease.
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PMID:Surgical management of necrotizing pancreatitis. 265 62

Serum trypsin esterolytic activity was measured in 106 sera from 61 controls and 45 patients with pancreatitis. A trypsin specific synthetic substrate, N-alpha-benzoyl-L-arginine-paranitroanilide, was used. High levels of enzymatically active trypsin were shown to be present in serum of patients with pancreatitis. No difference between the two samples was noticed for the serum concentrations of alpha-1-antitrypsin and alpha-2-macroglobulin (the two main serum trypsin inhibitors). Active trypsin was contained in the high molecular weight fraction of plasma proteins, corresponding to a complex with alpha-2-macroglobulin. The determination of serum typsin activity as a sensitive test for detection of pancreatitis was demonstrated to be statistically significant.
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PMID:"Tryptic-like" activity in sera of patients with pancreatitis. 625 87

The submitted investigation deals with the value of assessment of selected reactants of the acute stage in patients with acute pancreatitis and their follow-up in the course of the disease. For the investigation C-reactive protein, alpha-1-antitrypsin and haptaglobin were selected. In addition to the prognostic impact the authors focused attention on assessment of the dynamics of individual proteins and their importance for the early detection of complications in acute pancreatitis with different etiologies. The high CRP level on admission of severe cases of pancreatitis--mean 166.9 mg/l (range from 100 to 320 mg/l)--correlated with other signs suggesting severe pancreatitis and the latter was confirmed by computed tomography (CT), surgery or post-mortem examination in 90% of the patients with a CRP level above 100 mg/l. This correlation was not confirmed for alpha-1-antitrypsin or haptaglobin.
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PMID:[Acute phase proteins in acute pancreatitis]. 753 40

The observation that only a minority of heavy drinkers develop pancreatitis has prompted an intensive search for a trigger factor/cofactor/susceptibility factor that may precipitate a clinical attack. Putative susceptibility factors examined so far include diet, smoking, amount and type of alcohol consumed, the pattern of drinking and lipid intolerance. In addition, a range of inherited factors have been assessed including blood group antigens, human leukocyte antigen serotypes, alpha-1-antitrypsin phenotypes and several genotypes. The latter group comprises mutations/polymorphisms in genes related to alcohol-metabolizing enzymes, detoxifying enzymes, pancreatic digestive enzymes, pancreatic enzyme inhibitors, cystic fibrosis and cytokines. Disappointingly, despite this concerted research effort, no clear association has been established between the above factors and alcoholic pancreatitis. Experimentally, the secretagogue cholecystokinin (CCK) has been investigated as a candidate 'trigger' for alcoholic pancreatitis. However, the clinical relevance of CCK as a trigger factor has to be questioned, as it is difficult to envisage a situation in humans where abnormally high levels of CCK would be released into the circulation to trigger pancreatitis in alcoholics. In contrast, bacterial endotoxemia is a candidate cofactor that does have relevance to the clinical situation. Plasma lipopolysaccharide (LPS, an endotoxin) levels are significantly higher in drinkers (either after chronic alcohol intake or a single binge) compared to non-drinkers. We have recently shown that alcohol-fed animals challenged with otherwise innocuous doses of LPS exhibit significant pancreatic injury. Moreover, repeated LPS exposure in alcohol-fed rats leads to progressive injury to the gland characterized by significant pancreatic fibrosis. These studies support the concept that endotoxin may be an important factor in the initiation and progression of alcoholic pancreatitis. Scope remains for further studies examining proteins related to cellular anti-oxidant defenses, minor cystic fibrosis (CF) mutations and trans-heterozygosity involving a combination of mutations of different genes (such as CFTR alterations combined with SPINK1 or PRSS1 variants), as potential triggers of alcoholic pancreatitis.
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PMID:Individual susceptibility to alcoholic pancreatitis. 1833 67

There are genetic mutations taking part in the physiopathology of pancreatitis. The role of alpha-1-antitrypsin (AAT) deficiency in this pathology is debated. We report the case of a 60-year-old man with a pancreatic exocrine insufficiency. He was diagnosed with AAT deficiency. The phenotype was Pi SZ, with genotyping confirmation. The place of AAT deficiency in the midst of pancreatic diseases should be further studied.
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PMID:[Exocrin pancreatic deficiency revealing an alpha-1-antitrypsin deficiency]. 1892 50

Systemic lupus erythematosus (SLE) is a multisystem disorder which can affect the gastrointestinal (GI) system. Although GI symptoms can manifest in 50% of patients with SLE, these have barely been reviewed due to difficulty in identifying different causes. This study aims to clarify clinical characteristics, diagnosis and treatment of the four major SLE-related GI system complications: protein-losing enteropathy (PLE), intestinal pseudo-obstruction (IPO), hepatic involvement and pancreatitis. It is a systematic review using MEDLINE and EMBASE databases and the major search terms were SLE, PLE, IPO, hepatitis and pancreatitis. A total of 125 articles were chosen for our study. SLE-related PLE was characterized by edema and hypoalbuminemia, with Technetium 99m labeled human albumin scintigraphy (99mTc HAS) and alpha-1-antitrypsin fecal clearance test commonly used as diagnostic test. The most common site of protein leakage was the small intestine and the least common site was the stomach. More than half of SLE-related IPO patients had ureterohydronephrosis, and sometimes they manifested as interstitial cystitis and hepatobiliary dilatation. Lupus hepatitis and SLE accompanied by autoimmune hepatitis (SLE-AIH overlap) shared similar clinical manifestations but had different autoantibodies and histopathological features, and positive anti-ribosome P antibody highly indicated the diagnosis of lupus hepatitis. Lupus pancreatitis was usually accompanied by high SLE activity with a relatively high mortality rate. Early diagnosis and timely intervention were crucial, and administration of corticosteroids and immunosuppressants was effective for most of the patients.
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PMID:Gastrointestinal system involvement in systemic lupus erythematosus. 2852 68

Post-ERCP pancreatitis (PEP) is a common complication most feared by endoscopists. Incidence ranges widelly from 2.1% to 24.4%, which results from patient heterogeneity and differences in endoscopist expertise, method, PEP definition, and severity. Pathophysiology is multifactorial, and involves a combination of chemical, thermal, mechanical, hydrostatic, enzymatic, allergic, and microbiological factors resulting from papillary instrumentation and/or contrast administration within the pancreatic duct (volume and osmolarity). Even genetic abnormalities may represent a risk factor, as is the case with homozygous alpha-1-antitrypsin deficiency, which leads to an increase in hemorrhagic PEP rates.
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PMID:Pancreatic stents in ERCP. Where are we? 2968 42