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Eugene P Dimagno - One of the best experts on this subject based on the ideXlab platform.
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Chronic Pancreatitis.
Current opinion in gastroenterology, 2013Co-Authors: Matthew J Dimagno, Eugene P DimagnoAbstract:We review selected important clinical observations reported in 2012. Celiac disease is a risk factor for Pancreatitis. Patients with recurrent acute Pancreatitis likely have Chronic Pancreatitis, do not benefit from pancreatic sphincterotomy, and may not benefit from biliary sphincterotomy. Analysis of endoscopic ultrasonography (EUS) images with an artificial neural network (ANN) program may improve Chronic Pancreatitis diagnosis compared with clinical interpretation of images. In a multicenter, randomized controlled trial of Chronic Pancreatitis patients, 90 000 USP U of pancreatin with meals decreased fat malabsorption compared with placebo. Detection of visceral pain in Chronic Pancreatitis predicts pain relief from various treatments, but nonvisceral pain due to altered central pain processing may respond to agents such as pregabalin. Predictors of surgical pain relief include onset of symptoms less than 3 years and preoperatively no opioid use and less than five endoscopic procedures. Total pancreatectomy for presumed painful Chronic Pancreatitis remains controversial. Celiacs are at risk for Pancreatitis. The diagnosis of Chronic Pancreatitis may be enhanced by ANN analysis of EUS imaging. Treatment of fat malabsorption requires 90,000 USP U of lipase with meals. Relief of pain from organ directed treatment of Chronic Pancreatitis may depend upon timing of interventions and whether pain is visceral or nonvisceral.
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Chronic Pancreatitis.
Current opinion in gastroenterology, 2011Co-Authors: Matthew J Dimagno, Eugene P DimagnoAbstract:We review important new clinical observations in Chronic Pancreatitis made in the past year. Tropical Pancreatitis associates with SPINK1 and/or CFTR gene mutations in approximately 50% of patients, similar to the frequency in idiopathic Chronic Pancreatitis. Corticosteroids increase secretin-stimulated pancreatic bicarbonate concentrations in autoimmune Pancreatitis (AIP) by restoring mislocalized CFTR protein to the apical ductal membrane. Most patients with asymptomatic hyperenzymemia have pancreatic lesions of unclear significance or no pancreatic lesions. Common pitfalls in the use of diagnostic tests for exocrine pancreatic insufficiency (EPI) confound interpretation of findings in irritable bowel syndrome and severe renal insufficiency. Further study is needed to improve the accuracy of endoscopic ultrasonography (EUS) to diagnose Chronic Pancreatitis. Celiac plexus block provides short-term pain relief in a subset of patients. Results of this year's investigations further elucidated the genetic associations of tropical Pancreatitis, a reversible mislocalization of ductal CFTR in AIP, the association of asymptomatic pancreatic hyperenzymemia with pancreatic disorders, limitations of diagnostic tests for EPI, diagnosis of Chronic Pancreatitis by EUS and endoscopic pancreatic function testing and treatment of pain.
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Chronic Pancreatitis.
Current opinion in gastroenterology, 2010Co-Authors: Matthew J Dimagno, Eugene P DimagnoAbstract:We review important new clinical observations in Chronic Pancreatitis made in the past year. Cigarette smoking is a dose-dependent risk factor for acute Pancreatitis, recurrent acute Pancreatitis, and Chronic Pancreatitis. A minority of Chronic alcohol consumers develop recurrent acute Pancreatitis but very heavy drinking associates with Chronic Pancreatitis. More patients with alcohol-induced Chronic Pancreatitis have cirrhosis than patients with cirrhosis have Chronic Pancreatitis (39 vs. 18%). Most patients with asymptomatic hyperenzymemia have no pancreatic lesions. Pancreatic calcifications are most frequently due to Chronic Pancreatitis, followed by cystic neoplasms and other disorders. The new Rosemont consensus classification of endoscopic ultrasonography criteria for Chronic Pancreatitis is unvalidated. Zinc deficiency correlates only with severe Chronic Pancreatitis and the fecal elastase test is an inaccurate marker of pancreatic steatorrhea. Patients commonly receive insufficient lipase to abolish pancreatic steatorrhea. Ultrastructural neuropathies are common to Chronic Pancreatitis and pancreatic cancer and correlate with pain severity. Results of this year's investigations further elucidated risk factors for pancreatic disease, the natural history of alcoholic Pancreatitis, the differential diagnosis of pancreatic calcifications, the diagnosis of Chronic Pancreatitis with the Rosemont criteria, the limited diagnostic utility of fecal elastate test and zinc measurements, the proper dosing of pancreatic enzyme supplements, and treatment of pancreatic pain.
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Chronic Pancreatitis.
Current opinion in gastroenterology, 2009Co-Authors: Matthew J Dimagno, Eugene P DimagnoAbstract:We review important new clinical observations in Chronic Pancreatitis made in the last year. Cholecystokinin-stimulated endoscopic pancreatic function testing has low specificity for diagnosis of early Chronic Pancreatitis, likely because of correctable technical limitations or failure to exclude confounding diseases. Fecal elastase 1 is a poor test for diagnosing pancreatic malabsorption. A 2-week trial of corticosteroids may differentiate autoimmune Pancreatitis (AIP) from pancreatic cancer in select patients. A genetic screen may help to predict relapse of AIP. Repeated, 6-month visits to counsel against alcohol use may reduce recurrent attacks of alcoholic Pancreatitis. A five-component antioxidant cocktail may reduce the frequency of pain in Chronic Pancreatitis by 1.5 days per month. Researchers this last year have further characterized clinical aspects of Chronic Pancreatitis that may lead to improved detection of early Chronic Pancreatitis and AIP, risk stratification and application of risk-lowering strategies to prevent relapses in AIP or the development of recurrent (and possibly Chronic) alcoholic Pancreatitis and new evidence that antioxidants may have a modest effect on reducing the dominant symptom in Chronic Pancreatitis, abdominal pain.
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Chronic Pancreatitis.
Current opinion in gastroenterology, 2006Co-Authors: Matthew J Dimagno, Eugene P DimagnoAbstract:As in our previous reviews, we endeavor to review important new observations in Chronic Pancreatitis made in the past year. Topics recently reviewed were truncated to accommodate a surge in publications on clinical aspects of Chronic Pancreatitis, which contained new observations or insights into new or old concepts. Cystic fibrosis carriers have been found to be at increased risk of Pancreatitis. Autoimmune Pancreatitis may belong to a multiorgan immunoglobulin G4-related autoimmune disease, and the natural history of Chronic Pancreatitis differs among the etiologies. Diffusion-weighted magnetic resonance imaging improves upon previous methodologies for diagnosing reduced pancreatic exocrine secretion, and fecal elastase-1 has been found to be a poor test for diagnosing pancreatic malabsorption. Visceral hyperalgesia or heightened central pain perception may contribute to pain in Chronic Pancreatitis. Instruments are evolving to assess quality of life in Chronic Pancreatitis, and fibrolytic agents have been found to have therapeutic promise. Researchers this past year have further characterized genetic, molecular and clinical aspects of Chronic Pancreatitis. Advancing the understanding of fibrogenesis, mechanisms of exocrine insufficiency, calcification, and pain and continuing development/modification of diagnostic tests should lead to improved prevention, detection and treatment of the condition. More accurate quantification of outcomes is critical for translating potential therapies from bench to bedside.
Paul Georg Lankisch - One of the best experts on this subject based on the ideXlab platform.
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Chronic Pancreatitis.
Current opinion in gastroenterology, 2007Co-Authors: Paul Georg LankischAbstract:As in previous reviews in this journal, this review is focused on the most important new observations in Chronic Pancreatitis made in the past year and the beginning of this year. Important observations include the following: first, the natural history and course of Chronic Pancreatitis; second, that smoking enhances the risk of Chronic Pancreatitis; third, possible new function tests in combination with imaging procedures; fourth, the superiority of surgery compared with endotherapy for long-term pain relief; fifth, new insights in autoimmune Pancreatitis. All in all, little progress has recently been made in the field of diagnosis and therapy of Chronic Pancreatitis. There are some studies in the field of endotherapy and autoimmune Pancreatitis that are promising however.
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Chronic Pancreatitis: Epidemiology
Pancreatitis, 1998Co-Authors: Paul Georg Lankisch, Peter A BanksAbstract:Data we have on the incidence and prevalence of Chronic Pancreatitis in various countries is sparse. This deficiency may be explained by several factors: The diagnosis of Chronic Pancreatitis, especially at an early stage, is not easy and requires an experienced doctor, expensive equipment and invasive procedures. An unknown, but probably substantial number of patients with irritable bowel syndrome or other conditions may be incorrectly diagnosed as having Chronic Pancreatitis. The generally accepted classifications of pancreatic diseases are not generally known or used. As a result, an interinstitutional data comparison is not possible at present. For example, Chronic Pancreatitis may be misdiagnosed as acute Pancreatitis, and vice versa, and sequelae of acute Pancreatitis, e.g., scars seen on endoscopic retrograde cholangiopancreatography (ERCP), may be deemed Chronic Pancreatitis (see Chap. 4 and Sects. 17.3, 17.4). Postmortem examinations do not reflect the true incidence of the disease since in most countries the rate of autopsies is declining. In addition, diagnoses provided on death certificates are not sufficiently accurate for exact statistical analysis.
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Chronic Pancreatitis: Pathophysiology
Pancreatitis, 1998Co-Authors: Paul Georg Lankisch, Peter A BanksAbstract:There is no one generally accepted concept for explaining the pathophysiology of Chronic Pancreatitis. Four major hypotheses have been proposed, that may to some extent explain the development of Chronic Pancreatitis, but further studies are required.
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Chronic Pancreatitis: Pathology
Pancreatitis, 1998Co-Authors: Paul Georg Lankisch, Peter A BanksAbstract:The term Chronic Pancreatitis implies that clinical manifestation of the disease and anatomical changes of the gland persist or increase, even when the initial cause or factors leading to Pancreatitis have been eliminated. Chronic Pancreatitis without clinical manifestation may be more frequent than believed. In one postmortem study, mild or moderate Chronic inflammation of the gland was found in 13% of the cases [11]. Interstitial fibrosis with a few or no inflammatory cells is common in the elderly, and such findings should not be interpreted as Chronic Pancreatitis [9].
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Chronic Pancreatitis: Complications
Pancreatitis, 1998Co-Authors: Paul Georg Lankisch, Peter A BanksAbstract:The complications of acute Pancreatitis are well known and have a decisive influence on the prognosis of the disease, whereas the influence of complications of Chronic Pancreatitis (Table 18.1) on the natural history of Chronic Pancreatitis is not fully established.
Peter A Banks - One of the best experts on this subject based on the ideXlab platform.
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Chronic Pancreatitis.
Current opinion in gastroenterology, 2008Co-Authors: Darwin L Conwell, Peter A BanksAbstract:As in previous reviews in this journal, this review is focused on the most important new observations in Chronic Pancreatitis made in the last year. Important observations included the search for biomarkers and alternative methods for the detection of early Chronic Pancreatitis; stellate cell activation and their role in fibrogenesis; the natural history of Chronic Pancreatitis; reports outlining the complexity in diagnosis of autoimmune Pancreatitis; emerging roles of endoscopic ultrasound and magnetic resonance cholangiopancreatography in Chronic Pancreatitis diagnosis; a better understanding of neurobiology of Chronic Pancreatitis pain; and the potential role of surgery as first-line therapy in advanced Chronic Pancreatitis. In 2007, major advances were made in our understanding of central processing in Chronic Pancreatitis pain. New techniques are being utilized in search of a better means to diagnose early Chronic Pancreatitis. Important prospective studies are emerging, which compare endoscopic and surgical interventions. Furthermore, the complexities of diagnosing autoimmune Pancreatitis are being recognized. Overall, the future is promising as advances in genomic and proteomic techniques are applied to improve our understanding of Chronic Pancreatitis.
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Chronic Pancreatitis: Epidemiology
Pancreatitis, 1998Co-Authors: Paul Georg Lankisch, Peter A BanksAbstract:Data we have on the incidence and prevalence of Chronic Pancreatitis in various countries is sparse. This deficiency may be explained by several factors: The diagnosis of Chronic Pancreatitis, especially at an early stage, is not easy and requires an experienced doctor, expensive equipment and invasive procedures. An unknown, but probably substantial number of patients with irritable bowel syndrome or other conditions may be incorrectly diagnosed as having Chronic Pancreatitis. The generally accepted classifications of pancreatic diseases are not generally known or used. As a result, an interinstitutional data comparison is not possible at present. For example, Chronic Pancreatitis may be misdiagnosed as acute Pancreatitis, and vice versa, and sequelae of acute Pancreatitis, e.g., scars seen on endoscopic retrograde cholangiopancreatography (ERCP), may be deemed Chronic Pancreatitis (see Chap. 4 and Sects. 17.3, 17.4). Postmortem examinations do not reflect the true incidence of the disease since in most countries the rate of autopsies is declining. In addition, diagnoses provided on death certificates are not sufficiently accurate for exact statistical analysis.
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Chronic Pancreatitis: Pathophysiology
Pancreatitis, 1998Co-Authors: Paul Georg Lankisch, Peter A BanksAbstract:There is no one generally accepted concept for explaining the pathophysiology of Chronic Pancreatitis. Four major hypotheses have been proposed, that may to some extent explain the development of Chronic Pancreatitis, but further studies are required.
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Chronic Pancreatitis: Pathology
Pancreatitis, 1998Co-Authors: Paul Georg Lankisch, Peter A BanksAbstract:The term Chronic Pancreatitis implies that clinical manifestation of the disease and anatomical changes of the gland persist or increase, even when the initial cause or factors leading to Pancreatitis have been eliminated. Chronic Pancreatitis without clinical manifestation may be more frequent than believed. In one postmortem study, mild or moderate Chronic inflammation of the gland was found in 13% of the cases [11]. Interstitial fibrosis with a few or no inflammatory cells is common in the elderly, and such findings should not be interpreted as Chronic Pancreatitis [9].
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Chronic Pancreatitis: Complications
Pancreatitis, 1998Co-Authors: Paul Georg Lankisch, Peter A BanksAbstract:The complications of acute Pancreatitis are well known and have a decisive influence on the prognosis of the disease, whereas the influence of complications of Chronic Pancreatitis (Table 18.1) on the natural history of Chronic Pancreatitis is not fully established.
Joachim Mössner - One of the best experts on this subject based on the ideXlab platform.
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Hereditary Chronic Pancreatitis.
Best practice & research. Clinical gastroenterology, 2008Co-Authors: Niels Teich, Joachim MössnerAbstract:Hereditary Chronic Pancreatitis (HCP) is a very rare form of early-onset Chronic Pancreatitis. Apart from young age at diagnosis and a slower progression, the clinical course, morphological features and laboratory findings of HCP do not differ from those of patients with alcoholic Chronic Pancreatitis. Diagnostic criteria and treatment of HCP also resemble those of Chronic Pancreatitis of other causes. The clinical presentation is highly variable and includes Chronic abdominal pain, impairment of endocrine and exocrine pancreatic function, nausea and vomiting, maldigestion, diabetes, pseudocysts, bile-duct and duodenal obstruction, and rarely pancreatic cancer. Fortunately, the disease is mild in most patients. Mutations in the PRSS1 gene, encoding cationic trypsinogen, play a causative role in Chronic Pancreatitis. It has been shown that the PRSS1 mutations increase autocatalytic conversion of trypsinogen to active trypsin, and thus probably cause premature, intrapancreatic trypsinogen activation, disturbing the intrapancreatic balance of proteases and their inhibitors. Other genes—such as the anionic trypsinogen (PRSS2), the serine protease inhibitor Kazal type 1 (SPINK1), and the cystic fibrosis transmembrane conductance regulator (CFTR)—have also been found to be associated with Chronic Pancreatitis (idiopathic and hereditary). Genetic testing should only be performed in carefully selected patients by direct DNA sequencing, and antenatal diagnosis should not be encouraged. Treatment focuses on enzyme and nutritional supplementation, pain management, pancreatic diabetes, and local organ complications such as pseudocysts and bile-duct or duodenal obstruction. The disease course and prognosis of patients with HCP is unpredictable. The risk of pancreatic cancer is elevated. Therefore, HCP patients should strongly avoid environmental risk factors for pancreatic cancer.
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Hereditary Chronic Pancreatitis
Orphanet Journal of Rare Diseases, 2007Co-Authors: Jonas Rosendahl, Hans Bödeker, Joachim Mössner, Niels TeichAbstract:Hereditary Chronic Pancreatitis (HCP) is a very rare form of early onset Chronic Pancreatitis. With the exception of the young age at diagnosis and a slower progression, the clinical course, morphological features and laboratory findings of HCP do not differ from those of patients with alcoholic Chronic Pancreatitis. As well, diagnostic criteria and treatment of HCP resemble that of Chronic Pancreatitis of other causes. The clinical presentation is highly variable and includes Chronic abdominal pain, impairment of endocrine and exocrine pancreatic function, nausea and vomiting, maldigestion, diabetes, pseudocysts, bile duct and duodenal obstruction, and rarely pancreatic cancer. Fortunately, most patients have a mild disease. Mutations in the PRSS1 gene, encoding cationic trypsinogen, play a causative role in Chronic Pancreatitis. It has been shown that the PRSS1 mutations increase autocatalytic conversion of trypsinogen to active trypsin, and thus probably cause premature, intrapancreatic trypsinogen activation disturbing the intrapancreatic balance of proteases and their inhibitors. Other genes, such as the anionic trypsinogen (PRSS2), the serine protease inhibitor, Kazal type 1 (SPINK1) and the cystic fibrosis transmembrane conductance regulator (CFTR) have been found to be associated with Chronic Pancreatitis (idiopathic and hereditary) as well. Genetic testing should only be performed in carefully selected patients by direct DNA sequencing and antenatal diagnosis should not be encouraged. Treatment focuses on enzyme and nutritional supplementation, pain management, pancreatic diabetes, and local organ complications, such as pseudocysts, bile duct or duodenal obstruction. The disease course and prognosis of patients with HCP is unpredictable. Pancreatic cancer risk is elevated. Therefore, HCP patients should strongly avoid environmental risk factors for pancreatic cancer.
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Genetic aspects of Chronic Pancreatitis.
Medical science monitor : international medical journal of experimental and clinical research, 2004Co-Authors: Niels Teich, Joachim MössnerAbstract:The classical feature of hereditary Pancreatitis (HP) is characterized by recurrent episodes of acute Pancreatitis or a priori Chronic Pancreatitis in several members of one family. In 1996, the identification of the first HP-associated mutation in the cationic trypsinogen gene provided a breakthrough in our understanding of the pathogenesis of Chronic Pancreatitis. In the following years, several different mutations in the same gene have been found in a large number of investigated families. Most intriguing, HP patients have a more than 50-fold increased risk of pancreatic ductal cancer in comparison with expected pancreatic cancers in the general population. Variants of the major intrapancreatic trypsin antagonist SPINK1 have implications for more common forms of Chronic Pancreatitis. Research has focussed on the SPINK1-N34S-mutation, which is closely associated with tropical, alcoholic, or "idiopathic" Chronic Pancreatitis. Chronic Pancreatitis represents a variable part of the cystic fibrosis syndrome, which is caused by mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR). Several groups have reported an increased prevalence of CFTR mutations in patients with Chronic Pancreatitis of different etiology. In this review, we summarize interesting clinical and biochemical features of genetic variants in these genes which are associated with Chronic Pancreatitis.
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Metabolic Aspects of Chronic Pancreatitis
Pancreatic Enzymes in Health and Disease, 1991Co-Authors: Joachim MössnerAbstract:There are various causes of metabolic disorders in Chronic Pancreatitis (Table 1). In the industrialized countries more than 70% of all cases of Chronic Pancreatitis are caused by alcohol abuse. Thus, one has to differentiate between metabolic disorders as a consequence of Chronic ethanol intoxication of various organs such as liver and metabolic disorders related to Chronic Pancreatitis itself, such as maldigestion. Chronic Pancreatitis per se or via Table 1 Causes of metabolic disorders in Chronic Pancreatitis Cause Consequence of: Maldigestion Loss of acinar cell mass and function Obstruction of pancreatic ducts Obstruction of bile duct Malabsorption Chronic Pancreatitis (?) Toxic effects of ethanol on gut mucosa Endocrine insufficiency Insulin deficiency due to islet damage Deficiency of other islet hormones Ethanol abuse Direct toxic effects of ethanol on various organ systems such as liver Malnutrition Alcohol abuse Chronic pain Arteriosclerosis Smoking Malignancies Smoking (lung cancer), immune deficiency due to alcohol abuse Infections Immunedeficiency due to alcohol abuse Chronic bronchitis due to smoking Pulmonary dysfunction Chronic bronchitis due to smoking Acute replases of Chronic Pancreatitis maldigestion may impair absorption. Malassimilation in this disease may, therefore, have several causes. Furthermore, alcohol abuse is often associated with malnutrition which in addition will be responsible for metabolic disorders. Many people who abuse alcohol are smokers. The fate of patients with Chronic Pancreatitis, therefore, is often complicated by the consequences of smoking such as pulmonary and right heart insufficiency due to Chronic bronchitis. Metabolism may be disturbed due to lung cancer and very often due to general arteriosclerosis. Infectious complications have to be kept in mind which often occur in patients with impaired lung function and immune deficiency due to alcohol abuse. Chronic Pancreatitis may lead to insulin deficiency. However, I do not want to discuss the whole array of metabolic disorders seen in diabetes.
Niels Teich - One of the best experts on this subject based on the ideXlab platform.
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Hereditary Chronic Pancreatitis.
Best practice & research. Clinical gastroenterology, 2008Co-Authors: Niels Teich, Joachim MössnerAbstract:Hereditary Chronic Pancreatitis (HCP) is a very rare form of early-onset Chronic Pancreatitis. Apart from young age at diagnosis and a slower progression, the clinical course, morphological features and laboratory findings of HCP do not differ from those of patients with alcoholic Chronic Pancreatitis. Diagnostic criteria and treatment of HCP also resemble those of Chronic Pancreatitis of other causes. The clinical presentation is highly variable and includes Chronic abdominal pain, impairment of endocrine and exocrine pancreatic function, nausea and vomiting, maldigestion, diabetes, pseudocysts, bile-duct and duodenal obstruction, and rarely pancreatic cancer. Fortunately, the disease is mild in most patients. Mutations in the PRSS1 gene, encoding cationic trypsinogen, play a causative role in Chronic Pancreatitis. It has been shown that the PRSS1 mutations increase autocatalytic conversion of trypsinogen to active trypsin, and thus probably cause premature, intrapancreatic trypsinogen activation, disturbing the intrapancreatic balance of proteases and their inhibitors. Other genes—such as the anionic trypsinogen (PRSS2), the serine protease inhibitor Kazal type 1 (SPINK1), and the cystic fibrosis transmembrane conductance regulator (CFTR)—have also been found to be associated with Chronic Pancreatitis (idiopathic and hereditary). Genetic testing should only be performed in carefully selected patients by direct DNA sequencing, and antenatal diagnosis should not be encouraged. Treatment focuses on enzyme and nutritional supplementation, pain management, pancreatic diabetes, and local organ complications such as pseudocysts and bile-duct or duodenal obstruction. The disease course and prognosis of patients with HCP is unpredictable. The risk of pancreatic cancer is elevated. Therefore, HCP patients should strongly avoid environmental risk factors for pancreatic cancer.
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Hereditary Chronic Pancreatitis
Orphanet Journal of Rare Diseases, 2007Co-Authors: Jonas Rosendahl, Hans Bödeker, Joachim Mössner, Niels TeichAbstract:Hereditary Chronic Pancreatitis (HCP) is a very rare form of early onset Chronic Pancreatitis. With the exception of the young age at diagnosis and a slower progression, the clinical course, morphological features and laboratory findings of HCP do not differ from those of patients with alcoholic Chronic Pancreatitis. As well, diagnostic criteria and treatment of HCP resemble that of Chronic Pancreatitis of other causes. The clinical presentation is highly variable and includes Chronic abdominal pain, impairment of endocrine and exocrine pancreatic function, nausea and vomiting, maldigestion, diabetes, pseudocysts, bile duct and duodenal obstruction, and rarely pancreatic cancer. Fortunately, most patients have a mild disease. Mutations in the PRSS1 gene, encoding cationic trypsinogen, play a causative role in Chronic Pancreatitis. It has been shown that the PRSS1 mutations increase autocatalytic conversion of trypsinogen to active trypsin, and thus probably cause premature, intrapancreatic trypsinogen activation disturbing the intrapancreatic balance of proteases and their inhibitors. Other genes, such as the anionic trypsinogen (PRSS2), the serine protease inhibitor, Kazal type 1 (SPINK1) and the cystic fibrosis transmembrane conductance regulator (CFTR) have been found to be associated with Chronic Pancreatitis (idiopathic and hereditary) as well. Genetic testing should only be performed in carefully selected patients by direct DNA sequencing and antenatal diagnosis should not be encouraged. Treatment focuses on enzyme and nutritional supplementation, pain management, pancreatic diabetes, and local organ complications, such as pseudocysts, bile duct or duodenal obstruction. The disease course and prognosis of patients with HCP is unpredictable. Pancreatic cancer risk is elevated. Therefore, HCP patients should strongly avoid environmental risk factors for pancreatic cancer.
-
Genetic aspects of Chronic Pancreatitis.
Medical science monitor : international medical journal of experimental and clinical research, 2004Co-Authors: Niels Teich, Joachim MössnerAbstract:The classical feature of hereditary Pancreatitis (HP) is characterized by recurrent episodes of acute Pancreatitis or a priori Chronic Pancreatitis in several members of one family. In 1996, the identification of the first HP-associated mutation in the cationic trypsinogen gene provided a breakthrough in our understanding of the pathogenesis of Chronic Pancreatitis. In the following years, several different mutations in the same gene have been found in a large number of investigated families. Most intriguing, HP patients have a more than 50-fold increased risk of pancreatic ductal cancer in comparison with expected pancreatic cancers in the general population. Variants of the major intrapancreatic trypsin antagonist SPINK1 have implications for more common forms of Chronic Pancreatitis. Research has focussed on the SPINK1-N34S-mutation, which is closely associated with tropical, alcoholic, or "idiopathic" Chronic Pancreatitis. Chronic Pancreatitis represents a variable part of the cystic fibrosis syndrome, which is caused by mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR). Several groups have reported an increased prevalence of CFTR mutations in patients with Chronic Pancreatitis of different etiology. In this review, we summarize interesting clinical and biochemical features of genetic variants in these genes which are associated with Chronic Pancreatitis.