Der Internist
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Acute pancreatitis is characterized by the autodigestion of the pancreas by its own digestive enzymes. The pathophysiological onset of the disease occurs in the acinar cells. The normally inactive precursors of secreted proteases are prematurely activated and as a result digest the cells from within. ⋯ The consequence of this pronounced SIRS in the later course of the disease is a strong immunological counter-regulation, the so-called compensatory anti-inflammatory reaction syndrome (CARS). In the course of this immunosuppression commensal bacteria from the intestines can colonize the pancreatic necrosis. The outcome of the SIRS/CARS balance is decisive for the course and the prognosis of the patient.
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Chronic pancreatitis is characterized by recurrent inflammatory episodes. The diagnosis is based on the detection of pathognomonic morphological alterations by ultrasound-based methods as well as computed tomography and magnetic resonance imaging. In addition, imaging techniques play an important role for the differential diagnostics of potential complications in patients with chronic pancreatitis and for regular monitoring of patients with increased cancer risk. This article summarizes the current guideline recommendations for medical imaging procedures in chronic pancreatitis.
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The cardinal symptom of chronic pancreatitis is severe belt-like upper abdominal pain, which requires immediate and adequate treatment. Furthermore, advanced stage chronic pancreatitis is often associated with complications, such as pancreatic pseudocysts, pancreatic duct stones and stenosis as well as biliary stenosis. The various endoscopic and surgical treatment options for chronic pancreatitis patients have been controversially discussed for decades. ⋯ In contrast, pancreatic pseudocysts, solitary proximally situated pancreatic duct stones and benign biliary strictures (except in calcifying pancreatitis) can nowadays generally be managed endoscopically. For distal pancreatic duct stones and symptomatic pancreatic duct stenosis surgical treatment is again the method of choice. This review article discusses these indication-related procedures in detail and explains them in relation to the recently published S3 guidelines on pancreatitis of the German Society for Gastroenterology, Digestive and Metabolic Diseases (DGVS).
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Long-term alcohol consumption and gene mutations are the most important causes of chronic pancreatitis. In addition to mutations in acinar genes, such as digestive enzymes and their inhibitors, defects in genes that primarily or exclusively affect the duct cells have also been described in recent years. Genetic changes are found not only in patients with a positive family history (hereditary pancreatitis) but also in so-called idiopathic and, to a lesser extent, in alcoholic chronic pancreatitis. The coming years will likely show that there are very complex interactions between environmental influences and numerous genetic factors.
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In the European Union a disease is classified as rare if it affects no more than 5 out of 10,000 people. Currently, there are more than 6000 rare diseases, consisting of a large and heterogeneous number of different diseases that are complex in their symptomatology, multidimensional and therefore difficult to classify in everyday medical practice. ⋯ It also provides an overview of all German centers for rare diseases, which are a contact point for patients with an unclear diagnosis. The se-atlas serves as a compass through the heterogeneous amount of information on care facilities for rare diseases and provides low-threshold information for a broad user group, from affected persons to members of the medical care team.