Lege Artis Medicinae

[AUTOIMMUNE PANCREATITIS - AN UNDERDIAGNOSED DISEASE?]

CZAKÓ László

JUNE 21, 2006

Lege Artis Medicinae - 2006;16(06)

[Autoimmune pancreatitis is a recently recognized type of chronic pancreatitis that is clearly distinct from alcoholic chronic pancreatitis. Its clinical symptoms include jaundice, abdominal pain, weight loss and diabetes mellitus. It may be associated with other autoimmune diseases. IgG levels are elevated and autoantibodies can be detected. Pancreatic imaging reveals a diffuse enlargement of the pancreas and irregular narrowing of the main pancreatic duct. The characteristic histological features are lymphoplasmacytic infiltration and fibrosis. Autoimmune pancreatitis responds dramatically to steroid therapy, in contrast to other types of chronic pancreatitis, which hardly respond to any of the various therapies. It is important to be aware of this disease because it may be mistaken for other forms of chronic pancreatitis or pancreatic cancer, which leads to pancreatic resection when steroid treatment would be sufficient. This review discusses the clinical, laboratory, histological and imaging findings that are seen in autoimmune pancreatitis with particular focus on diagnosis. With the improvement of the diagnostic work-up less unnecessary pancreatic resections are expected to happen in patients with autoimmune pancreatitis.]

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[ENDOTHELIAL DYSFUNCTION IN DYSLIPIDAEMIA - THE ROLE OF THE RENIN-ANGIOTENSIN SYSTEM]

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[Dyslipidaemia is one of the known main risk factors of atherosclerosis by causing endothelial dysfunction that initiates and promotes vascular remodelling. Recent data from experimental and clinical studies suggest the existence of lipoprotein- neurohormonal interactions that may adversely affect vascular structure and function. Elevated lipid levels enhance the activation of the renin-angiotensin system, and, on the other hand, activation of the renin-angiotensin system leads to increased LDL cholesterol biosynthesis and oxidized-LDL uptake. These findings may explain the synergistic effect on cardiovascular risk observed in patients with coexisting hypertension and dyslipidaemia. The combined use of cholesterol-lowering drugs and inhibition of the tissue renin-angiotensin system may be more efficient in reducing cardiovascular risk.]

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[WAYS OF PREVENTION OF SEPTIC COMPLICATIONS IN ACUTE PANCREATITIS]

OLÁH Attila

[Similarly to other acute inflammatory responses, the mortality curve of acute pancreatitis has two distinct peaks. The first one, which coincides with a hyperinflammatory phase, is due to the development of an overwhelming systemic inflammatory response syndrome and subsequent multi-organ failure. The second peak of mortality is detected much later, after 14 days from the onset of the disease, when the compensatory antiinflammatory phase results in the infection of the necrotising pancreatic glandular substance. Since no therapy has been shown to efficiently prevent the activation of inflammatory and proteolytic cascades that evoke and sustain the disease, the treatment of acute pancreatitis is basically symptomatic. Beside adequate fluid and volume replacement and pain relief, medical and mechanical support may become necessary if organ failure develops. Recent studies suggest that there are ways to decrease the incidence of infection in pancreatic necrosis, which is usually due to bacterial translocation from the gut. The results of attempts to decrease the frequency of septic complications are controversial. A number of studies support the need of antibiotic prophylaxis but the evidence is weak. Furthermore, the increasingly observed infections by multi-resistant strains of Gram-positive bacteria and Candida species are due to long-term antibiotic use, which strongly questions the grounds for prophylactic antibiotic treatment. Recently, various clinical studies aimed to decrease bacterial translocation in other ways, including probiotic use and enteral feeding. This paper provides a systematic review of the data available in the evidence-based literature on the use of antibiotics and the role of alternative and adjuvant therapy in the treatment of severe acute pancreatitis.]

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dr. ALEXIN Zoltán

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[THE ROLE OF OBESITY IN GASTROENTEROLOGICAL DISEASES]

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[This paper reviews the current knowledge on the association of obesity and gastrointestinal disorders. While the relationship between obesity and cardiovascular disorders has recently gained wide professional publicity, there are few data on the gastroenterological aspects of obesity. After a discussion on obesity as an epidemic, its international and national prevalence, and public health risks, the most common obesity-related gastrointestinal disorders, their incidence, pathomechanism and consequences are presented by the organ systems affected, including gastrooesophageal reflux disease, fatty liver, gallstone disease, diseases of the pancreas, and colorectal carcinoma. Finally, the means of prevention and treatment are summarized.]

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[THE DIFFERENTIATION OF CHRONIC PANCREATITIS FROM PANCREATIC CANCER]

CZAKÓ László

[The poor prognosis of pancreatic cancer is mainly due to late diagnosis. The differentiation between pancreatic cancer and chronic pancreatitis is difficult, because the two diseases cause similar clinical symptoms and morphological alterations. Furthermore, chronic pancreatitis is associated with an increased risk of malignant transformation, thus the two diseases may be present simultaneously. The recent developments in molecular genetic tests and in imaging techniques, such as multidetector computed tomography, magnetic resonance cholangio- pancreatography, endoscopic ultrasoundguided fine needle aspiration and positron emission tomography/computed tomography, have fundamentally transformed the differentiation of the two disorders. This paper provides a systematic review of the recent evidence-based results concerning the differentiation of pancreatic cancer and chronic pancreatitis.]

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[DISEASES OF THE EXOCRINE PANCREAS AND DIABETES MELLITUS]

CZAKÓ László

[Exocrine and endocrine pancreas constitutes close anatomical and functional links accordingly, any disease affecting one of these sectors will inevitably affect the other. Acute and chronic pancreatitis, pancreatic surgery, cystic fibrosis and pancreatic cancer are those pancreatic conditions that might cause diabetes mellitus. The development of diabetes greatly influences the prognosis and quality of life of patients with exocrine pancreatic diseases. The lack of glucagon and the impaired absorption of nutrients may cause life-threatening complications, such as hypoglycaemia, and the micro- and macrovascular complications may impair the organ functions. Diabetes mellitus is an independent risk factor of mortality in those with exocrine pancreatic diseases. Pancreatic diabetes is a distinct metabolic and clinical form of diabetes, requires special treatment. Diet and pancreatic enzyme replacement therapy may be sufficient in the early stages. Oral antidiabetic drugs are not recommended. If the diet proves inadequate to reach the glycaemic goals, regular insulin treatment is demanded. There are special impairments of the exocrine function and the pancreatic morphology at diabetic patients that resemble to chronic pancreatitis. Atrophy of the exocrine tissue may caused by the lack of trophic insulin. Hyperglycaemia can activate the stellate cells that lead to pancreatic fibrosis. The microangiopathy and neuropathy, as well as the lack of islet hormone action - responsible for the exocrine pancreas regulation - will cause further damage on the pancreas glandular tissue. In the event of a proven impairment of the pancreatic exocrine function in diabetes mellitus, pancreatic enzyme replacement therapy is recommended. This may improve the nutritional condition and decrease the metabolic instability.]

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[Autoimmune pancreatitis in Hungary: a national multicenter study]

CZAKÓ László, GYÖKERES Tibor, TAKÁCS Tamás, TOPA Lajos, SAHIN Péter, DUBRAVCSIK Zsolt, SZEPES Attila, PAP Ákos, FÖLDESI Imre, TISZLAVICZ László, WITTMANN Tibor

[BACKGROUND - Autoimmune pancreatitis (AIP) is an increasingly recognised, special form of chronic pancreatitis, which greatly differs from other forms of chronic pancreatitis. Most papers on this condition have been published in Japan. METHODS - In our multicenter study, we aim to present the characteristics (demographics, clinical symptoms, laboratory and morphological findings, extrapancreatic symptoms, response to therapy, remission) of the first 13 Hungarian cases of AIP. RESULTS - The mean age at presentation was 44.2 years (range: 19-74); 54% of patients were women. New-onset mild abdominal pain (77%), weight loss (38%) and jaundice (31%) were the most common symptoms, with ulcerative colitis as the most frequent (38%) extrapancreatic manifestation. Diffuse pancreatic swelling was seen in 7 patients (54%), and a focal mass in 5 (38%). Pancreatic duct strictures were present in all patients. Serum immunoglobulin-G4 level was elevated in 71% of the patients in whom it was measured. All percutaneous core biopsies (4 patients) and surgical specimens (2 patients), and 2 of the 4 biopsies of the papilla of Vater revealed hystological findings typical for AIP: periductal, diffuse lymphoplasmacytic infiltration, marked interstitial fibrosis, and obliterative phlebitis. Immunostaining revealed IgG4-positive plasma cells in 57% of the patients in whom it was examined. Granulocytic epithelial lesions (GEL) were detected in 3 patients. These patients were younger (mean age 34 years), 66% were women and 6% had ulcerative colitis, whereas the mean age of patients without GEL was 65 years, and the majority of them were men. Steroid treatment resulted in remission of the symptoms in all patients. Because of suspicion of pancreatic tumour, 2 patients with focal AIP underwent partial pancreatectomy. Symptoms relapsed in one patient, but an increased dose of steroid resulted in a remission, which was maintained by azathioprine therapy. CONCLUSIONS - In our first Hungarian cases, we have confirmed previously reported characteristics of AIP. AIP with GEL is frequent among our patients; this condition is more frequent among women and younger patients, and is often associated with ulcerative colitis. If AIP is suspected, the performance of percutaneous biopsy is highly recommended. The therapeutic response to steroid therapy was excellent.]

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[THE ROLE OF ENZYME REPLACEMENT THERAPY IN PANCREATIC DISEASES ASSOCIATED WITH MALDIGESTION]

TAKÁCS Tamás

[The pancreas synthesizes and secretes more than 20 digestive enzymes that hydrolyze the major nutritive components, i.e., carbohydrates, fat and protein, within the lumen of the small bowel. In several pancreatic diseases the secretory capacity of the pancreas gradually decreases and the release of pancreatic juice becomes blocked resulting in the characteristic symptoms of maldigestion. Pancreas-associated maldigestion is most often caused by chronic pancreatitis, which can be diagnosed primarily by imaging beside history and clinical symptoms. There is no decisive serological test. The goal of the treatment of pancreatic insufficiency is to reduce symptoms (bloating, abdominal pain, weight loss, and, most importantly, steatorrhea) and improve digestion. One way to do this is to replace pancreatic enzymes. The efficiency of the available enzyme preparations can be increased by the improvement of lipase activity.]

Hungarian Radiology

[Examination of pancreatic exocrine function with secretin stimulated magnetic resonance cholangiopancreatography]

ENDES János, CZAKÓ László, TAKÁCS Tamás, BODA Krisztina, LONOVICS János

[INTRODUCTION - The aim of this study was to assess the feasibility and usefulness of SS-MRPD for evaluation of the pancreatic exocrine function. PATIENTS AND METHODS - SS-MRPD was performed in 20 patients with mild (n=8) or severe (n=12) chronic pancreatitis (according to the grade of exocrine pancreatic insufficiency indicated by the Lundh test) and in 10 volunteers without pancreatic disease. MRPD images were evaluated before and 10 min after the iv. administration of 0.5 IU/kg secretin. The changes in pancreatic tissue T2 signal intensity and duodenal filling after the injection of secretin were determined by means of SS-MRPD. The SSMRPD findings were then compared with those of the Lundh test. RESULTS - The basal pancreatic T2 signal intensity was significantly higher in the patients with a mild or a severe exocrine pancreatic insufficiency as compared with the controls (826.5±36.36 and 908±80.51 vs 659.2±41.67). The pancreatic T2 signal intensity exhibited a significant elevation after secretin administration both in the volunteers and in the patients with mild or severe chronic pancreatitis. This elevation was significantly lower in both the mild and the severe chronic pancreatitis patients than in the volunteers (66.85±15.77 and 24.45±5.85, respectively, vs. 200.0± 45.07). After the administration of secretin, the diameter of the duodenum was significantly increased in all three groups. This duodenal filling was significantly reduced in patients with a mild or a severe exocrine pancreatic insufficiency as compared with the volunteers (4.12±1.33 and 1.70±0.77 vs. 15.38± 1.73). There was no significant difference in pancreatic T2 signal intensity changes or in duodenal filling in patients with a mild or a severe exocrine pancreatic insufficiency. There were significant correlations between the pancreatic T2 signal intensity changes and the duodenal filling and the results of the Lundh test (r= -0.616 and -0.78). CONCLUSION - These results demonstrate that the administration of secretin increases the T2 signal intensity of the pancreatic tissue and the diameter of the duodenum to different extents in normal subjects and in patients with chronic pancreatitis. This suggests that SS-MRPD can provide information of value in the assessment of an exocrine pancreatic insufficiency.]