1. Prof. Giuseppe Castaldo, a.y. 2015-16 Course of Advanced Diagnostics Laboratory evaluation of exocrine pancreas

3. Hormones produced by pancreatic islets CellHormone Effect Alphaglucagonhyperglycemic Betainsulinhypoglycemic Deltasomatostatininhibition of GH secretion PPpancreatic polypeptidereduce intestinal motility

7. -Acute pancreatitis: -Autoactivation of pancreatic enzymes -Autodigestion of the pancreas -Release of pancreatic enzymes in blood -Chronic pancreatitis: -Fibrotic replacement of exocrine pancreas -Reduced production of pancreatic enzymes -Reduced digestion

12. Laboratory diagnosis of acute pancreatitis Amylase and Lipase

15. -Serum pancreatic isoenzyme of amylase (immunoassay) -P3 isoenzyme of amylase (electrophoresis) To increase the diagnostic specificity of amylase

20. J Gastroenterol. 2006 Sep;41(9):901-8. Fecal pancreatic elastase: a reproducible marker for severe exocrine pancreatic insufficiency. Naruse S1, Ishiguro H, Ko SB, Yoshikawa T, Yamamoto T, Yamamoto A, Futakuchi S, Goto H, Saito Y, Takahashi S. Author information Abstract BACKGROUND: In order to apply fecal pancreatic elastase for follow-up of exocrine pancreatic function in chronic pancreatitis and cystic fibrosis, we examined the sensitivity, specificity, and long-term variability of a new polyclonal antibody-based enzyme-linked immunosorbent assay (ELISA). METHODS: Patients with definite chronic pancreatitis (n = 23), probable or possible chronic pancreatitis (n = 14), autoimmune pancreatitis (n = 7), or acute pancreatitis (n = 11), and 51 healthy subjects and 11 healthy infants participated in this study. Pancreatic function was graded as normal (n = 3), mild (n = 18), moderate (n = 9), or severe (n = 18) exocrine insufficiency on the basis of secretin tests. Fecal pancreatic elastase was measured by a new ELISA. RESULTS: Fecal pancreatic elastase concentration in control subjects varied widely, with a median of 478 microg/g. The specificity of this test was 90.2% with a cutoff value of >200 microg/g. The sensitivities were 60.9% for detecting definite chronic pancreatitis, 76.5% for calcifying pancreatitis, 71.4% for autoimmune pancreatitis, and 7.1% for probable or possible chronic pancreatitis. The sensitivities were 16.7% for mild, 12.5% for moderate, and 72.2% for severe exocrine pancreatic insufficiency. Forty patients were reexamined after a median interval of 347 days. The fecal pancreatic elastase levels between the first and second tests were not significantly different. Two infants, 4.5 and 5 months old, had abnormally low values, but after a median of 304 days all infants showed normal levels (median, 444 microg/g). CONCLUSIONS: Fecal pancreatic elastase is a reproducible marker for severe exocrine pancreatic insufficiency. This test is valuable for longitudinal follow-up of exocrine pancreatic function.

22. Families with two or more relatives with idiopathic pancreatitis. Families with at least one case of pancreatitis and a confirmed causative mutation in the PRSS1 gene.

23. Hereditary pancreatitis (HP) -Recurrent acute pancreatitis -Chronic pancreatitis -Ealry onset (before 10 years) -Absence of known etiologic factors -Familiarity -Mild onset -Pancreatic calcifications

24. 3 – 10 : 100.000 / year 180 – 600 cases in Campania region per year ? Onset (median): 10 – 11 years

25. Evolution and complications of HP Recurrent episodes of pancreatitis Pseudocysts, ascites Evolution in exocrine and endocrine pancreatic insufficiency High risk (60x) of adenocarcinoma

26. Genetic factors Sono geni implicati nel controllo dell'attivita' della tripsina nel pancreas

27. SPINK1 and CFTR in the pathogenesis of HP AP: Activation peptide

28. PRSS1 in the pathogenesis of HP

30. Molecular analysis Patients with recurrent acute pancreatitis Patients with chronic pancreatitis When ? Familiarity for pancreatitis Early onset Absence of known etiologic factors