1. IL FEGATO IL PANCREAS…..ed I loro segnali
Valerio Nobili, M.D.
Responsabile UOC Malattie Epatometaboliche
Direttore Laboratorio di Ricerca Malattie Epatiche
Ospedale Bambino Gesu’

2. I do not have any conflict of interest

6. ……….AND A LITTLE MORE FOR THE PEDIATRICIAN !!!
ISOLATED ALTERATIONS OF BIOCHEMICAL MARKERS OF LIVER DAMAGE IN AN APPARENTLY HEALTHY PATIENT CAN REPRESENT A CHALLENGE FOR THE CLINICIAN…….
……….AND A LITTLE MORE FOR THE PEDIATRICIAN !!!

8. ….Therefore, persistently elevated aminotransferases in a child should alert the physician to the possibility of an underlying liver or multisystem disorder and prompt a referral to a specialized center for diagnostic evaluation and management.
T. Lamireau et al 2014

9. 2008, Volume 371, Number 9628,
VENTO S, and NOBILI V

10. “the finding of even modest increases of aminotransferase concentrations should not be overlooked, as it could provide an opportunity for clinicians to reveal and treat not only serious hepatic diseases but also to modify detrimental habits that can be life-threatening in the long term.”

11. Hepatology 2008;47(3):880-87

13. which is not directly correlated to disease severity.
The degree of emergency is indicated by the association with increased prothrombin time indicating impending liver failure, and not by the level of increased aminotransferases
which is not directly correlated to disease severity.
T. Lamireau et al 2014

14. T. Lamireau et al 2014

29. Autoimmune Hepatitis Histology
Lymphoplasmacytic infiltrate
Interface hepatitis
Portal inflammation and invasion of limiting plate

33. Serum immunoglobulin G4 and immunoglobulin G1 for distinguishing immunoglobulin G4-associated cholangitis from primary sclerosing cholangitis
Hepatology 59, 5, 1954–1963, May 2014
The recent addition of immunoglobulin (Ig)G4-associated cholangitis (IAC), also called IgG4-related sclerosing cholangitis (IRSC),…………,but interpretation of elevated sIgG4 levels remains unclear.

38. macrovesicular fatty infiltrate (lipocytes, black arrowhead) and chronic portal inflammation (inflammatory cells, white arrows), characteristic seen in nonalcoholic steatohepatitis

39. 40x views, respectively, of trichrome stain highlighting perivenular fibrosis (black arrows) and steatosis (white arrows).

41. CF liver disease:
up to 25% with clinically significant disease
Pathogenesis: biliary ductal obstruction from inspissated secretions  biliary fibrosis and cirrhosis
Monitoring: assess HSM, liver enzymes & GGT annually, abdominal ultrasound
Treatment: ursodeoxycholic acid; referral to hepatologist if cirrhosis/portal hypertension