Biliary diseasesBiliary diseases Vascular diseasesVascular diseases ParasitesParasites TumorsTumors Liver transplantationLiver transplantation Liver Pathology.

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Presentation transcript:

Biliary diseasesBiliary diseases Vascular diseasesVascular diseases ParasitesParasites TumorsTumors Liver transplantationLiver transplantation Liver Pathology 4:

Causes of Large Bile Duct Obstruction

Extrahepatic Biliary Atresia (EHBA) Reovirus 3 Rotavirus Immune targeting?

Kasai Hepatic Portoenterostomy Loop of small intestine EHBA Rx Liver Transplantation

Primary Sclerosing Cholangitis (PSC) ERCP: endoscopic retrograde cholangiopancreatography

, fewer Crohn’s

Mrs. Elizabeth Peacock Primary Biliary Cirrhosis (PBC) Guys’ Hospital Report London, 1851 Addison & Gull

PBC: Primary biliary cirrhosis---- Chronic nonsuppurative destructive cholangitis middle-aged women + AMA (anti-mitochondrial antibodies); M2 subtype Alkaline Phosphatase IgM AMA directed against inner mitochondrial membrane PDC-E2 (pyruvate dehydrogenase complex-E2) florid bile duct lesion bile duct PDC-E2 HLA II AMA T T T Assoc. with other autoimmune dis: e.g. RA, sicca, celiac, scleroderma Molecular Mimicry? Gram – bacteria

Vascular Disease & the Liver Veins Sinusoids Arteries Liver is often involved in heart disease or other outflow problems (“hepatic venous outflow obstruction”)

Budd-Chiari Syndrome (Hepatic venous outflow obstruction) ascites oral contraceptives coagulopathy (prot. S, C, factor V Leiden def., anti-cardiolipin Ab’s tumor invasion of hep. V’s or IVC: renal cell CA + HCC small vein disease: VOD

Peliosis hepatis : blood lakes of the liver -HIV, C17-alkylated steroids (OC’s, anabolic steroids; systemic inflamm.

Infarct: Arterial disease (PAN, ligation, chemoRx installation) Portal vein: Zahn infarct

P.V. Pylephlebitis : -perforated ulcer, -ruptured appendix, etc. Pylephlebitis : -perforated ulcer, -ruptured appendix, etc.

Schistosomiasis: Pre-sinusoidal, intrahepatic portal hypertension (Symmers’ “clay pipestem fibrosis”)

pancreas

LIVER-CELL ADENOMA -oral contraceptives -hepatocyte nuclear factor 1α (HNF-1α) mutations -benign hepatocytes, bld vessels, NO b.d.’s

FOCAL NODULAR HYPERPLASIA (FNH) -malformation: central scar with artery, outgrowth of cirrhosis-like mass

HCC 75% of pts have cirrhosis 75% are serum AFP + Risks: Cirrhosis due to HBV, HCV, alcohol, hemochromatosis Invades veins: portal vein/hepatic vein/IVC-  to lungs

FIBROLAMELLAR Ca: young, no risks -better prognosis with resection/tx No cirrhosis

Cholangiocarcinoma associated with: Liver flukes: - Clonorchis sinensis -Opisthorcis viverrini PSC Gallstone disease, hepatolithiasis

Liver Transplantation Cirrhosis - HBV, HCV PBC PSC Extrahepatic biliary atresia AFLD/NAFLD Metabolic disorders

Portal tract HLA II T Acute Rejection BD endothelium

1.Acute rejection: triad 2.Preservation injury (ischemia/reperfusion) 3.CMV hepatitis 4.Recurrent original disease 5.De novo autoimmune hepatitis 6.Chronic rejection 6 mos. inflammation bile duct damage endotheliitis Liver Transplantation Pathology

Dame Sheila Sherlock The Royal Free Hospital London, U.K. “Diseases of the Liver & Biliary System”

Peter J. ScheuerHans Popper

Peter J. Scheuer, M.D. Royal Free Hospital Prof. of Histopathology “Liver Biopsy Interpretation” -1 st Edition: th Edition: 2005