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CIRRHOSIS. DEFINITION END STAGE CHRONIC LIVERDISEASE CHARECTERISED BY 1. BRIDGING FIBROUS SEPTAE 2. PARENCHYMAL NODULES 3. DISRUPTION OF THE ARCHITECTURE OF THE ENTIRE LIVER. ETIOLOGIC FACTORS. 1. ALCOHOLIC LIVER DISEASE -60-70% OF CASES
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CIRRHOSIS DEFINITION END STAGE CHRONIC LIVERDISEASE CHARECTERISED BY 1. BRIDGING FIBROUS SEPTAE 2. PARENCHYMAL NODULES 3. DISRUPTION OF THE ARCHITECTURE OF THE ENTIRE LIVER
ETIOLOGIC FACTORS 1. ALCOHOLIC LIVER DISEASE -60-70% OF CASES 2. POSTNECROTIC CIRRHOSIS INCLUDING VIRAL HEPATITIS – 10% 3. BILIARY DISEASES- 5 – 10%
ETIOLOGIC FACTORS 4. HEREDITARY HAEMOCHROMATOSIS -5% 5. WILSON’S DISEASE 6. ALFA 1 ANTITRYPSIN DEFICIENCY 7. CRYPTOGENIC CIRRHOSIS- 10-15%
OTHER INFREQUENT CAUSES OF CIRRHOSIS IN INFANTS CHILDREN GALACTOSEMIA & TYROSINOSIS DRUG INDUCED CIRRHOSIS SYPHILIS CARDIAC CIRRHOSIS INDIAN CHILDHOOD CIRRHOSIS
PATHOGENESIS THREE MAJOR PATHOLOGIC MECHANISMS 1. HEPATOCELLULAR DEATH 2. REGENERATION 3. PROGRESSIVE FIBROSIS
POSTNECROTIC CIRRHOSIS(MACRONODULAR CIRRHOSIS) LARGE IRREGULAR NODULES WITH BROAD BANDS OF CONNECTIVE TISSUE.
ETIOLOGY OF POSTNECROTIC CIRRHOSIS 1. VIRAL HEPATITIS–HEPATITIS B HEPATITIS C –MOST COMMON CAUSE 2. DRUGS & CHEMICAL HEPATOTOXINS 3. IDIOPATHIC
MORPHOLOGY OF POSTNECROTIC CIRRHOSIS GROSS - SMALL LIVER < 1 Kg DISTORTED NODULES OF VARYING SIZES WITH IRREGULAR COARSE SCARS
MICROSCOPIC FEATURES OF POSTNECROTIC CIRRHOSIS 1. NORMAL LOBULAR ARCHITECTURE OF LIVER IS NOT COMPLETELY LOST 2. THICK FIBROUS SEPTA CONTAINING CHRONIC INFLAMMATORY CELLS & PROLIFERATING BILE DUCTULES.
MICROSCOPIC FEATURES OF POSTNECROTIC CIRRHOSIS ACTIVE LIVER CELL NECROSIS MAY BE SEEN REGENERATING NODULES WITH VARYING SIZED LIVER CELLS WITH LARGE NUCLEI.
BILIARY CIRRHOSIS FOLLOW LONG CONTINUED CHOLESTASIS OF INTRAHEPATIC OR EXTRAHEPATIC ORIGIN TWO TYPES – 1. PRIMARY BILIARY CIRRHOSIS OBSTRUCTIVE PROCESS OF UNKNOWN ETIOLOGY AFFECTING INTRAHEPATIC BILE DUCTS 2.SECONDARY BILIARY CIRRHOSIS
PRIMARY BILIARY CIRRHOSIS MIDDLE AGED WOMEN FAMILIAL INCIDENCE HYPERCHOLESTEROLEMIA AUTOIMMUNE ORIGIN
SECONDARY BILIARY CIRRHOSIS PROLONGED OBSTRUCTION OF EXTRAHEPATIC BILIARY PASSAGES EXAMPLE- 1. CHOLELITHIASIS , 2. BILIARY ATRESIA,
BILIARY SECONDARY CIRRHOSIS 3. CANCER OF BILIARY TREE & HEAD OF PANCREAS 4. POSTOPERATIVE STRICTURES
MORPHOLOGY OF BILIARY CIRRHOSIS GROSS - (BOTH PRIMARY & SECONDARY) - INITIALLY ENLARGED GREENISH YELLOW LIVER, LATER SMALLER FIRMER MICRONODULAR
MICROSCOPY OF PRIMARY BILIARY CIRRHOSIS MICROSCOPY – PRIMARY BILIARY CIRRHOSIS STAGE 1. PORTAL TRACTS SHOW DESTRUCTION OF BILE DUCTS, INFLAMMATION ,GRANULOMAS STAGE2. BILE DUCTULAR PROLIFERATION
MICROSCOPY OF PRIMARY BILIARY CIRRHOSIS INFLAMMATION EXTENDING INTO THE PERIPORTAL HEPATIC PARENCHYMA WITH MELLORY BODIES. STAGE 3. FIBROUS SCARRING CONNECTING THE PORTAL AREAS
MICROSCOPY OF PRIMARY BILIARY CIRRHOSIS NUMBER OF BILE DUCTS REDUCED INFLAMMATORY INFILTRATE IS REDUCED.
MICROSCOPY OF PRIMARY BILIARY CIRRHOSIS STAGE 4. WELL FORMED MICRONODULAR CIRRHOSIS
MICROSCOPIC FEATURES OF PRIMARY BILIARY CIRRHOSIS LATE STAGE
SECONDARY BILIARY CIRRHOSIS-GROSS & MICROSCOPY GROSS 1. YELLOW GREEN PIGMENTATION 2. O/S –HARD FINELY GRANULAR MICROSCOPY – JIGSAW PATTERN OF COARSE FIBROUS SEPTAE
SECONDARY BILIARY CIRRHOSIS-GROSS & MICROSCOPY DISTENDED LARGE & SMALL BILE DUCTS CONTAINING BILE PLUGS EXTENSSIVE PROLIFERATION OF SMALL BILE DUCTS WITH ASCENDING CHOLANGITIS,PYLEPHLEBITIS, BILE LAKES
SECONDARY BILIARY CIRRHOSIS-MICROSCOPY –DILATED PROLIFERATING BILE DUCTS WITH BILE PLUGS
CLINICAL FEATURES OF BILIARY CIRRHOSIS INCIDIOUS ONSET PRURITUS DARK URINE
CLINICAL FEATURES OF BILIARY CIRRHOSIS PALE STOOL JAUNDICE SKIN PIGMENTATION
PIGMENT CIRRHOSIS IN HAEMOCHROMATOSIS AN IRON STORAGE DISORDER EXCESSIVE ACCUMULATION OF IRON IN PARENCHYMAL CELLS WITH EVENTUAL TISSUE DAMAGE
PIGMENT CIRRHOSIS IN HAEMOCHROMATOSIS ORGANS MAINLY AFFECTED – LIVER, PANCREAS,HEART, PITUITARY BRONZE DIABETES
CLASSIFICATIONOF HAEMOCHROMATOSIS 1. IDIOPATHIC( PRIMARY)TYPE 2. SECONDARY(AQUIRED) TYPE
ETIOPATHOGENESIS OF IDIOPATHIC HAEMOCHROMATOSIS AUTOSOMAL RECESSIVE EXCESSIVE INTESTINAL ABSORPTION OF DIETARY IRON
ETIOPATHOGENESIS OF IDIOPATHIC(PRIMARY) HAEMOCHROMATOSIS HEREDITARY HEMOCHROMATOSIS GENE ON SHORT ARM OF CHROMOSOME 6 DISEASE MANIFESTS AFTER 20 g OF STORAGE IRON GETS ACCUMULATED
ETIOPATHOGENESIS OF IDIOPATHIC HAEMOCHROMATOSIS MECHANISM OF INJURY 1. LIPID PEROXIDATION
ETIOPATHOGENESIS OF IDIOPATHIC HAEMOCHROMATOSIS 2. STIMULATION OF COLLAGEN FORMATION 3. DIRECT INTERACTION WITH DNA – HEPATOCELLULAR CARCINOMA
ETIOPATHOGENESIS OF SECONDARY HAEMOCHROMATOSIS EXCESS IRON RESULTS FROM MULTIPLE TRANSFUSIONS IN SICKLE CELL ANEMIA,APLASTIC ANEMIA
ETIOPATHOGENESIS OF SECONDARY HAEMOCHROMATOSIS PARENCHYMAL INJURY OCCURS RARELY IN SUB SAHARAN AFRICA – ALCOHOLIC BEVERAGES - BANTU SIDEROSIS
MORPHOLOGY OF HAEMOCHROMATOSIS 1. DEPOSITION OF HEMOSIDERIN IN LIVER , PANCREAS , MYOCARDIUM PITUITARY,ADRENAL THYROID & PARATHYROID GLANDS,JOINTS & SKIN
MORPHOLOGY OF HAEMOCHROMATOSIS 2. CIRRHOSIS 3. PANCREATIC FIBROSIS
MORPHOLOGY OF LIVER IN HEMOCHROMATOSIS GROSS –ENLARGED ,FIRM , CHOCOLATE BROWN INTENSELY PIGMENTED LIVER WITH MICRONODULAR CIRRHOTIC PATTERN WITH FIBROUS BANDS
LIVER IN HAEMOCHROMATOSISMICROSCOPIC FEATURE INITIALLY GOLDEN YELLOW HEMOSIDERIN GRANULES IN THE PERIPORTAL HEPATOCYTES
LIVER IN HAEMOCHROMATOSISMICROSCOPIC FEATURE WITH PROGRESSIVE INVOLVEMENT OF REST OF THE LOBULE, BILE DUCT EPITHELIUM, KUPFFER CELLS. PRUSSIAN BLUE STAIN
LIVER IN HAEMOCHROMATOSISMICROSCOPIC FEATURE IRON HAS DIRECT HEPATOTOXICITY FIBROUS SEPTA DEVELOP SLOWLY INFLAMMATION-- IS ABSENT
MORPHOLOGY OF PANCREAS IN HEMOCHROMATOSIS INTENSELY PIGMENTED WITH DIFFUSE INTERSTITIAL FIBROSIS PARENCHYMAL ATROPHY HEMOSIDERIN IN – ISLET CELLS ACINAR CELLS & FIBROUS STROMA