3rd Shifting - LQ1 part 2 Flashcards
connect the bile canaliculi to bile ductules in the periportal region
Canals of Hering
Major 1° Liver diseases
(1) viral hepatitis
(2) alcoholic liver disease
(3) nonalcoholic fatty liver disease (NAFLD)
(4) hepatocellular carcinoma
PATTERNS OF HEPATIC INJURY
Degeneration and intracellular accumulation Necrosis and apoptosis Inflammation Regeneration Fibrosis
Most severe clinical consequence of liver disease
Hepatic Failure
the encephalopathy develops rapidly within 2 weeks of the onset of jaundice
fulminant liver failure
if the encephalopathy develops within 3 months of the onset of jaundice
subfulminant liver failure
Acute liver illness that is associated with encephalopathy within 6 months after the initial diagnosis
Acute Liver Disease
characteristic “musty” or “sweet and sour” body odor due to Shunting of sphlancnic blood from the portal into the systemic circulation
Fetor hepaticus
Hepatopulmonary Syndrome Clinical triad
Chronic liver disease
Hypoxemia
Intrapulmonary vascular dilation
12th most common cause of death; Account for most liver-related death
CIRRHOSIS
CHARACTERISTICS of Liver Cirrhosis
Bridging fibrous septae
Parenchymal nodules
Disruption of architecture of entire liver
Transient mild unconjugated hyperbilirubinemia
NEONATAL JAUNDICE (physiologic jaundice of the newborn)
Defect in hepatocellular excretion of bilirubin glucuronides across canalicular membrane
Dubin-Johnson syndrome
mutation in the ATP8B1 gene on chromosome 18q21 that causes impaired bile secretion
PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS (PFIC) 1
caused by mutations in the hepatocyte canalicular bile salt export pump (BSEP), encoded by the ABCB11 gene.
PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS (PFIC) 2
caused by mutations in the ABCB4 gene, characterized by cholestasis with a high serum GGT
PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS (PFIC) 3
Chronic hepatitis indistinguishable from chorionic viral hepatitis; pathogenesis is attributed to T cell–mediated autoimmunity
AUTOIMMUNE HEPATITIS
Antibodies circulating in type 1 Autoimmune Hepatitis
antinuclear (ANA)
anti–smooth muscle (SMA)
anti–actin (AAA),
anti–soluble liver antigen/liver-pancreas antige (anti-SLA/LP)
Antibodies circulating in type 2 Autoimmune Hepatitis
anti–liver kidney microsome-1 (ALKM-1) antibodies
anti–liver cytosol-1 (ACL-1)
is the leading cause of drug-induced acute liver failure
acetaminophen
rare and potentially fatal syndrome of mitochondrial dysfunction in liver, brain, and elsewhere
Reye syndrome
Clinical Features of Alcoholic Liver Disease
Hepatic Steatosis (Fatty Liver)
Alcoholic Hepatitis
Alcoholic Cirrhosis
cytokeratin intermediate filaments and other proteins; eosinophilic cytoplasmic inclusions in degenerating hepatocytes
Mallory Bodies
Mutations in the Jagged 1 on chromosome 20p; Syndrome of paucity of bile ducts; Arteriohepatic Dysplasia
Alagille syndrome
larger ducts of the intrahepatic biliary tree are segmentally dilated
Caroli Disease
portal tracts are enlarged by irregular, broad bands of collagenous tissue, forming septa that divide the liver into irregular islands
Congenital hepatic fibrosis
small clusters of modestly dilated bile ducts embedded in a fibrous, sometimes hyalinized, stroma
Von Meyenburg Complexes
Hepatic vein thrombosis
Budd- Chiari syndrome
HELLP syndrome
Hemolytic anemia
Elevated Liver enzymes
Low Platelet
denotes a liver entirely transformed into roughly spherical nodules, in the absence of fibrosis
Nodular regenerative hyperplasia
appears as a well-demarcated but poorly encapsulated nodule, ranging up to many centimeters in diameter
Focal nodular hyperplasia
Cavernous hemangiomas
are the most common benign liver tumors.
benign neoplasms developing from hepatocytes
Liver cell adenoma
most common liver tumor of young childhood
Hepatoblastoma
third most frequent cause of cancer deaths
Hepatocellular carcinoma
Four major etiologic factors of Hepatocellular carcinoma
- chronic viral infection (HBV, HCV)
- chronic alcoholism
- non-alcoholic steatohepatitis (NASH)
- food contaminants (primarily aflatoxins)
second most common hepatic malignant tumor after HCC
Cholangiosarcoma
extrahepatic forms of Cholangiosarcoma located at the junction of the right and left hepatic ducts forming the common hepatic duct, and distal bile duct tumors
Klatskin tumors
most common anomaly of the gall bladder
folded fundus
Five F’s of Cholelithiasis prevalence
fair, fat, fertile, female, forty
Occasionally a large stone may erode directly into an adjacent loop of small bowel
Gallstone ileus or Bouveret’s syndrome
Characterized by recurrent attacks of severe pancreatitis usually beginning in childhood; Germline (inherited) mutation in the cationic trypsinogen gene (a.k.a. PRSS1)
HEREDITARY PANCREATITIS
Defined as inflammation of the pancreas with irreversible destruction of exocrine parenchyma, fibrosis and in the late stages, the destruction of endocrine parenchyma
Chronic Pancreatitis
o Autoimmune pancreatitis
o Characterized by a duct-centric mixed inflammatory cells infiltrate
LYMPHOPLASMACYTIC SCLEROSING PANCREATITIS
Migratory thrombophlebitis may occur due to elaboration of platelet-aggregating factors and procoagulants in Pancreatic cancer
Trousseau sign
rare neoplasms that occur primarily in children aged 1 to 15 years; distinct microscopic appearance with squamous islands admixed with acinar cells
PANCREATOBLASTOMA