Rubin's Liver (2) Flashcards
“dilation of collateral veins radiating about the umbilicus”
caput medusae
“dilated inferior hemorrhoidal veins”
Anorectal varices
“dilation of veins in the lower esophagus due to portal HTN”
esophageal varices
How does splenomegaly affect elements in blood?
Hypersplenism leads to a decreased lifespan of the formed elements (increased removal rate)
“fibrotic, Fe laden nodules caused by focal hemprrhages in the spleen”
Gamna- Gandy bodies
“accumulation of fluid in the peritoneal cavity”
ascites
“translocation of intestinal bacteria into the systemic circulation with secondary infection of ascites fluid”
spontanteous bacterial peritonitis
“renal hypoperfusion int he setting of cirrhosis due to systemic vasodilation, which prostaglandins are unable to counter”
hepatorenal syndrome
“creation of shunts of various sizes within the pulmonary vascular bed in the setting of portal hypertension”
Hepatopulmonary syndrome (HPS)
Pleural effusions, like ascites but in the chest”
hepatic hydrothorax
Most common cause of acute hepatitis?
hep A
Are Hep B/ Hep C directly cytopathic?
No! Mediated by CD8 T cell injury
“massive liver cell necrosis, hepatic failure, and high mortality”
fulminant hepatitis B
What are the common extrahepatic manifestatoins of acute hepatitis C?
mixed cryoglobulinemia–> vasculitis, sicca sundrome (salivary glands), mononeuritis, multiplex (nervous syndrome), MPGN (kidney
What is the hallmark of acute viral hepatitis?
Liver cell death
What are councilman bodies?
Apoptotic liver cells with pyknotic nuclei
What is confluent hepatic necrosis?
affects whole regions of the lobule, it is a severe form of acute viral hepatitis
“chronic hepatitis of unknown cause that is associated with circulating autoantibdies and high levels of serum immunoglobulins”
Autoimmune hepatitis
Which autoimmune hepatitis is associated with ANA(+) and anti- smooth muscle (+)?
Type 1
Which HLA type is associated with Type 1 Autoimmune hepatitis?
HLA- DRB1
Which autoimmune hepatitis is associated with anti LKM antibodies?
Type II
Which autoimmune hepatitis is usually seen in children ages 2-14?
Tpye II
“accumulation of fat in hepatocytes”
steatosis
3 risk factors for nonalcoholic fatty liver?
Obesity, T2DM, hyperlipidemia
“immune mediated chronic progressive cholestatic liver disease”
Primary biliary cirrhosis
“chronic cholestatic liver disease of unknown cause”
primary sclerosing cholangitis
“segmental disease associated with “beaded” intrahepatic biliary tree”
primary sclerosing cholangitis
Immune findings in primary sclerosing cholangitis?
High Ig, p-ANCA, and T cell count
“excessive accumulation of iron in the body”
siderosis
“excess body Fe leading to deposition in tissues (hemosiderosis) and organ damage (hemochromatosis)”
hemochromatosis
What causes the tissue damage in hemochromiatosis?
generation of free radicals
Mutation in juvenile hemochromatosis?
HJV mutation
Mutation in hemochromatosis?
AR mutation in HFE transmemebrane gene
primary iron storage protein?
ferritin
What results from ferritin degradation?
Hemosiderin
What regulates plasma Fe by binding to ferroportin?
Hepcidin
What controls hepcidin synthesis?
TfR2, HJV, HFE
Pancreatic complications of hereditary hemochromatosis?
diabetes (bronze diabetes= pigmented skin + glucose intolerance)
cause of hyperpigmentation in hereditary hemochromatisos?
increased melanin (Fe not always present in pigments)
When does HH become symptomatic?
midlife
Cirrhosis + HH can lead to….
hepatocellular carcinoma
causes of secondary Fe overolad syndromes?
alcohol abuse, hemolytic anemia,s