Path 7 Intrahepatic Flashcards
Bile secreted per day
1 liter
Bile pathway
Bile canaliculi -> Canals of Hering -> bile ductules -> terminal bile ducts
Biliverdin v Bilirubin
water soluble v lipid soluble
Direct bilirubin
- Unconjugated
- Transported on Albumin
Bilirubin in liver
Conjugated with Glucaronic acid by
-UGT1A1 (Uridine diphosphate glucuronyl transferase)
Bilirubin in gun
Bilirubin diglucuronide
-Hydrolized by bacteria to Urobilinogen
Bile composition
Bile salts (90% reabsorbed)
Phospholipids
Cholesterol
Bilirubin
Bile function
- Excretion of endogenous lipids
- Absorption of dietary lipids
Jaudice
-Bilirubin above 2.0 mg/dl
Unconjugated bilirubinemia (3 causes)
- Hemolysis
- Reduced uptake (drug, genetic)
- Impaired conjugation (genetic, physiologic newborn)
Conjugated bilirubinemia (2 causes)
- Decreased excretion (cirrhosis, hepatitis)
- Impaired bile flow
Physiologic jaundice of newborn (cause, pathology, exacerbation, Tx)
- Transient low UGT1A1 activity
- Direct bilirubin deposited in basal ganglia (kernicterus)
- Exacerbated by breast feeding
- Tx with phototherapy
Dubin-Johnson syndrome
- Recessive conjugated hyperbilirubinemia
- Pigmented cytoplasmic globules
- MRP2 mutation (canalicular multidrug protein2)
- Impaired bilirubin glucuronide excretion
Rotor syndrome
- Recessive conjugated hyperbilirubinemia
- Decreased hepatic uptake and storage
Gilbert syndrome
- Recessive unconjugated hyperbilirubinemia
- Decreased UGTA1
Criglar-Najjar syndrome type 1
- Recessive unconjugated hyperbilirubinemia
- Absent UGTA1
- Fatal
Crigler-Najjar syndrome type 2
- Recessive unconjugated hyperbilirubinemia
- Decreased UGTA1
- Mild course, occasional kernicterus
Secondary biliary cirrhosis
Due to obstruction of extrahepatic biliary tree
Primary biliary cirrhosis clinical
- Obstruction symptoms
- Xanthomas, skin pigmentation
- Osteomalacia (due to vit. D malabsorption)
- Cirrhosis
Primary biliary cirrhosis labs
- Antimitochondrial Ab
- Elevated biliarubin, alkaline phosphatase
Primary biliary cirrhosis histo
- Granulomas (epithelioid histiocytes)
- Mixed inflammatory cells around ducts
- Duct proliferation, native ductopenia
Primary sclerosing cholangitis histo
-Onion-skin periductal fibrosis and inflammation
Primary sclerosing cholangitis
- 80% have Ulcerative Colitis
- Obstruction symptoms
- Cholangiocarcinoma risk
Primary sclerosing cholangitis labs
- p-ANCA
- Elevated IgG4 (autoimmune pancreatitis)
- Elevated serum alkaline phosphatase
