Diseases Of The Gallbladder And Bile Ducts Flashcards
Defective transporter in progressive familial intrahepatic cholestasis type 2 and benign recurrent intrahepatic cholestasis type 1
F1C1
An amino phospholipid transferase (flippase) essential for maintaining the lipid assymetry of the canalicular membrane
F1C1
Hemitransporters ABCG5/G8 transport the __ and ___.
Cholesterol
Phytosterol
Defective transporter in progressive familial intrahepatic cholestasis type 3
MDR3 (ABCB4)
Defective ABCG5/G8 will lead to
Sitosterolemia
Ablation of all other ATP-dependent transporter functions is a result from a defective___ transporter.
F1C1
Mutation in Dubin-Jonhnson syndrome
MRP2 (ABCC2)
Inherited form of conjugated hyperbilirubinemia
Dubin-Jonhnson syndrome
Defective membrane regulator in cystic fibrosis
CFTR, ABCC7
Located on bile duct epithelial cells
Impaired cholangiocellular pH regulation during ductular bile formation and chronic cholestatic liver disease is associated with
Cystic fibrosis
Resulting in biliary cirrhosis
Normal bile acid pool size
2-4 grams
How many times per day the bile acid pool circulates?
5-10 times
Fecal loss of the bile acid is in the range of __
0.2-0.4 g/day
Bile salts are actively absorbed at the__
Distal ileum
Secondary bile acids:
Deoxycholate
Lithocholate
Ursodeoxycholic acid
Most frequently absorbed secondary bile acid
Deoxycholic acid
Maximum rate of bile acid synthesis
5 grams per day
Release by the intestine to suppress synthesis of bile acids from cholesterol by inhibiting the rate-limiting enzyme P450 7a1 (CYP P7A1)
FGF 19
Upregulator of ABCG5/G8
LXR - liver X receptor (an oxysterol sensor)
Upregulator of bile salt export pump (ABC B11)
FXR- Farnesoid X receptor (a bile acid sensor)
Normal capacity of the gallbladder
30 ml
Major factor that controls the evacuation of bile from the gallbladder
CCK
Action of CCK: (3)
- Powerful contraction of the gallbladder
- Decrease resistance of the sphincter of Oddi
- Enhanced flow of biliary contents into the duodenum
The most important mechanism in bile stone formation
Increased biliary secretion of cholesterol
Rate-limiting enzyme of hepatic cholesterol synthesis
HMG-CoA reductase
Variables that predict survival in PSC:
- age
- serum bilirubin
- histologic stage
- splenomegaly
Dose of UDCA in sclerosing cholangitis
20 mg/kg
Beaded appearance on cholangiography
PSC
Disease entities associated with PSC:
IBD
Ig G4-associated cholangitis
treatment for IgG4 associated cholangitis
Glucocorticoids
Signs and symptoms of PSC :
Biliary obstruction
RUQ pain
Jaundice, pruritus
Acute cholangitis
biliary pain, obstructive jaundice, melena
Hemobilia
Most common cause of obstructive jaundice
Pancreatic head mass
Bile leak or abcess formation in the early postoperative period, biliary obstruction or cholangitis as long as 2 years or more following the inciting trauma
strictures
Laboratory picture after relief of obstruction :
aminotransferase returns to normal rapidly
serum bilirubin 1-2 weeks to normalize
alkaline phosphatase level usually falls slowly
Acute cholecystitis presenting with back pain or pain to the left of the abdominal line, prolonged vomiting with paralytic ileus and pleural effusion on the left side
Pancreatitis
Cholecystitis with S. bilirubin 5-15 mg/dL
CBD stones
Cholecystitis with serum bilirubin of 20mg/dL
neoplastic obstruction
medical therapy for acute cholecystitis :
NPO Nasogastric suction Correct electrolytes and volume depletion Meperidine or NSAID IV antibiotic therapy
