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GEMS > Cholestasis & Gallstones > Flashcards

Cholestasis & Gallstones Flashcards

1
Q

Cholestasis

A

Functional –> decreased bile flow through the canaliculus & reduced secretion of water, bilirubin and by hepatocytes

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2
Q

Cholestasis

Gross & EM

A

+Presence of brownish bile pigment w/in dilated canaliculi in hepatocytes

+Microvilli are blunted, in fewer number or absent
–> bile accumulates in large, bile-laden lysosomes

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3
Q

Cholestasis

Symptoms

A

+ Jaundice: accumulation in blood of materials normally transferred to bile (bilirubin, cholesterol, bile acids)

+Pruritus: deposits of bile acids in skin

+ Skin xanthomas: focal accumulation of cholesterol

+ Intestinal malabsorption: nutritional deficiencies of vit A, D, K

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4
Q

Cholestasis

Etiology

A
  • Bile duct carcinoma
    -Enlarged lymph node (Hodkin)
  • Sclerosing cholangitis
    -Postoperative stricture
    -Pancreatic carcinoma
    -Gallstones
    -Congenital biliary atresia
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5
Q

Ascending Cholangitis

A

Bacterial infection of the bile ducts

Usually 2/2 an “ascending” infection w/ enteric gram NEG bacteria

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6
Q

Ascending Cholangitis

Histology, Sx, Complications

A

H: neutrophils

Sx: RUQ pain, fever, jaundice (Charcot triad)

Comp: Suppurative cholangitis, Sepsis

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7
Q

Suppurative Cholangitis

A

Severe form of cholangitis

Includes hypotension & mental confusion (Reynold’s pentad)

Sepsis (rather than cholangitis –> grave prognosis

Gross: purulent bile fills & distends bile ducts

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8
Q

Chronic biliary obstruction

Complications

A

-If uncorrected

-Hepatic scarring & regenerative nodule formation

-Secondary or obstructive biliary cirrhosis

-Ascending cholangitis may be superimposed on this chronic process –> some trigger acute on chronic liver failure

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9
Q

Chronic biliary obstruction

Micro

A

Feathery degeneration of periportal hepatocytes

-Hydropic swelling
-Diffuse impregnation w/ bile pigment
-Reticulated appearance

Formation of bile infarcts from detergent effects of extravasated bile

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10
Q

Secondary or Obstructive Biliary Cirrhosis

A

Complications of uncorrected biliary obstructions

Cirrhosis – destruction of normal liver architecture by fibrous bands around regenerative nodules 2/2 persistent liver cell necrosis

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11
Q

Secondary or Obstructive Biliary Cirrhosis

Pathogenesis

A

+ Involves death & regeneration of hepatocytes

+Extracellular matrix deposition by activated hepatic stellate cells

+Alterations in hepatic vascular architecture

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12
Q

Primary Biliary Cirrhosis (Cholangitis)

A

Chronic cholestatic disease in which intrahepatic bile ducts are destroyed by nonsuppurative inflammation

AUTOIMMUNE

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13
Q

Primary Biliary Cirrhosis (Cholangitis)

Epidemiology
Sx

A

E: Northern Europeans

40-60 yo; females»»

Sx: Insidious onset w/ pruritus, fatigue, jaundice, associated autoimmune disorders

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14
Q

Primary Biliary Cirrhosis (Cholangitis)

Labs

A

AMA+ (serum antimitochondrial antibodies)

50% ANA+
40% ANCA+

elevation of GGT and/or alkaline phosphatase out of proportion to transaminases

Elevated bilirubin, elevated IgM

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15
Q

Primary Biliary Cirrhosis (Cholangitis)

Micro

A

Florid duct lesion –> destructive cholangitis

+Portal-based nodular inflammation composed of lymphocytes, plasma cells, eosinophils, macrophages
-Bile duct injury
-Lymphocytic cholangitis –> granulomas variable

Chronic Chole: swollen/rarefied hepatocytes adjacent to portal tracts (chole stasis)

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16
Q

Primary Biliary Cirrhosis (Cholangitis)

Gross

A

Cirrhosis is a biliary type w/ irregular nodules (jigsaw puzzle)

Nodular liver
Regenerating nodules
Bile staining in fibrotic planes btw regenerating nodules

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17
Q

Primary sclerosing cholangitis

A

Fibroinflammatory disorder that affects intrahepatic & extrahepatic biliary tree –> biliary stricture & cirrhosis

HLAB8 & DR2
Associated dx: IBD (UC)

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18
Q

Primary sclerosing cholangitis

Epidemiology
Etiology

A

Any age; most common in 2nd decade

Male&raquo_space;»

Autoimmune (ANCA+)
OR
Nonimmune –> no associated antibodies

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19
Q

Primary sclerosing cholangitis

Sx

A

Fatigue
Abdominal pain
Pruritus
Jaundice
Diarrhea
Anorexia
WT Loss
Fever
Hepatomegaly
Splenomegaly asymptomatic

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20
Q

Primary sclerosing cholangitis

Lab tests

A

+ Increase serum GGT
+ Increase serum aminotransferase levels
+ Elevated ANCA, ANA
+Unreliable serum alkaline phosphatase activity (difficult to interpret bc of bone growth, normal in up to 50% of children /w PSC)

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21
Q

Primary sclerosing cholangitis

Imaging

A

Cholangiography
+Beaded appearance of intrahepatic & extrahepatic biliary tree – segmental strictures & saccular dilations of affected bile ducts

22
Q

Primary sclerosing cholangitis

Histopathology

A

+ Concentric fibrosis around affected bile ducts –> “Onion skin”

+ Fibro-obliterative lesion

+Degeneration & atrophy of duct epithelium

+Lymphocytic infiltration of bile ducts

23
Q

Primary sclerosing cholangitis

Gross

A

Biliary cirrhosis ++
Highly irregular, jigsaw puzzle nodular pattern

24
Q

Primary sclerosing cholangitis

Risk of ..

A

Cholangiosarcoma

+Frequent overlapping w/ autoimmune hepatitis (autoimmune sclerosing cholangitis)

+Recurring in 10 of pts after liver tx

25
Q

Primary hepatolithiasis

A

Intrahepatic gallstone formation

East Asia –> “Oriental Cholangitis”

Repeated bouts of ascending cholangitis (recurrent pyogenic cholangitis)

Progressive inflammatory destruction of hepatic parenchyma

Risk of biliary neoplasia (cholangiocarcinoma)

26
Q

Gallstones

A

Concentrations w/in the biliary system

27
Q

Choledocholithiasis

A

Stones w.in common bile duct

28
Q

Gallstones: 2 types

A

Cholesterol stones
Bilirubin stones

29
Q

Cholesterol stones

A

+Yellow (stones in developed nations)

+Bile supersaturation of cholesterol, decreased phospholipids (lecithin) & stasis or hypomotility of gallbladder

+Formation of cholesterol crystals, which grow & aggregate w/ mucin proteins –> STONES

30
Q

Bilirubin stones

A

Black or brown stones

31
Q

Cholesterol stones

Risk factors

A

Age, obesity, familial predisposition, estrogen replacement therapy/OCPs, hypertriglyceridemia

Latin American women
Native Americans

32
Q

Black Stones

A

+Bilirubin

Contain calcium bilirubinate
- 2-5 mm, shiny, irregular, multifaceted

Risk factors: older/undernourished, chronic hemolysis, biliary infection (E. coli, Ascaris lumbricoides, Clonorchis sinensis), total parenteral nutrition (TPN)

33
Q

Brown Stones

A

+Bilirubin

Calcium salts of bilirubin & palmitate, MORE cholesterol than black stones

Softer texture & flaky appearance, often larger than black

Strongly assoc w/ ascending cholangitis & biliary inflammation, ESP. 2/2 E. coli & flukes

34
Q

Gallstones

Gross

A

+Smooth, mixed cholesterol stones

+ Pure cholesterol stones

+Lobulated cholesterol stones

35
Q

Gallstones

Presentation

A

+Can be clinically silent

+ RUQ pain, flatulence, intolerance of fatty foods

Complications: acute & chronic cholecystitis, choledocholithiasis, fistulas, acute pancreatitis, gallbladder cancer

36
Q

Gallstones

Radiographic

A

Many radiolucent (many cholesterol)

Black may be radiopaque

US: modality of choice –> can detect if >3 mm diameter

37
Q

Acute cholecystitis

A

Obstruction of cystic duct by stones & bile supersaturated with cholesterol

38
Q

Acute cholecystitis

Trauma to mucosa releases phospholipase from lysosomes

A

Phospholipase converts lecithin in bile –> lysolecithin –> damages gallbladder epithelium

39
Q

Acute cholecystitis

Secondary bacterial infection

A

with enteric organisms

40
Q

Acute cholecystitis

Overgrowth by gas-producing organism

A

Emphysematous cholecystitis

41
Q

Acute acalculous cholecystitis (5%)

Risk factors
Symptoms

A

Risk: critical illness, burns, trauma, major surgical procedures, diabetes, immunosuppression, biliary sludge formation, mucosal ischemia

Sx: RUQ pain, tenderness, guarding
+Murphy sign –> inspiratory arrest
+Fever, leukocytosis, elevated WBC
+ Gangrenous cholecystitis
+Perforation
+Emphysematous cholecystitis –> caused by overgrowth of organisms

42
Q

Gallbladder Carcinoma

A

Adenocarcinoma arising from glandular epithelium that lines the gallbladder wall

+Most common malignancy of extrahepatic biliary tract

Two patterns of tumor growth: infiltrating & exophytic

43
Q

Gallbladder Carcinoma

Risk factors
Sx

A

Risk: gallstones, esp when complicated by porcelain gallbaldder

sx: cholecystitis in elderly women
Abd pain
Jaundice
Anorexia
N/V

POOR Prognosis

Location: Asia – chronic bacterial/parasitic infections that have been implicated as risk fx

44
Q

Chronic cholecystitis

A

Chronic inflammation of gallbladder –> wall thickening & fibrosis

Almost always assoc w/ gallstones

45
Q

Chronic cholecystitis

Etiology

A

Almost always assoc w/ gallstones
Cholesterol stones → supersaturation of bile w/ cholesterol
d/t chemical irritation from longstanding cholelithiasis w/ or w/out superimposed bouts of acute cholecystitis

46
Q

Chronic cholecystitis

Sx

A

Episodic, steady, abdominal pain
+ Usually RUG
+ May be precipitated by ingestion of food

Microscopy: Rokitansky-Aschoff sinuses

47
Q

Chronic cholecystitis

Complications

A

Porcelain gallbladder → dystrophic calcification

Risk of adenocarcinoma

Acute cholecystitis, acute pancreatitis, choledocholithiasis, GB cancer or cholangiocarcinoma

48
Q

Chronic cholecystitis

Rare Complications

A

Biliary enteric fistula

Gallstone ileus: migration of stone into bowel through fistula w/ obstruction of bowel at distal ileum

Bouveret syndrome: gastric outlet obstruction d/t stone in duodenum or distal stomach

Dropped gallstones: gallstones spilled into abdomen during laparoscopic cholecystectomy

49
Q

Gallstone ileus

A

Migration of stone into bowel through fistula w/ obstruction of bowel at distal ileum

50
Q

Bouveret syndrome

A

Gastric outlet obstruction d/t stone in duodenum or distal stomach

51
Q

Dropped gallstones

A

Gallstones spilled into abdomen during laparoscopic cholecystectomy

GEMS (20 decks)

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