Mehlman PBC/PSC, gallstone ileus/bile duct leak

Question 1

Primary biliary cirrhosis. what case?

Answer 1

woman 20s-50s who has generalized
pruritis, ­incr. cholesterol, ­ incr. ALP, ­incr. direct bilirubin, and Hx of autoimmune
disease in her or a relative.

Question 2

Primary biliary cirrhosis - probably will mention other autoimmune diaseases because they all go together.
eg DM1, RA, SLE

Answer 2

.

Question 3

Primary biliary cirrhosis. first step diagnosis?

Answer 3

Diagnose with anti-mitochondrial antibodies as first step.

Question 4

Primary biliary cirrhosis. second step diagnosis?

Answer 4

liver biopsy to confirm.

Question 5

Primary biliary cirrhosis. initial Tx?

Answer 5

ursodeoxycholic acid (ursodiol)

Question 6

Primary biliary cirrhosis.
They can mention a stone is present in the gallbladder on ultrasound, which
gets some students real emotional / confused, but it makes sense since patient
has ­ cholesterol. It’s just a distractor point and doesn’t relate to the actual diagnosis at hand.

Answer 6

.

Question 7

Primary biliary cirrhosis.
- New NBME material asks a couple Qs on fat-soluble vitamin malabsorption for
PBC (deficiency of A and D on NBME for each Q, respectively, with no info
supporting those presentations; they just ask for hypothetical vitamin
deficiency). In theory, would be due to biliary obstruction, where decr. bile entering small bowel merely means decr. fat absorption. This is nothing special to PBC, but I mention it because it’s asked twice.

Answer 7

.

Question 8

Primary biliary cirrhosis. CP?

Answer 8

pruritus, fatigue, XANTOMAS, hepatomegaly

Question 9

Primary biliary cirrhosis. Tx for advanced disease?

Answer 9

liver transplant

Question 10

Bile duct leak. 2CK Surg

They give you patient with cholecystectomy
within the past week who now has fever, abdominal pain, and ­ direct bilirubin.

Answer 10

.

Question 11

Bile duct leak. 2CK Surg.

Answer 11

During the surgical anastomoses created during cholecystectomy, sometimes there can be a post-op bile leak, literally. Weird diagnosis but it shows up on occasion.

Question 12

gallstone ileus. when suspect?

Answer 12

if they mention “air in the biliary tree” (pneumobilia).

Question 13

gallstone ileus. Can be caused by cholecystoduodenal fistula, which is when a patient with cholelithiasis can develop a conduit between the gallbladder and small bowel.

Answer 13

USMLE gives long viggnete + penumobilia = so answer is “gallstone ileus” straight up, or it will be “cholecystoduodenal fistula.”

Question 14

gallstone ileus. pneumobilia on Xray.

+ cannot pass stools, hyperactive bowel sounds, abdominal distension.

Answer 14

CT scan - confirmativeL pneumobilia, obstructing stone, gallbladder wall thickening.

Question 15

Primary sclerosing cholangitis (PSC).
Mehlman case?

Answer 15

UC + high bilirubin + high ALP –> primary sclerosing cholangitis.

Question 16

Primary Sclerosing Cholangitis. UW table. clinical?

Answer 16

fatigue, pruritus; majority asymptomatic
ABOUT 90 PROC WILL HAVE UNDERLYGIN IBD!!!!!!! mainly UC

Question 17

Primary Sclerosing Cholangitis. UW table. labs?

Answer 17

Aminotranslerases normal (<300)
Main finding - cholestatic liver enzymes increased

Question 18

Primary Sclerosing Cholangitis. UW table. imaging?

Answer 18

multifocal sticturing/dilation of intrahepatic and/or extrahepatic bile ducts on cholangiography

Question 19

Primary Sclerosing Cholangitis. UW table. biopsy?

Answer 19

fibrous obliteration of bile ducts with concentric replacement by connective tissue in an ,,onion-skin” pattern.

Question 20

Primary Sclerosing Cholangitis. UW case?

Answer 20

middle age man with fatigue, pruritus, UC and elevated AP + pANCA