Lecture 6 - Liver Misc/Cancer

Question 1

Aka primary biliary cholangitis

Autoimmune destruction of small intrahepatic bile ducts and cholestasis

Answer 1

Primary biliary cirrhosis

destruction of bile duct epithelium -> loss of intralobular ducts -> cholestasis -> cirrhosis/live failure

Question 2

Usually women (diagnosed in 40s/50s)

Insidious onset

Associated w/ other autoimmune disorders

Answer 2

Primary biliary cirrhosis

Question 3

Many asymptomatic at diagnosis (detected by LFT abnormalities)

Vague, non-specific ssx (fatigue, pruritus, XANTHAMATUOS lesions)

In later stages, you might see jaundice, steatorrhea, portal HTN

Answer 3

Primary biliary cirrhosis

Question 4

Lab findings for primary biliary cirrhosis show a cholestatic pattern… meaning?

What might you see on specialized lab testing?

Answer 4

Cholestatic pattern = increased ALK PHOS (ALP)
(slightly elevated bilirubin, maybe increase transaminases)

Specialized lab testing = ANTIMITOCHONDRIAL ANTIBODIES (AMAs) and ANAs

Question 5

Treament for primary biliary cirrhosis?

Answer 5

First r/o other etiologies (carcinoma, cholangitis)

URSODEOXYCHOLIC ACID

(symptomatic tx for pruritis = bile salt sequestrant)

Question 6

Autosomal recessive disorder

Results in accumulation of iron as hemosiderin in the liver, pancreas, heart, adrenals, testes, pituitary, and kidneys

What is this and what might it lead to?

Answer 6

Hemochromatosis

may lead to cirrhosis and/or hepatic failure

Question 7

Typical onset after age 50

Diagnosis usually made earlier (found incidentally due w/ elevations in AST and ALK PHOS and plasma IRON, serum ferritin)

Consider genetic testing in patients w/ iron overload/fam hx

Answer 7

hemochromatosis

Question 8

Treatmetn options for hemochromatosis

Answer 8

Chelation w/ deferoxamine

phlebotomy

(liver transplant if advanced)

Question 9

RARE autosomal recessive disorder (usually in people under 40)

Excessive absorption of Cu in the small intestine coupled w/ decreased hepatic excretion

What disease… and results in what?

Answer 9

Wilson’s disease

results in deposition of copper in the cornea, liver, brain

Question 10

Highly variable but may present w/:

liver disease
neuro ssx
psycho ssx

pathognomonic sign = Kayser Fleischer rings…

Answer 10

Wilson Dz

Kayser Fleischer ring = brownish/greenish ring at the corneo-scleral junction

Question 11

What kinda labs willl you see w/ Wilson disease?

Treatment?

Answer 11

DECREASED serum ceruloplasmin

increased Cu in urine and liver (via biopsy)

Tx = chelation

Question 12

Hepatitis Venous Outflow Tract Obstruction

Resulting in post-hepatic portal HTN

Primary vs secondary?

Answer 12

Budd Chiari syndrome

Primary = obstruction due to a predominantly venous process (thrombosis/phlebitis)

Secondary = Compression/invasion of the hepatic veins and/or inferior vena cava by lesion that originates outside of the vein

Question 13

Budd Chiari presents w/

Tender/painful hepatomegaly
Jaundice
Splenomegaly
Ascites

Radiographic test of choice/

Answer 13

Color Doppler US

Question 14

Tx for Budd Chiari?

Answer 14

Tx underlying cause…

ADMIT ANY PATIENT W/ SUSPECTED HEPATIC VEIN OBSTRUCTION

Question 15

Pyogenic hepatic abscess… hepatic invasion through…

• bile duct (ASCENDING CHOLANGITIS)
• portal vein (pylephlebitis)
• hepatic artery (bacteremia)
• direct extension from an infectious process
• traumatic implantation of bacteria through the abdominal wall

Answer 15

got it

Question 16

FEVER
RUQ pn
JAUNDICE
nausea, vomiting
anorexia, wt loss, malaise

Labs show leukocytosis, pos blood cultures, nonspecific abnormalities on hepatic panel

Answer 16

pyogenic hepatic abscess

fever, jaundice, leukocytosis

Question 17

Pyogenic hepatic abscess diagnostic imaging?

Answer 17

CT scan (CT guided percutaneous drainage)

hepatic US is an option too, but may not be deep enough… use CT

Question 18

Benign liver neoplasm… no associated ssx…. usually an incidental finding on CT or US

Answer 18

cavernous hemangioma

Question 19

Risk factors for hepatocellular carcinoma….

Answer 19

Cirrhosis in 80% of cases

Hep B/C infxn
NAFLD
Tob/ETOH use
Diabetes
OBesity
Hemochromatosis/Wilson's

Question 20

Ssx of hepatocellular carcinoma… mostly related to underlying chronic liver disease

Answer 20

Fever, chills, wt loss

bone pain from metastases

Question 21

SUDDEN AND SUSTAINED elevation of ALK PHOS

leukocytosis on CBC
increase in AFP

Answer 21

Hepatocellular carcinoma

imaging = CT or MRI (will not be demarcated like an abscess)

Biospy for histologic diagnosis

Question 22

Hepatocellular carcinoma tx?

Answer 22

surgical resection or hepatic transplant

poor prognosis

Question 23

Screening for hepatocellular carcinoma rec’d in patients:

with cirrhosis
with chronic HBV/HCV
fam hx of HCC

How often and by what means/

Answer 23

Hepatic US and alpha fetoprotein (AFP) levels

every 6 months

Question 24

Essentials of Dx:

Middle aged women
Often asymptomatic
Increased ALP
\+ anti mitochondrial AB’s
Increased IgM
Increased cholesterol
Characteristic livery biopsy
Later stages presentation: fatigue, jaundice, features of cirrhosis, xanthelasma, xanthoma, and steatorrhea

Answer 24

Primary Biliary Cholangitis