Specific Causes of Cirrhosis

Question 1

Alcoholic Liver Disease The most accurate

test

Answer 1

liver biopsy.

Question 2

Alcoholic Liver Disease Labs

Answer 2

greater elevation in AST
compared to ALT
drinking
gives a sudden rise in GGTP.

Question 3

Primary Biliary Cholangitis (PBC) CxFx

“most likely diagnosis”=

Answer 3

“most likely diagnosis”=
Woman in 40s or 50s
Fatigue and itching
Jaundice
hepato-splenomegaly
Normal bilirubin with an elevated alkaline phosphatase

Question 4

Primary Biliary Cholangitis (PBC) Most unique features

Answer 4

Xanthelasma/xanthoma

Osteoporosis

Question 5

Primary Biliary Cholangitis (PBC) most accurate test.

Answer 5

liver biopsy

Question 6

Primary Biliary Cholangitis (PBC) The most accurate blood test

Answer 6

antimitochondrial antibody.

N.B: Bilirubin and IgM levels do not elevate until the
disease is very far advanced.

Question 7

Primary Biliary Cholangitis (PBC) tx

Answer 7

ursodeoxycholic acid OR

obeticholic acid that decreases fibrosis.

Question 8

Primary Sclerosing Cholangitis asoc.!

Answer 8

80% of them w inflammatory bowel disease.

Question 9

Primary Sclerosing Cholangitis Cx Fx

Answer 9

Signs of cholestasis: like Jaundice and Pruritus

Symptoms of chronic IBD

Question 10

Primary Sclerosing Cholangitis lab test show:
alkaline phosphatase
GGTP
bilirubin level

Answer 10

Elevated alkaline phosphatase and GGTP as well as elevated bilirubin level

Question 11

Primary Sclerosing Cholangitis most accurate test

Answer 11

MRCP or ERCP that shows **beading, narrowing, or **strictures in the biliary system

Liver biopsy is:
Not an essential part of the workup; usually done if small duct PSC is suspected

Question 12

Primary Sclerosing Cholangitis tx

Answer 12

cholestyramine or ursodeoxycholic acid, the same as PBC

THO if there is IBD, it wont improve EVEN after colectomy. Transplant when needed

Question 13

Alpha 1-Antitrypsin Deficiency CxFx

Answer 13

liver disease + emphysema (COPD)+ young

patient <40) and NONsmoker

Question 14

Alpha 1-Antitrypsin Deficiency Hx

Answer 14

family history of

COPD at an early age.

Question 15

Alpha 1-Antitrypsin Deficiency tx

Answer 15

replacing the enzyme. tho onlmed says u can do transplant

Question 16

Hemochromatosis Patho

Answer 16

genetic disorder leading to overabsorption of iron in the duodenum.
The mutation is the C282y gene.

Question 17

Hemochromatosis epidem

Answer 17

Men present earlier than women because menstruation delays the onset of liver
fibrosis and cirrhosis.

Look for a patient in his 50s

Question 18

Hemochromatosis lab tests

Answer 18

mildly
abnormal liver function tests (LFTs), alkaline
phosphatase,
or iron levels.

Question 19

Hemochromatosis Cx Fx

Answer 19

Fatigue and joint pain (pseudogout)
Erectile dysfunction in men, and amenorrhea in women (from pituitary
involvement)
Skin darkening
Diabetes
Cardiomyopathy

Question 20

Hemochromatosis asoc. infections

Answer 20

Vibrio vulnificus, (it is killed by normal human blood but grows rapidly in blood from patients with hemochromatosis)

Yersinia, and Listeria

Question 21

Hemochromatosis best initial test

Answer 21

iron studies that show:

Increased serum iron and ferritin
Decreased iron binding capacity

Question 22

Hemochromatosis most accurate test

Answer 22

liver biopsy for increased iron BUT

MRI and HFE (C282y) gene testing can be done instead!!!!!

Question 23

Hemochromatosis Cardio tests

Answer 23

EKG may show
conduction defects and the echocardiogram can show dilated or restrictive
cardiomyopathy.

Question 24

Hemochromatosis Treatment

Answer 24

Phlebotomy is clearly the best therapy for those with overabsorption of iron. this can prevent cirrhosis!

Question 25

transferrin saturation= (formula)

Answer 25

(iron divided by TIBC)

Question 26

Iron chelation therapy is used in hemochromatosis for those who:

Answer 26

1.- Cannot be managed with phlebotomy
2.- Are anemic and have hemochromatosis from overtransfusion such
as thalassemia

Question 27

Iron chelation agentes

Answer 27

Deferasirox and deferiprone PO, acutelly
Deferoxamine has to be given lifelong by injection.

DElete the IRON si? ROXanne
Delete the Iron PROffesional
DElete the IRON of MINE

Question 28

Alpha 1-Antitrypsin Deficiency Dx

Answer 28

Serum: decreased antitrypsin protein levels

Bx shows (Periodic acid–Schiff stain) PAS + macrophages
might be definitive

Question 29

PSC sclerosis colangitis is asoc w

Answer 29

Ucerative colitis or IBD in general

Question 30

PSC sclerosis colangitis epidem and CxFx n patho

Answer 30

man 30-50y. (sons of bitches)
pruritus + jaundice
extrahepatic ducts

Question 31

PSC sclerosis colangitis first test and BX

Answer 31

MRCP:shows beads on a strain

bx shows onion skin fibrosis

Question 32

PSC sclerosis colangitis tx

Answer 32

NO STENT
transplant
ursodeoxicholic? acid while waiting for transplant

Question 33

PBC epidemiology and location of the lession

Answer 33

women .

intrahepatic ducts