Specific Causes of Cirrhosis Flashcards
Alcoholic Liver Disease The most accurate
test
liver biopsy.
Alcoholic Liver Disease Labs
greater elevation in AST
compared to ALT
drinking
gives a sudden rise in GGTP.
Primary Biliary Cholangitis (PBC) CxFx
“most likely diagnosis”=
“most likely diagnosis”= Woman in 40s or 50s Fatigue and itching Jaundice hepato-splenomegaly Normal bilirubin with an elevated alkaline phosphatase
Primary Biliary Cholangitis (PBC) Most unique features
Xanthelasma/xanthoma
Osteoporosis
Primary Biliary Cholangitis (PBC) most accurate test.
liver biopsy
Primary Biliary Cholangitis (PBC) The most accurate blood test
antimitochondrial antibody.
N.B: Bilirubin and IgM levels do not elevate until the
disease is very far advanced.
Primary Biliary Cholangitis (PBC) tx
ursodeoxycholic acid OR
obeticholic acid that decreases fibrosis.
Primary Sclerosing Cholangitis asoc.!
80% of them w inflammatory bowel disease.
Primary Sclerosing Cholangitis Cx Fx
Signs of cholestasis: like Jaundice and Pruritus
Symptoms of chronic IBD
Primary Sclerosing Cholangitis lab test show:
alkaline phosphatase
GGTP
bilirubin level
Elevated alkaline phosphatase and GGTP as well as elevated bilirubin level
Primary Sclerosing Cholangitis most accurate test
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
Primary Sclerosing Cholangitis tx
cholestyramine or ursodeoxycholic acid, the same as PBC
THO if there is IBD, it wont improve EVEN after colectomy. Transplant when needed
Alpha 1-Antitrypsin Deficiency CxFx
liver disease + emphysema (COPD)+ young
patient <40) and NONsmoker
Alpha 1-Antitrypsin Deficiency Hx
family history of
COPD at an early age.
Alpha 1-Antitrypsin Deficiency tx
replacing the enzyme. tho onlmed says u can do transplant
Hemochromatosis Patho
genetic disorder leading to overabsorption of iron in the duodenum.
The mutation is the C282y gene.
Hemochromatosis epidem
Men present earlier than women because menstruation delays the onset of liver
fibrosis and cirrhosis.
Look for a patient in his 50s
Hemochromatosis lab tests
mildly
abnormal liver function tests (LFTs), alkaline
phosphatase,
or iron levels.
Hemochromatosis Cx Fx
Fatigue and joint pain (pseudogout) Erectile dysfunction in men, and amenorrhea in women (from pituitary involvement) Skin darkening Diabetes Cardiomyopathy
Hemochromatosis asoc. infections
Vibrio vulnificus, (it is killed by normal human blood but grows rapidly in blood from patients with hemochromatosis)
Yersinia, and Listeria
Hemochromatosis best initial test
iron studies that show:
Increased serum iron and ferritin
Decreased iron binding capacity
Hemochromatosis most accurate test
liver biopsy for increased iron BUT
MRI and HFE (C282y) gene testing can be done instead!!!!!
Hemochromatosis Cardio tests
EKG may show
conduction defects and the echocardiogram can show dilated or restrictive
cardiomyopathy.
Hemochromatosis Treatment
Phlebotomy is clearly the best therapy for those with overabsorption of iron. this can prevent cirrhosis!
transferrin saturation= (formula)
(iron divided by TIBC)
Iron chelation therapy is used in hemochromatosis for those who:
1.- Cannot be managed with phlebotomy
2.- Are anemic and have hemochromatosis from overtransfusion such
as thalassemia
Iron chelation agentes
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
Alpha 1-Antitrypsin Deficiency Dx
Serum: decreased antitrypsin protein levels
Bx shows (Periodic acid–Schiff stain) PAS + macrophages might be definitive
PSC sclerosis colangitis is asoc w
Ucerative colitis or IBD in general
PSC sclerosis colangitis epidem and CxFx n patho
man 30-50y. (sons of bitches)
pruritus + jaundice
extrahepatic ducts
PSC sclerosis colangitis first test and BX
MRCP:shows beads on a strain
bx shows onion skin fibrosis
PSC sclerosis colangitis tx
NO STENT
transplant
ursodeoxicholic? acid while waiting for transplant
PBC epidemiology and location of the lession
women .
intrahepatic ducts
