Chronic Liver Disease

Question 1

Alcoholism Genetics

Answer 1

• Tumor Necrosis Factor

- ADH (Asians)

Question 2

Disulfuram

Answer 2

Blocks ADH enzyme to cause reaction; usd in alcoholics

Question 3

Alcohol induced Fatty liver Presentation

• LabDx
• PE

Answer 3

• Asymptomatic
• PE: Hepatomegaly; non-sepcific complaints
• Lab: Mild INC LFTs; AST>ALT
• Prog: Reversible

Question 4

DF

Answer 4

Discriminant Function: Used in Alcoholic Hepatitis

• 4.6(PT-PT Control) + Tbili
• DF > 32 -> >50% Mortality; Tx Steroids

Question 5

PBC (PRimary Biliary Cirrhosis)

• EPI
• Path
• S/S
• LabDx
• Tx

Answer 5

• EPI: Women 30-65 YO
• Path: TLC mediated attack on bile ducts
• S/S: Hyperpigmentation, Xanthelasma, Hepatosplenomegaly, fatigue, pruritis
• LabDx: Anti-mitochondrial Abs (AMA); Striking ALP Elevation; “Onion Skin” Histo
• Tx: Ursodeoxycholic acid + Liver Transplant

Question 6

HH (Dereditary Hemochromatosis)

• Genetics
• S/S
• LabDX
• Tx
• A/S

Answer 6

• G: AR - Chromosome 6;
• Path: INC in Fe absorption over decades
• S/S: Cirrhosis (Hepatocellular Carcinoma!!!), Bronzing of skin, Diabetes, arthralgia, impotence, Restrictive CM
• Lab Dx: Nonspecific LFTs Check Transferrin Saturation
• Tx: Phlebotomy

Question 7

Wilson’s Disease

• Genetics
• EPI
• Path
• S/S
• Lab DX
• Tx

Answer 7

• G: AR Chromosome 13; >100 mutations
  EPI: Median age 12-23 YO presentation;
• Path: DEC Cu secretion due to DEC ceruloplasmin
• S/S: Hepatic (Portal HTN to failure); Neuro/Psych (PD, Depression); Hemolysis
• LabDx: Copper Bands at inf/sup poles of eye (Kayser-Fleischer Rings); Low Ceruloplasminl; High Urine Cu
• TX: Penicillamine or Trientine; Zn; Transplant

Question 8

Two “Presentations” of WD?

Answer 8

1. Young person presenting with severe liver dysfunction and hemolytic anemia
2. Young person presenting with unexplained parkinsonism

Question 9

Primary Sclerosing Cholangitis:

• EPI
• LabDx
• A/S

Answer 9

• EPI: PRedominantly Men (“Sweetness”
• LabDx; ERCP
• A/S: a-1 antitrypsin

Question 10

Autoimmune Hepatitis (AIH)

• S/S:
• LabDx
• Tx

Answer 10

• S/S: Acute Liver Failure
• LabDx: ASMA; AK/LMA; ANA
• Tx: Steroids

Question 11

A 45 y.o. obese, diabetic woman with htncomes into your office for a check up and is found to have abnormal lfts.
She does not drink alcohol
She is on no hepatotoxic drugs
Her father died of cirrhosis but she was told he was an alcoholic
PE: BMI = 40, hepatomegaly
- Neg ANA
-

A.  Primary biliary Cirrhosis
B.  Non alcoholic fatty liver disease (NAFLD
)C.  Hereditary Hemochromatosis
D.  Hepatitis C
E.  Occult alcoholism

Answer 11

B. Non alcoholic fatty liver disease (NAFLD