Exam 2 LIVER DZ

Question 1

Non-EtOH Fatty Liver Dz (NAFLD)?

Answer 1

Inflamed, fatty liver w/o excess EtOH consumption

Question 2

NAFLD risk factors? (5)

Answer 2

Obesiety
Hypertrigly
DM
Meds (steroids, E2, tamoxifen, amiodarone, HIV)
Metabolic syndromes

Question 3

NAFLD presentation? (3)

Answer 3

U asymp
Hepatomegaly
Mild ↑ AST/ALT (ratio <1)

Question 4

NAFLD diagnostics? (3)

Answer 4

r/o EtOH and other liver dz

US = hyperchoic texture

Bx = diagnostic
Use when unclear, suspect cirrh, >45yo obese DM

Question 5

NAFLD tx? (5)

Answer 5

Weight loss!
No EtOH
Monitor LFT
Control DM/hyperlip
Vaccine for Hep A/B

Question 6

Hereditary Hemochromatosis is?

Answer 6

Auto-recessive
↑ GI absorb of Fe ->
accumulation of Fe in liver, other organs

Question 7

Hereditary Hemochromatosis presentation? (6)

Answer 7

Aysmpt until 30-40s
Fatigue/Malaise/Weak (early)
Hepatomegaly/Insuff/Cirrhosis
Cardiomegaly w/ or w/o CHF
ED, arthropathy

BRONZE DM:
DM triad
Bronze skin
Cirrhosis

Question 8

Hereditary Hemochromatosis screening?

Answer 8

If ↑AST/ALT, liver dz, suggestive sxs of HH,
And all 1st degree relatives

Tests: Serum Fe, Transferrin Sat, TIBC, Ferritin

Question 9

Hereditary Hemochromatosis genetic testing if?

Answer 9

Transferrin Sat > 45,

Ferritin >200 in M or 150 in F

Question 10

Hereditary Hemochromatosis diagnosis?

Answer 10

Genetic test

+/- liver bx for extent of dz

Question 11

Hereditary Hemochromatosis tx? (3)

Answer 11

Prevent cirr and end-organ damage:
Avoid Fe and Vit C
Avoid EtOH
Phlebotomy

Question 12

Autoimmune Hepatitis is?

Answer 12

Hepatocellular inflamm 2° to immune rxn

U females w/ autoimm dz

Question 13

Autoimmune Hepatitis presentation?

Answer 13

Variable
Asympt w/ ↑ LFT
Cirrhosis
Acute Hep
Vague sxs

Question 14

Autoimmune Hepatitis labs? (3)

Studies?

Answer 14

ANA = +
ASMA = +
IgG = ↑

Bx for confirm and extent

Question 15

Autoimmune Hepatitis tx? (3)

Answer 15

By GI physician:
Prednisone +/- Azothioprine
Hep A/B vacc
P transplant

Question 16

α-1 Antitrypsin Deficiency is?

Answer 16

Genetic

Low α-1 antitrypsin (protects liver and lung tissue from injury)

Question 17

α-1 Antitrypsin Deficiency presentation?

Answer 17

Severe U kids

Accelerated lung fxn ↓

Question 18

α-1 Antitrypsin Deficiency screening when? (4)

Answer 18

All w/ Hx unexplained chronic liver dz or clinical findings

Emphysema if:
<45 yo
Non-smoker
Basilar ∆s on CXR

FHx emphys, liver dz

Hx panniculitis (inflamm of adipose)

Question 19

α-1 Antitrypsin Deficiency diagnostics? (2)

Answer 19

Serum α-1 antitrypsin = ↓

α-1 antitrypsin phenotype = Z (M is normal)

Question 20

α-1 Antitrypsin Deficiency tx? (3)

Answer 20

Liver bx
Manage complications
Transplant

Question 21

Wilson’s Dz is?

Results in?

Answer 21

Auto-recessive
↑ copper accumulation/↓ copper excretion

Toxic levels in Liver, Brain
cornea, kidney, heart, joints, pancreas

Question 22

Wilson’s Dz presentation?

Answer 22

U 5-35yo
Liver dz
Neuro sxs (tremor, ↓speech, ↓coord, behavior ∆s)
Psych
KAYSER-FLEISCHER RING

Question 23

Wilson’s Dz labs?

Answer 23

LFT = ↑
Serum ceruloplasmin = ↓
24hr urine copper

Question 24

Wilson’s Dz studies?

Answer 24

Ophthal for slit lamp
Liver bx for [copper]
Screen family

Question 25

Wilson’s Dz tx? (3)

Answer 25

Copper restriction (shellfish, nuts, mushrooms, chocolate)
Chelation (penicillamine)
Transplant

Question 26

Wilson’s Dz prognosis?

Answer 26

Good if prior to brain damage