Midgut

Question 1

Monitoring test in fulminant hepatic failure

Answer 1

PT/INR the best lab to monitor status (up to four times per day)

• Extensive and complete lab evaluation
• Doppler ultrasonography of the liver

Question 2

Transplant criteria for fulminant hepatic failure

Answer 2

Acetaminophen induced liver failure
* Arterial pH <7.30
OR
* Grade 3 or 4 encephalopathy with PT>100seconds and Cr>340 mg/L

Non-acetaminophen induced liver failure
* PT>100 seconds
OR
* Any three of :
* Age <10 or >40 years
* Non-A and non-B viral hepatitis, idiosyncratic drug reaction, Wilson
* Jaundice >7 days prior to encephalopathy
* PT>50 seconds
* Bilirubin >180 mg/L

Question 3

Reservoir for Hep A

Answer 3

only in humans

Question 4

hep A % -> fulminant liver failure?

Answer 4

< 1% (usually if already have liver disease)

Question 5

hep B % -> fulminant liver failure?

Answer 5

0.1-0.5% -due to massive immune-mediated lysis of infected hepatocytes (often DNA negative)

Question 6

HBV extra-hepatic manifestations

Answer 6

in 20%
* Polyarteritis nodosa
* Glomerular disease (membranous nephropathy, MPGN, nephrotic syndrome)
* Serum sickness (arthritis, rash)

Question 7

HBV treatment criteria

Answer 7

• Anyone with cirrhosis
• Immune active chronic HBV

Maybe (guidelines changing)
* HBV DNA >2000 IU/ml (HBeAg neg) with ALT 2x ULN
* HBV DNA >20,000 IU/ml (HBeAg pos) with ALT 2x ULN

Question 8

HBV that doesn’t need treatment

Answer 8

• Immune tolerant phase (HBeAg positive, High DNA, normal transaminases)
• Inactive carrier phase (low or no DNA, normal transaminases)
• Latent HBV infection (DNA without HBsAg)

Question 9

Which HBV treatment safe if preggers?

Answer 9

Lamivudine

Question 10

HCV viral halflife?

Answer 10

~ 45 mins

Question 11

Percent of acute HCV -> Chronic -> Cirrhosis

Answer 11

60-85% to chronic
20-30% with chronic to cirrhosis (over 20-30 years)

Question 12

Extra-hep manifestations HCV

Answer 12

Cognitive impairment independent of liver disease stage

Lichen planus
* 19% with lichen planus will have HCV
* Six fold rise in risk for HCV in persons with lichen planus
* a T-cell mediated autoimmune disorder in which inflammatory cells attack an unknown protein within the skin and mucosal keratinocytes.

Essential Mixed Cryoglobulinemia
- Half of persons with HCV will have cryoglobulins
- Leukocytoclastic vasculitis
- Arthralgias
- Membranoproliferative glomerulonephritis
- Neurologic disease, peripheral neuropathy

Porphyria Cutanea Tarda
(a rare disorder characterized by painful, blistering skin lesions that develop on sun-exposed skin (photosensitivity))

Question 13

Answer 13

Porphyria Cutanea Tarda
* Decreased activity of uroporphyrinogen decarboxylase
* May be inherited or acquired
* Sun exposed skin changes
* Elevated urine uroporphyrin levels
* 50% of those with PCT have HCV

Question 14

HEV transmisison

Answer 14

• RNA virus transmitted in water/feces
• Highest incidence in Asia, Africa, Middle East, and Central America
• Animal reservoir (rodents, deer, boar)
• Acute disease in nonimmunocompromized patients
• Chronic disease in those with transplants

Question 15

Fulminant HEV?

Answer 15

• Fulminant hepatic failure in 15-25 % in women who are pregnant

Question 16

Most common meds -> DILD?

Answer 16

• Paracetamol / acetaminophen most common
• Amoxacillin/clavulanate most common antibiotic

Question 17

Treatment for DILD

Answer 17

Drug withdrawal

Specific therapies
* N-acetylcysteine for paracetamol
* L-carnitine for valproic acid overdose

Glucocorticoids - may have a role in hypersensitivity reactions

Transplant if needed

Question 18

Treatment in alcoholic hepatitis

Answer 18

Rule out other stuff

Prednisone
- 40 mg daily for 28 days, followed by a 16 day taper
- Lille score can be used to determine response to tx

Pentoxifylline
- 400 mg three times per day as an alternative

Inhibitor of TNF (Controversial) Discontinue nonselective beta-blockers

Question 19

Risk scores in Alc Hep

Answer 19

Maddrey Discriminant Function
* DF= (4.6 x PT elevation)+ bilirubin mg/dl
* If >= 32, high short-term mortality, consider steroids

MELD
* If >11, high mortality

Glasgow alcoholic hepatitis score

Question 20

Type 1 vs type 2 AI hepatitis

Answer 20

Type 1 AIH
* SMA and ANA
* Sens 43%, Spec 99%
* Anti-actin Ab

Type 2 AIH
* Anti-LKM1
* Spec 99%

Question 21

Simplified diagnostic criteria for AI hep

Answer 21

Question 22

Autoimmune hepatitis treatment

Answer 22

• Treatment not required in asymptomatic patients with normal or minimally elevated transaminases and gamma globulin levels and minimal necroinflammatory activity on biopsy
• Monotherapy
• prednisone 60, 40, 30, 30, 20 indefinitely
• Combination
• Prednisone 30, 20, 15,15,10, … 5…..?
• Azathioprine 50 mg daily (or 1-2 mg/kg/day)

Investigational
* Budesonide (9mg/day) and azathioprine an option if no cirrhosis

Question 23

Haemachromatosis genes

Answer 23

C282Y or H63D

• C282Y homozygosity in 1 in 250 white northern European descendents
• Compound heterozygotes (C282Y/H63D) in 10%

Question 24

what upregulates hepcidin?

Answer 24

Interleukin-6 upregulates Hepcidin Ç Anemia of chronic disease

Question 25

Hepcidin main actions

Answer 25

* reducing transfer across the basolateral membrane of enterocytes by binding ferroportin * induces macrophages to store iron

Question 26

Haemachromatosis diagnostic criteria

Answer 26

* Transferrin saturation >45% * Ferritin >200 ng/ml in men or >150 ng/ml in women * Liver biopsy is only needed in select cases

Question 27

Haemachromatosis extra-hepatic manifestations

Answer 27

No Improvement with phlebotomy * Diabetes in 50% * Arthropathy (pseudogout, chondrocalcinosis) Second and Third MCP Improvement * Cardiomyopathy * Hypogonadism (secondary)

Question 28

ceruloplasmin in Wilson's disease

Answer 28

* Low serum ceruloplasmin - due to instability from lack of Cu incorporation * Binds and transports the copper from the cytoplasm to the late endosomal lumen

Question 29

Clinical manifestations Wilson's disease

Answer 29

Liver disease – 18-84% * Steatosis, fulminant hepatic failure with Coombs-negative hemolytic anemia, cirrhosis * Acute liver failure – alkaline phosphatase is typically normal or subnormal * Only 50% have Kayser-Fleischer rings Eye signs Neurologic Disease – 18-73% * Behavior deterioration, difficulty writing, tremor, speech change, Parkinsonlike, dysarthria, dystonia, ataxia, drooling * MR may show basal ganglia hyperintensity on T2-weighted images * 98% will have Kayser-Fleischer rings, Sunflower cataracts * Elevated copper in CSF Psychiatric – 10-100% * psychosis, depression, anxiety Most diagnosed between 5 and 35 years

Question 30

Treatment for Wilson's?

Answer 30

Copper removal 1. Chelators - usually 6 -12 months to remove the excess copper * D-penicillamine – 30% cannot tolerate * Trientine – Lower AE, expensive 2. Tetrathiomolybdate – Being studied Low Copper diet – Avoid liver, kidney, shellfish, nuts, dried fruits or beans, peas, unprocessed wheat, chocolate, cocoa, and mushrooms * Oral Zinc- interferes with Cu absorption

Question 31

Which patients should D-penicillamine not be used in

Answer 31

– Not ideal for neurologic disease – may worsen neuro symptoms in general ~ 30% cannot tolerate

Question 32

What is the number one cause of death in individuals with nonalcoholic steatoheapatitis (NASH)?

Answer 32

Cardiovascular disease

Question 33

MELD score needed for liver transplant

Answer 33

15 and above are approaching it

Question 34

Leading cause of death in a person who has had a liver transplant?

Answer 34

Infection

Question 35

Liver transplantation complications?

Answer 35

Infection - leading cause for mortality after transplant - Highest risk in the first three months CMV (arthralgias, leukopenia, recent treatment for rejection) Biliary obstruction or hepatic artery thrombus if cholestatic Hypertension - develops in approximately 70% post-transplant Diabetes Dyslipidemia Metabolic bone disease Malignancy