Metabolic Liver Diseases Flashcards
what do you find on liver bx for NASH?
NASH requires histology for dx
- hepatic steatosis
- lobular inflammation
- ballooned hepatocytes
what does vitamin E do for NASH?
improves inflammation and steatosis on histology, but not fibrosis
Dubin Johnson syndrome.
- LAE pattern
- risk of HCC
normal LAE
no liver failure or risk of HCC
caused by defect in canalicular transport (tf, black liver) of conjugated bilirubin into bile. Causes conjugated hyperbilirubinemia. at birth.
for pts suspected of having hemochromatosis, what is the significance of ferritin >1000?
ferritin >1000 is a/w increased risk for having advanced hepatic fibrosis or cirrhosis, and therefore warrants liver bx
what is the role of hepcidin and ferroportin in hemochromatosis?
normally, hepcidin is secreted by liver and down regulates iron absorption from intestine and iron release from macrophages.
when there’s iron excess, hepcidin usually increases
ferroportin is an iron transport protein on basolateral membrane of enterocytes to transfer iron from cell cytoplasm into extracellular space. Hepcidin causes ferroportin to be degraded.
true or false - pts w/ PBC are at risk of developing varices in the absence of cirrhosis
true - PBC can be associated w/ pre-sinusoidal portal hypertension in the absence of cirrhosis, resulting in risk of developing varices
what is the best prognostic marker for pts w/ PBC?
total bilirubin level is the best predictor of survival in PBC
what are the benefits of ursodeoxycholic acid in management of PBC?
- decrease LDL
- reduce risk of variceal bleed
- slow progression of PBC
- reduce risk of needing liver transplant
histology associations with…
- PBC
- PSC
- autoimmune hepatitis
- acute cellular rejection after liver transplant
- PBC (florid duct lesion)
- PSC (periductal concentric fibrosis (“onion-skinning”)
- autoimmune hep (hepatocyte rosettes w/ plasma cell infiltrate)
- acute cellular rejection (endotheliitis)
what kind of cancer screening is recommended for pts w/ PSC?
- cholangiocarcinoma (all adults >=20yo)
- gallbladder cancer (all adults >=20yo)
- colon cancer (OC q1-2yr for pts w/ concomitant IBD. OC q3-5yr if w/o IBD)
- HCC
tx for IgG4-related sclerosing cholangitis?
-corticosteroids
must distinguish from PSC since they have diff tx and prognoses
what are features of early hepatic artery thrombosis after liver transplant?
tx?
- worsening clinical status
- severe elevation in AST, ALT
- rising INR
- acidosis
tx: urgent liver transplant. a surgical emergency. status 1A for transplant.
what are the free hepatic venous pressure (FHVP), wedge hepatic venous pressure (WHVP) and hepatic venous pressure gradient (HVPG) for the following?
- schistosomiasis
- cirrhosis
- R heart failure
WHVP / FHVP / HVPG
schisto (pre-sinusoidal)
n n n
cirrhosis (sinusoidal)
H n H
R heart failure (post-sinusoidal)
H H n
What radiographic findings do you see in Budd Chiari syndrome?
Budd Chiari syndrome - hepatic vein problem
caudate lobe hypertrophy - since caudate lobe has separate venous drainage directly into IVC (bypassing the hepatic veins), it gets larger.
venous collaterals
which liver disease is Mallory Denk bodies associated w/?
alcoholic hepatitis
though mallory denk bodies are not exclusive to alc hep.
what cutoff of Maddrey discriminant function do you use to determine tx of alc hep? why?
how do you assess tx?
MDF >32, predicts 20-50% 30-day mortality if >32
tx w/ prednisolone
assess tx response w/ Lille score on day 7 to see if you should continue for total 28 days of prednisolone
what is a safe med to use for ethos abstinence for ethos cirrhotics?
baclofen
acamprosate and naltrexone have possible hepatotoxicity
what is the gold standard for dx of alpha 1 antitrypsin deficiency?
a1at phenotyping - tells you if the folded protein is M, S, or Z.
measuring serum a1at is tricky, since a1at is an acute phase reactant and may be falsely elevated during inflammation
if azathioprine is a no go for autoimmune hep, what are second line?
mycophenolate mofetil and calcineurin inhibitors (such as cyclosporin)
you diagnose portal vein thrombosis in a cirrhotic. What should you check for?
when a cirrhotic pt has PVT, must consider possibility of HCC-related tumor thrombus
for suspicion of HCC-associated PVT, what should you check? how do you treat PVT?
check AFP and get further imaging
suspect HCC-related tumor thrombus if endoluminal material enhances during the arterial phase of imaging
tx w/ anticoagulation (coumadin or LMWH) at least 6 months and reimagine w/ ultrasound to check for recanalization
what is the goal of tx for acute PVT?
we tx acute PVT w/ anticoagulation, with goal of preventing extension of clot into the mesenteric veins and reanalyzing the portal system to prevent portal HTN
what is sinusoidal obstruction syndrome? when does it happen?
sinusoidal obstruction syndrome
injury to the sinusoidal vascular epithelium, leading to fibrinogen deposition, vascular congestion, and eventually hepatocyte necrosis
most often seen w/n 3 weeks of hematopoietic stem cell transplant and also associated w/ specific myeloablative regimens (containing cyclophosphamide), or high dose XRT.
how does sinusoidal obstruction syndrome present?
rapid onset ascites, edema, weight gain
tender hepatomegaly, elevated LAE are common
what are the diagnostic criteria for sinusoidal obstruction syndrome?
Seattle criteria
-Tbili >2
-hepatomegaly/RUQ Pain
wt gain > 2% body weight
Baltimore criteria:
-Tbili >2
and 2 of the following: hepatomegaly, ascites, wt gain >5%`
how do you reduce risk of and treatment sinusoidal obstruction syndrome?
use less toxic myeloablative regimen
give ursodeoxycholic acid or low dose heparin pre-transplant
