Liver dz Flashcards
Which combo of mom and baby can get AFLP?
Mother heterozygous for long chain hydroxyacyl CoA DH def, fetus homozygous
Sxs of AFLP?
Abd pain, HPM, liver fx
What is AFLP histo?
microvesicular steatosis
Which liver pts have the best post TP survival?
Cholestatic liver dz like PSC, alc second
Which post TP pts have worst survival
HCV
Liver bx in sarcoidosis shows what?
Noncaseating granulomas
What LFT is typically abnl in sarcoid of the liver? What other lab test can be helpful?
Alk phos; ACE
T/F: Pt with hepatic sarcoid should undergo pulm workup
True
What common drug can cause SOS?
Azathioprine, chemotx
What do pts with SOS present with?
Abd pain, distention, ascites
Which AI conditions more likely in pts with PBC?
Sjogrens, CREST, Raynauds, Thyroid Dz
T/F: UDCA can be used for pruritis in PBC?
False, no effect on itching
What % of normal ppl have +AMA?
<1%
What % of PBC pts are AMA neg?
5%
Type 1 GSD (Von Gierkes) has an inc risk of what?
Hepatic adenomas which can become HCC
T/F: GSD 1 is AW cirrhosis.
False, not AW cirrhosis
Which GSD AW cirrhosis?
Type 3 (Forbes-Cori) - debranching enzyme def and abnl prots accumulate in liver, hrt, skel muscle
What is the defect of GSD1?
G6Pase def
What should pts with GSD1 avoid and why?
Glc, galactose, frc, and Fasting - lead to hypoglycemia which can cause lactic acidosis, HPM, FTT, etc
What foods should be taken in more in GSD 1?
Complex carbs such as starches
Why should lipid emulsifications not be added in GSD 1 pts?
Already high risk of HLD
Erlenmeyer flask deformity of distal femur in what?
Gaucher dz
What is abnl in gaucher?
Glucocerebroside
Gaucher dz presentation
TCTopenia, bone pain, fx, HSM, anemia
Gaucher dz bx
Crumpled paper cells (Gaucher cells)
Name 4 bx findings in NASH.
Hepatocyte ballooning, MD bodies, acidophil bodies, hepatic steatosis