Williams: Liver function Flashcards
what to also check for if patient has ADHD
Wilson disease
liver labs to understand function of liver
albumin
PT/INR
hepatocellular damage
AST/ALT elevation
biliary tract damage
Alk Phos and GGT
how to tease out liver or bone problem when Alk Phos is elevated
GGT lab
how to evaluate jaundice
get total bilirubin and fractionate it
bilirubin metabolism steps:
-old RBCs has hemeprotein and gives to liver macrophage
-heme protein broken down to biliverdin by hemeoxygenase
-biliverdin to bilirubin (unconjugated) by biliverdin reductase
-unconjugated bilirubin travels attached to albumin to the liver
-bilirubin + glucuronic acid (and UGT1A1)= conjugated
-leaves through MRP2 and fine until runs into bacteria and turned into urobilinogen and excreted
of what?
liver disease
mostly indirect bilirubin
failure of conjugation
Crigler Najjar
mostly indirect bilirubin
increased load
hemolytic disease
mostly direct bilirubin in the serum mechanism
cant evacuate hepatocyte/hepatocyte damage
blockage of bile ducts inside or outside liver
Having a ____ that measures above normal size does not automatically equal pathology
liver
H and E
cholestasis
-Crucial to digest and absorb lipids
-Synthesis and maintain balance with cholesterol
-GI antimicrobial defense
-Prevent formation of Ca2+ gallstones
bile acids
concentrated black bear derived bile acids that help break down gallstones
URSODIOL
primary carbohydrate storage in the body
glycogen (liver, muscle)
releases glucose to maintain normal blood glucose levels
glycogen
glucose-6-phosphatase deficiency
Type I GSD (Von Gierke)
hypoglycemia
hepatomegaly
Doll Face
Type I GSD (Von Gierke)
decifiency in glycogen debranching enzyme
hepatomegaly
Type III GSD (Forbes-Cori)
deficiency in glycogen branching enzyme
cirrhosis
Type IV GSD (Anderson’s)
mutation in SERPINA1 gene
alpha-1-antitrypsin deficiency
phenotype of A1AT deficiency most commonly associated with liver disease
PI*ZZ
main way to diagnose alpha-1-antitrypsin deficiency
A1AT phenotype
hepatocytes stained with PAS concentrated in what zone for Alpha-1-antitrypsin deficiency
zone 1 (periportal)
no____exposure for patients with alpha-1-antitrypsin deficiency
smoke
copper overload in liver and other organs
Wilson’s Disease
mutated _____ seen in Wilson’s disease
ATP7b
Kayser-Fleischer rings
Wilson’s disease
____ are seen in 90% of patients with neuro and psychiatric involvement with wilson’s disease
K-F rings
diagnose this with:
slit lamp exam
ceruloplasmin
urine Cu2+
Wilson’s disease
to Rx Wilson’s disease
TRIENTINE (copper chelator)
excessive storage of iron
hereditary hemochromatosis
mutation in HFE gene
hereditary hemochromatosis
to Rx hereditary hemochromatosis
phlebotomy
