Liver Metabolism Flashcards
Prehepatic Jaundice
Caused by hemolytic anemia (EBF. HbF)
elevated UBr (+indirect)
Total Br=5
negative urinary urobili (Ubr cannot be excreted because it is attached to albumin) however the urine is often dark to increase in CBr and Hb from lysed blood stools
brown stool, because you are still able to conjugate some.
Hepatic Jaundice
infection or damage to liver elevated UBr elevated CBr -urobili \+urine br= dark yellow urine Pale stool =decreased starcobili
Post hepatic jaundice
bile duct obstruction elevated CBr (+direct) -urobili, \+urine Br =dark yellow urine -starcobili=clay colored stool
Gilbert’s syndrome
Mild unconjugated hyperbilirubinemia due to LOW UDPGT stress induced jaundice Mid-high UBr low-norm CBr neg Urine Br low urobili low stercobili
hepatic jaundice
Crigler Najjar syndrome type I
complete loss of UDPGT VERY high Ubr low CBr autosomal recessive kernicterus by age 1 neg Urine Br low urobili low stercobili
tx= liver transplant
Crigler Najjar syndrome type II
partial deficiency of UDPGT
VERY high Ubr
low CBr
autosomal dominant
neg Urine Br
low urobili
low stercobili
tx= phenobarbital, good outcomes
Dubin-Johnson syndrome
Decreased canalicular excretion normal UBr High CBr \+Urine Br -Urobilinogen -Stercobilin
Black liver biopsy due to melanin in hepatocytes and Kupffer cells
(conjugated hyperbilirubinemia)
Rotor’s syndrome
decreased uptake in the liver normal UBr High CBr \+Urine Br -Urobilinogen -Stercobilin
non-itching, no black liver
L-Glutamine
acts as a temporary storage of N
can donate the amino group when needed for biosynthesis
Ornithine transcarboxylase
converts Carbamoyl Phosphate with Ornithine to form citrulline
first step of the Urea cycle
Argininosuccinate synthase
converter citrulline to arginoino-succinate
PKU
Loss of Phenylalanine hydroxylase
increases production of toxic metabolites
Tx= avoid aspartame, low Phe diet+ exo Tyr
AR
