Budd-Chiari, A1A deficiency , Hemochromatosis, Wilson and other causes of Jaundice Flashcards
What is Budd-Chiari Syndrome and what predisposes one to it?
This is venous outflow obstruction at the Hepatic veins most often due to a Venous Thrombosis.
Anyone in a hypercoagulable state is predisposed.
What are some causes of hypercoagulable states that can lead to Budd-Chiari?
Myeloproliferative disorders such as Polycythemia, Exogenous Estrogen use as in OCPs or Even just pregnancy and post pregnancy hyperestrogen.
What is Nutmeg liver and what is it most associated with?
Nutmeg liver is a stupid name for Congestive Hepatopathy in which there is a back up of blood flow either due to Right Sided Heart Failure (RHF) or due to Budd-Chiari.
What is a1-trypsin deficiency and what two organs are primarily affected?
Lungs and Liver. Liver produces the a1-antitrypsin enzyme but is making a poor version of it leading to a build up of granules known as PAS+ globules.
What disease gives you Emphysema and Cirrhosis without the fun of drinking or smoking?
A1 Antitrypsin deficiency. (Which is not actually a deficiency or it wouldn’t be hurting the liver also with globular congestion of the enzyme).
How is a smoker’s emphysema histologically different than an a1-antritrypsin pt?
Smoker emphysema tends to be worse around the central lobules where the carcinogens are deposited whereas A1A deficiency pts have a panacinar diffuse emphysema.
Where is Heme converted to Biliverdin and then Bilirubin?
Macrophages are responsible for RBC death. They hand off the Bilirubin to Hepatocytes.
Why does Bilirubin need to be conjugated to UDP-glucorynltransferase?
Bilirubin needs the chaperone for water solubility and then becomes direct or conjugated bilirubin and is excreted in the biliary tract.
Why does physiologic jaundice occur in newborns?
Fetus previously sent its unconjugated bilirubin through the placenta for mother to conjugate it with her liver. She’s doing the work for two. Neonate does not develop the enzymes for conjugation fully until 6 months.
Why is neonate RBC turnover so much faster than adults?
y-globin chains for high oxygen uptake are in fetal cells until they begin making beta chains.
Prehepatic jaundice has which type of bilirubin while Post-Hepatic aka Obstructive has which?
Prehepatic has Unconjugated; Obstructive has conjugated. Primary or Intrahepatic can be mixed, except in the cases of an actual genetic defect in which the specific cause will determine whether the bilirubin has been conjugated.
Which 4 genetic diseases in cause intrahepatic jaundice and what type of bilirubin is found in each?
Gilbert and Crigler Najjar have a problem with UDP-GT, hence they will have unconjugated bilirubin with the potential for Kernicterus. Dubin-Johnson and Rotor Syndrome have problems with a transport protein for the bile caliculi - so they have conjugated hyperbilirubinemia. Dubin Johnson has hyperpigmented dark cells on biopsy.
Biliary Atresia is so much worse than any other Hyperbilirubinemia despite being conjugated…why?
Can it cause Kernicterus?
It cannot cause Kernicterus bc it is conjugated bilirubin which can’t cross the BBB. Yet - the backup and congestion from the fibrosis and obliteration of the biliary tract right outside the liver leads to fulminant hepatitis and liver failure.
When does Biliary Atresia present and what causes it?
Idiopathic. It presents by 2 months of age.
Why would someone with Biliary Atresia have acholic stools and dark urine?
Bilirubin is being sent to the kidneys and not reaching the feces.
What is the most common indication for emergent radically invasive surgery or liver transplant if the biliary fibrosis has already ruined the liver?
Biliary Atresia by far.
