18-4 Flashcards
Cholestasis
Decrease in bile flow due to:
- impaired hepatocyte secretion OR
- obstruction
Cholestasis
Decrease in bile flow due to:
- impaired hepatocyte secretion OR
- obstruction
Decrease in bile flow?
Cholestasis
What 2 things can cause Cholestasis?
- Impaired hepatocyte secretion
- obstruction
What is the hallmark of Cholestasis?
Green-brown plugs of bile pigment in hepatocytes and dilated canaliculi
What is the hallmark of Cholestasis?
Green-brown plugs of bile pigment in hepatocytes and dilated canaliculi
What labs will be elevated with Cholestasis?
Bilirubin
Alkaline phosphatase
GGT
Symptoms of Cholestasis?
Jaundice, pruritus
Xanthomas
Decreased Fat-soluble vitamins
2 bile functions?
- Fat absorption
- Excretion of bilirubin, cholesterol, xenobiotics in stool
What is a toxic end product of heme degradation that is normally eliminated from the liver into bile?
Bilirubin
Jaundice will present when bilirubin is above?
2
What will help determine the cause of the hyperbilirubinemia?
Testing for unconjugated and conjugated bilirubin
Where is conjugated bilirubin eliminated?
In the urine
Where is unconjugated bilirubin eliminated?
INSOLUBLE - cannot be excreted in urine
Unconjugated bilirubin is normally bound to?
Albumin until levels are too high and then it diffuses into tissues
= Kernicterus
Increased production of bilirubin (hemolytic anemias) and decreased uptake/conjugation causes?
Unconjugated hyperbilirubinemia
What are 2 syndromes that can cause conjugated hyperbilirubinemia?
Dubin-Johnson - black liver
Rotor syndrome
Bile flow obstruction causes?
Conjugated hyperbilirubinemia
Physiologic jaundice of a newborn is due to?
LOW levels of UGT1A1
- bilirubin glucuronidation enzyme
Symptoms of Physiologic jaundice of a newborn, what makes it better and what makes it worse?
= Mild, transient jaundice
- Breast milk makes it worse
- Phototherapy makes it better
What type of hyperbilirubinemia is Physiologic jaundice of a newborn?
Unconjugated
- low UGT1A1
Breast milk includes _____ enzymes that makes jaundice worse for newborns
Deconjugating enzymes
Phototherapy makes bilirubin _____
Soluble = excreted into urine
If jaundice persists for 14-21 days after birth, what are 2 categories that it could be due to?
- Extrahepatic Biliary Atresia
2. Nonobstructive Neonatal Cholestasis
If jaundice persists for 14-21 days after birth, what are 2 categories that it could be due to?
- Extrahepatic Biliary Atresia
2. Nonobstructive Neonatal Cholestasis
Extrahepatic Biliary Atresia
Obstruction of extrahepatic biliary tree
– unknown cause
Gallbladder changes with Extrahepatic Biliary Atresia?
Small or absent gallbladder
With Extrahepatic Biliary Atresia, there is ____ secretion of technetium-99m in bile and the biliary tree is NOT _____
NO secretion of technetium-99m into bile
Biliary tree is NOT VISUALIZED
If the biliary tree is not visualized in a neonate, they have jaundice, dark urine and acholic stools, what is the likely diagnosis?
Extrahepatic Biliary Atresia
Nonobstructive Neonatal Cholestasis
Group of disorders that cause newborn jaundice
Inborn errors of metabolism and Alpha1 antitrypsin deficiency fall into what category?
Nonobstructive Neonatal cholestasis
Alagille Syndrome
- Nonobstructive Neonatal cholestasis
= Abnormal biliary tree – altered facies
Alagille Syndrome has mutations in?
JAG1 or NOTCH2
Abnormal biliary tree with altered facies?
Alagille Syndrome
