18-4

Question 1

Cholestasis

Answer 1

Decrease in bile flow due to:

• impaired hepatocyte secretion OR
• obstruction

Question 2

Cholestasis

Answer 2

Decrease in bile flow due to:

• impaired hepatocyte secretion OR
• obstruction

Question 3

Decrease in bile flow?

Answer 3

Cholestasis

Question 4

What 2 things can cause Cholestasis?

Answer 4

• Impaired hepatocyte secretion

- obstruction

Question 5

What is the hallmark of Cholestasis?

Answer 5

Green-brown plugs of bile pigment in hepatocytes and dilated canaliculi

Question 6

What is the hallmark of Cholestasis?

Answer 6

Green-brown plugs of bile pigment in hepatocytes and dilated canaliculi

Question 7

What labs will be elevated with Cholestasis?

Answer 7

Bilirubin
Alkaline phosphatase
GGT

Question 8

Symptoms of Cholestasis?

Answer 8

Jaundice, pruritus
Xanthomas
Decreased Fat-soluble vitamins

Question 9

2 bile functions?

Answer 9

• Fat absorption

- Excretion of bilirubin, cholesterol, xenobiotics in stool

Question 10

What is a toxic end product of heme degradation that is normally eliminated from the liver into bile?

Answer 10

Bilirubin

Question 11

Jaundice will present when bilirubin is above?

Answer 11

2

Question 12

What will help determine the cause of the hyperbilirubinemia?

Answer 12

Testing for unconjugated and conjugated bilirubin

Question 13

Where is conjugated bilirubin eliminated?

Answer 13

In the urine

Question 14

Where is unconjugated bilirubin eliminated?

Answer 14

INSOLUBLE - cannot be excreted in urine

Question 15

Unconjugated bilirubin is normally bound to?

Answer 15

Albumin until levels are too high and then it diffuses into tissues
= Kernicterus

Question 16

Increased production of bilirubin (hemolytic anemias) and decreased uptake/conjugation causes?

Answer 16

Unconjugated hyperbilirubinemia

Question 17

What are 2 syndromes that can cause conjugated hyperbilirubinemia?

Answer 17

Dubin-Johnson - black liver

Rotor syndrome

Question 18

Bile flow obstruction causes?

Answer 18

Conjugated hyperbilirubinemia

Question 19

Physiologic jaundice of a newborn is due to?

Answer 19

LOW levels of UGT1A1

- bilirubin glucuronidation enzyme

Question 20

Symptoms of Physiologic jaundice of a newborn, what makes it better and what makes it worse?

Answer 20

= Mild, transient jaundice

• Breast milk makes it worse
• Phototherapy makes it better

Question 21

What type of hyperbilirubinemia is Physiologic jaundice of a newborn?

Answer 21

Unconjugated

- low UGT1A1

Question 22

Breast milk includes _____ enzymes that makes jaundice worse for newborns

Answer 22

Deconjugating enzymes

Question 23

Phototherapy makes bilirubin _____

Answer 23

Soluble = excreted into urine

Question 24

If jaundice persists for 14-21 days after birth, what are 2 categories that it could be due to?

Answer 24

1. Extrahepatic Biliary Atresia

2. Nonobstructive Neonatal Cholestasis

Question 25

If jaundice persists for 14-21 days after birth, what are 2 categories that it could be due to?

Answer 25

1. Extrahepatic Biliary Atresia | 2. Nonobstructive Neonatal Cholestasis

Question 26

Extrahepatic Biliary Atresia

Answer 26

Obstruction of extrahepatic biliary tree | -- unknown cause

Question 27

Gallbladder changes with Extrahepatic Biliary Atresia?

Answer 27

Small or absent gallbladder

Question 28

With Extrahepatic Biliary Atresia, there is ____ secretion of technetium-99m in bile and the biliary tree is NOT _____

Answer 28

NO secretion of technetium-99m into bile | Biliary tree is NOT VISUALIZED

Question 29

If the biliary tree is not visualized in a neonate, they have jaundice, dark urine and acholic stools, what is the likely diagnosis?

Answer 29

Extrahepatic Biliary Atresia

Question 30

Nonobstructive Neonatal Cholestasis

Answer 30

Group of disorders that cause newborn jaundice

Question 31

Inborn errors of metabolism and Alpha1 antitrypsin deficiency fall into what category?

Answer 31

Nonobstructive Neonatal cholestasis

Question 32

Alagille Syndrome

Answer 32

- Nonobstructive Neonatal cholestasis | = Abnormal biliary tree -- altered facies

Question 33

Alagille Syndrome has mutations in?

Answer 33

JAG1 or NOTCH2

Question 34

Abnormal biliary tree with altered facies?

Answer 34

Alagille Syndrome