Pediatric Cholestasis

Question 1

When is cholestasis most common?

Answer 1

In infancy

Question 2

How does Direct Bili get converted into Urobilinogen?

Answer 2

Intestinal Bacteria

Question 3

What are the Components of Bile?

Answer 3

Water
Bile Acids
Phospholipids
Cholesterol

Question 4

What are the functions of Bile?

Answer 4

Excrete Toxins
Modulating cholesterol metabolism
Aid intestinal digestion and absorption of lipids and FSV

Question 5

Difference between jaundice and icterus?

Answer 5

Skin vs eye

Question 6

What are some presentaions of Cholestasis?

Answer 6

Jaundice/icterus, RUQ pain, Pruritis, N/V
Dark urine
Acholic (no bile in stool. Grey, tan, white poo) Microcephaly, dysmorphism, poor feeding

Question 7

Total Bili equals

Answer 7

Conj. Bili + Unconj.=

Question 8

Bili levels that tell you cholestasis

Answer 8

2mg/kg or >20% Direct

Question 9

Obstructive vs Hepatocellular Pattern

Answer 9

Obstructive- Inc. Direct Bili, Alk Phos, GGT

Hepatocellular- AST, ALT Inc more than Bili, AP, and GGT

Question 10

At what point is the pt too old to have physiologic jaundice?

Answer 10

2 weeks

Question 11

What is the cause of Neonatal jaundice?

Answer 11

Excess Unconjugated bili from:

Elevated Hct, shorter LS of RBCs, immature Gluc. Transferase

Question 12

What can worsen Physiologic Jaundice?

Answer 12

Breast Feeding Jaundice (poor feeding)

Question 13

What can prolong Physiologic Jaundice?

Answer 13

Breast milk jaundice (reabsorb bilirubin)

Prematurity

Question 14

What is Biliary aTresia?

Answer 14

Progressive inflammatory destruction of bile ducts, initailly extrahepatic.

Question 15

How is Biliary Atresia Diagnosed?(imaging)

Answer 15

Ultrasound

Question 16

How is Biliary Atresia Treated?

Answer 16

Kasai procedure. Add a bypass straight from the jejunum to the liver. (fat malabsorption is a side effect)

Question 17

What determines the effectiveness of the Kasai procedure?

Answer 17

How quickly they can be diagnosed and treated.

Question 18

What are the different types of Gallstones?

Answer 18

Cholelithiasis- Stones in GB
Cholecystitis- Inflamed GB
Choledocolithiasis- Stone in Common BD
Acalculous Cholecystitis-Cholecystitis without stones

Question 19

What is a unique finding of Choledochal Cysts?

Answer 19

a palpable mass in a newborn. It is a pre-malignant state!

Question 20

What gene is involved with Alagille syndrome?

Answer 20

Jagged 1 gene/Notch Receptor

Question 21

How does Alagille Sydrome present?

Answer 21

Xanthomas 
Characteristic face 
Pulmonic stenosis
Butterfly vertebra
Posterior Embryotoxin (eye finding)
Growth retardation
(Cardiovascular problems kill them first!)

Question 22

What is the genotype of Alpha 1 AT deficiency?

Answer 22

PiZ and PiS (reduced activity)
ZZ (most common in liver and lung disease)
Null(A1AT not detected)

Question 23

Treatment of A1AT deficiency

Answer 23

Wait til liver gets bad enough, then do a transplant.

Question 24

What are the Lab findings in PFIC?

Answer 24

GGT normal to low

Question 25

What is the most common medication induced Cholestasis?

Answer 25

TPN (total parenteral nutrition)

Question 26

What are Metabolic causes of Cholestasis?

Answer 26

Congenital Hypothyroidism (no symptoms and can have BAD effects)
Congenital Panhypopituitarism
UTI and Sepsis
Galactosemia

Question 27

How are MCFAs absorbed?

Answer 27

No transporter needed! Give them MCFAs to help them gain weight.