Pediatric Cholestasis Flashcards
When is cholestasis most common?
In infancy
How does Direct Bili get converted into Urobilinogen?
Intestinal Bacteria
What are the Components of Bile?
Water
Bile Acids
Phospholipids
Cholesterol
What are the functions of Bile?
Excrete Toxins
Modulating cholesterol metabolism
Aid intestinal digestion and absorption of lipids and FSV
Difference between jaundice and icterus?
Skin vs eye
What are some presentaions of Cholestasis?
Jaundice/icterus, RUQ pain, Pruritis, N/V
Dark urine
Acholic (no bile in stool. Grey, tan, white poo) Microcephaly, dysmorphism, poor feeding
Total Bili equals
Conj. Bili + Unconj.=
Bili levels that tell you cholestasis
2mg/kg or >20% Direct
Obstructive vs Hepatocellular Pattern
Obstructive- Inc. Direct Bili, Alk Phos, GGT
Hepatocellular- AST, ALT Inc more than Bili, AP, and GGT
At what point is the pt too old to have physiologic jaundice?
2 weeks
What is the cause of Neonatal jaundice?
Excess Unconjugated bili from:
Elevated Hct, shorter LS of RBCs, immature Gluc. Transferase
What can worsen Physiologic Jaundice?
Breast Feeding Jaundice (poor feeding)
What can prolong Physiologic Jaundice?
Breast milk jaundice (reabsorb bilirubin)
Prematurity
What is Biliary aTresia?
Progressive inflammatory destruction of bile ducts, initailly extrahepatic.
How is Biliary Atresia Diagnosed?(imaging)
Ultrasound
How is Biliary Atresia Treated?
Kasai procedure. Add a bypass straight from the jejunum to the liver. (fat malabsorption is a side effect)
What determines the effectiveness of the Kasai procedure?
How quickly they can be diagnosed and treated.
What are the different types of Gallstones?
Cholelithiasis- Stones in GB
Cholecystitis- Inflamed GB
Choledocolithiasis- Stone in Common BD
Acalculous Cholecystitis-Cholecystitis without stones
What is a unique finding of Choledochal Cysts?
a palpable mass in a newborn. It is a pre-malignant state!
What gene is involved with Alagille syndrome?
Jagged 1 gene/Notch Receptor
How does Alagille Sydrome present?
Xanthomas Characteristic face Pulmonic stenosis Butterfly vertebra Posterior Embryotoxin (eye finding) Growth retardation (Cardiovascular problems kill them first!)
What is the genotype of Alpha 1 AT deficiency?
PiZ and PiS (reduced activity)
ZZ (most common in liver and lung disease)
Null(A1AT not detected)
Treatment of A1AT deficiency
Wait til liver gets bad enough, then do a transplant.
What are the Lab findings in PFIC?
GGT normal to low
What is the most common medication induced Cholestasis?
TPN (total parenteral nutrition)
What are Metabolic causes of Cholestasis?
Congenital Hypothyroidism (no symptoms and can have BAD effects)
Congenital Panhypopituitarism
UTI and Sepsis
Galactosemia
How are MCFAs absorbed?
No transporter needed! Give them MCFAs to help them gain weight.
