Liver

Question 1

Causes of acute liver failure

Answer 1

Infection: hepatitis a,b,c
Poison/drugs: paracetamol, isoniazid, halothane, amanita phalloides (mushroom) 
Metabolic: Wilson disease 
Autoimmune hepatitis 
Reye syndrome

Question 2

Causes of chronic liver disease in children

Answer 2

Chronic hepatitis: Post viral hepatitis B and C 
Autoimmune hepatitis 
Drugs: nsaids 
Inflammatory bowel disease 
Primary sclerosing cholangitis

Wilson disease
Alpha antrypsin deficiency 
CF
Neonatal liver disease 
Bile duct lesion

Question 3

Kernicterus

Answer 3

Deposition of UGB in basal ganglia and brain stem nuclei

Early: drowsy, hypotonia and vomiting
Mid: hypertonia, opisthotonus, high pitched cry, bulging fontanelle, seizure

Complication: CP, MR, and hearing loss
Treatment: exchange transfusion

Question 4

Pathophys of physiological jaundice

Answer 4

Short lifespan of RBC leading to increases bilirubin production, deficiency of UGT (glucuronusyltransferase) leading to decreases clearance. Increased enterohepatic circulation
Appears 2-3 DOL, disappears within first week. Peak bilirubin (13-18 mg/dl)

Question 5

Pathophys of hyperbilirubinemia

Answer 5

Increased production 
Decreases hepatic uptake 
Decreases conjugation 
Impaired excretion 
Impaired bile flow (cholestasis) 
Increased enterohepatic circulation

Question 6

Pathological hyperbilirubinemia

Answer 6

Within first 24 hours
After first WOL
last > 2 w
Usually above 18 mg/dl

MCC: hemolytic, hematoma resorption
Sepsis, hypothyroidism

Question 7

Dubin Johnsons syndrome

Answer 7

AR

Defect in hepatocytes to SECRETE conjugated bilirubin

Question 8

Hepatic causes of hyperbilirubinemia (conjugated)

Answer 8

Infection: hepatitis, sepsis, TORCH
Metabolic: galactosemia AT1 def, CF
Generic: Dubin johonson syndrome
Drugs, TPN, idiopathic neonatal hepatitis

Question 9

MCC of hyperbilirubinemia:

Answer 9

Physiological
breast feeding
breast milk
hemolysis