Jaundice of the newborn

Question 1

Jaundice in the first week of life is more likely to be _____ than ______

Answer 1

normal than abnormal- physiologic jaundice

Question 2

List features that suggest physiologic jaundice

Answer 2

• onset after first day of life
• peaks at 3 to 5 days, resolves within 1-2 weeks
• bilirubin is mostly unconjugated, peaks at 12-15

Question 3

What is breast milk jaundice syndrome?

Answer 3

Later occurrence- 10-15 days

Possibly due to UGT1 inhibition of deficiency, leads to increased enteral reabsorption

Question 4

Describe the differential diagnosis of a jaundiced infant with mostly unconjugated bilirubin

Answer 4

hemolysis

defects of glucoronidation: Gilbert’s, Crigler-Jajjar

Question 5

What is the concerning complication of extreme unconjugated hyperilirubinemia?

Answer 5

Kernicterus

Question 6

What are the goals of treatment for jaundice due mainly to unconjugated bilirubin?

Answer 6

Decrease bilirubin levels, prevent neurotoxicity

Question 7

What is the differential diagnosis of a jaundiced infant with mostly conjugated bilirubin?

Answer 7

• defects of handling/ export of conjugated bilirubin: Rotor, Dubin Johnson
• hepatocyte injury
• biliary obstruction

Question 8

What is the concerning complication of conjugated hyperilirubinemia?

Answer 8

liver failure

Question 9

How is it possible to distinguish between the causes of conjugated hyperbilirubinemia in infants?

Answer 9

• Defects in export of conjugated bilirubin like Rotor, Dubin-Johnson do produce liver injury
• hepatocyte injury will also have elevated ALT, AST
• biliary obstruction will have elevated GGT

Question 10

List risk factors for severely increased levels of unconjugated bilirubin in the neonate

Answer 10

• ABO incompatibility
• G6PD deficiency
• prematurity
• exclusive breastfeeding

Question 11

_____ at <24 hours of age requires urgent evaluation

Answer 11

icterus

evaluation: bilirubin levels, ABO type/ Coombs, CBC

Question 12

How is unconjugated hyperbilirubinemia treated?

Answer 12

phototherapy
trial of formula feeding
exchange transfusion if high risk for kernicterus

Question 13

Guidelines for initiation of phototherapy stratify the risk of a given level of bilirubin according to other factors. Infants of ____age are at a greater risk at any given level of total bilirubin

Answer 13

younger age

other risk factors: prematurity, acidosis, lethargy, temperature instability, hemolysis, asphyxia, sepsis

Question 14

Icterus at > ______ age always requires evaluation

Answer 14

2 weeks

Question 15

List causes of conjugated hyperbilirubinemia in the neonate

Answer 15

extrahepatic biliary atresia
metabolic/ genetic conditions: Rotor, Dubin-Johnson, hemochromatosis
Non-bacterial infection: CMV, rubella, HSV, toxoplasma
sepsis, UTI
Endocrine: hypothyroidism
Maternal drugs

Question 16

What is extrahepatic biliary atresia?

Answer 16

the common bile duct between the liver and the small intestine is either blocked or absent

Question 17

What are signs of extrahepatic biliary atresia?

Answer 17

Jaundice beyond 2 weeks of age
acholic stools
failure to thrive
GGT elevation > ATL/AST

Question 18

How is extrahepatic biliary atresia diagnosed?

Answer 18

ultrasound- gallbladder present?

biopsy- bile duct proliferation, duct plugs

Question 19

What is the treatment for extrahepatic biliary atresia?

Answer 19

Kasai procedure (porto-enterostomy)

Must be performed by about 8 months, diagnosis must be made by 8 weeks for best surgical outcomes