Pediatric Jaundice

Question 1

Which type of bilirubin is toxic to the CNS? (Be specific!)

Answer 1

FREE (not bound to albumin); UNCONJUGATED bilirubin

Question 2

The large amounts of unconjugated bilirubin in the blood of a new baby is due to what factors?

Answer 2

1. Hemolysis
2. Inadequate conjugation (due to RBC with shorter life span and immature glucouronosyltransferase) and clearance from GI tract.

Question 3

__________ is the most common type of bilirubin in neonatal hyperbilirubinemia due to ___________.

Answer 3

1. Unconjugated/indirect bilirubin (lipid soluble type of bilirubin bound to albumin, before reaching the liver)
2. Hemolysis of RBCS

Question 4

Differentiate breast feeding jaundice vs. breast milk jaundice?

Answer 4

• Breast feeding jaundice is more of a function of dehydration and decreased excretion of bilirubin in the stool (related to the supply of the breast milk, which is sometimes low in first few days)
• Breast milk jaundice is due to presence of deconjugating enzymes in milk

Question 5

What is jaundice?

Answer 5

Yellow-orange tint found in the sclera and skin in infants due to hyperbilirubinemia.

Question 6

_____ of newborns appear jaundiced during the first weeks of life.

Answer 6

2/3

Question 7

What 3 characters are newborns born with?

Answer 7

• 1. High hematocrits and RBC volume
• 2. RBC have a shorter life span (70-90 days)
• 3. Immature glucuronosyltransferase => cant conjugate bilirubin

Question 8

Conjugation of bilirubin occurs where?

Answer 8

Liver

Question 9

______ it initially low, overwhelming the amount of UCB that goes to the liver. BUT increases over the first few weeks.

Answer 9

UGT1A1

Question 10

What is the fate of conjugated/direct BR?

Answer 10

• => intestines via the gallbladder and bile duct.
• Once in the intestines:
  
  • 1. Beta-glucuronidase can deconjugate conjugated BR (water-soluble) from gut => blood
  • 2. Rest is pooped out.

Question 11

• Conjugated/direct BR is _____-soluble
• UCB is ______- soluble

Answer 11

• CBR = water soluble
• UCB = lipid soluble

Question 12

Visible jaundice early in life usually means that the TSB is at least ______

Answer 12

5mg/dL

Question 13

Non-pathologic hyperbilirubinemia is ALWAYS __________-

Answer 13

UNCONJUGATED

Question 14

Which type of hyperbilirubinemia is always pathologic?

Answer 14

Conjugated hyperbilirubinemia

Question 15

What are common causes of unconjugated hyperbilirubinemia?

Answer 15

1. Increase production of BR
2. Deficiency of liver upatake (bc must be with albumin)
3. Impaired conjugation (Gilbert, CN syndrome type 1, severe UGT1A1 deficiency)
4. Impaired enterohepatic circulation (decreased intake, decreased passage of stool)

Question 16

What are some of the main signs of Kernicterus in a newborn?

Answer 16

1. • Choreoathetotic movements
2. • Ballismus
3. • Upward gaze
4. • Dental dysplasia

Question 17

What are 4 pathologic abnormalities resulting in ↑ production unconjugated bilirubin in a new born?

Answer 17

• 1. Erythrocyte-enzyme deficiencies
• 2. Blood group incompatibility (ABO)
• 3. Structural defects in RBC’s
• 4. G6PD deficiency (enzyme deficiency)

Question 18

What is Rh and why should Rh testing be done on all pregnant women?

Answer 18

• Rh is inherited and found on surface of RBC’s
• If mom is Rh (-) and baby is Rh (+) –> some of babies RBC’s get into Mom’s circulation during pregnancy and Mom develops Rh antibodies
• No big deal in 1st pregnancy, but w/ subsequent pregnancies the Rh antibodies can cross placenta and wreak havoc on Rh (+) baby (hydrops fetalis or erythroblastosis fetalis)

Question 19

When does ABO incompatability occur and what can it cause?

Answer 19

• Mom = O blood type; Baby has A/B/AB blood type => unconjugated hyperbilirubinemia due to increase BR production.

Question 20

_____________ and ________ testing should be done on all pregnant women?

Answer 20

• ABO
• Rh (D) testing

Question 21

If Mom has type O blood or if Mom is Rh (-), the infant’s cord blood should be evaluated for what 3 things/how?

Answer 21

1. Blood type
2. Determine what their Rh is
3. DAT (Direct antibody/Combs test)

Question 22

If mom is ______ or ________, DO NOT test the cord blood.

Answer 22

1. Not O
2. Rh (+)

Question 23

If you see a high conjugated/direct BR, where is there a problem?

Answer 23

IN the liver or PAST the liver.

Bc conjugating occurs IN the liver

Question 24

What is the difference between Direct and Indirect Combs?

When is Direct Combs use?

Answer 24

• Direct: You are looking for moms antibodies that have attached themselves directly on the bbs RBC.
  
  • Used in situations where hyperbili is thought to be due to hemolysis, esp in ABO incompatibility.

Question 25

When do we have c**onjugated hyperbillirubinemia?**

Answer 25

1. Conjugated BR is \> 1mg/dL when TSB (Total serum BR) is less than 5mg/dL 2. Or when \>20% of our TSB is conjugated BR.

Question 26

\_\_\_\_\_\_\_ hyperbilirubinemia in a newborn bb is **RARE**.

Answer 26

**Conjugated hyperbilirubinemia**

Question 27

Causes of **conjugated hyperbilirubinemia?**

Answer 27

1. **UTI/sepsis** 2. **Biliary atresia/cholestasis** 3. **Hypothyroidism** 4. **Galactosemia**

Question 28

If a newborn has **conjugated hyperbilirubinemia** in the **first few post-natal weeks** what is the first thing you should think?

Answer 28

**Biliary atresia/cholestasis**

Question 29

What is acute bilirubin toxicity?

Answer 29

When you have a super **super high** *indirect bilirubin* (& bc lipid-soluble), can **cross BBB.**

Question 30

What is seen in **Phase 1 (the first 1-2 days)** of **acute bilirubin toxicity** in an infant (high unconjugated bilirubinemia)?

Answer 30

1. - Poor suck 2. - High pitched cry 3. - Stupor 4. - Hypotonia 5. - Seizures

Question 31

What is seen in **Phase 2 (middle of the 1st week of life)** of **acute bilirubin toxicity** in an infant (w/ high unconjugated bilirubinemia)?

Answer 31

1. **Hype**rtonia of extensor ms. 2. Opisthotonus 3. Retrocollis 4. Fever

Question 32

What is seen in **Phase 3 (after 1st week of life)** of **acute bilirubin toxicity** in an infant (w/ high unconjugated bilirubinemia)?

Answer 32

**Hypertonia**

Question 33

What is the chronic and permanent sequelae of **Bilirubin Induced Neurologic Dysfunction (BIND)?** ## Footnote **why does this happen?**

Answer 33

**Kernicterus:** UCB is lipid soluble and passwes through the BBB.

Question 34

If there is **jaundice in the first 24 hrs** or the **jaundice is excessive** for infants age; which tests should be run? When should Total Serum Bilirubin (TSB) be rechecked?

Answer 34

* **TSB**

Question 35

What are te 3 biggest risk factors for hyperbilirubinemia?

Answer 35

1. **Prematurity (before 35/36 weeks)** 2. **High hematocrit at birth** 3. **ABO incompatibility**

Question 36

What is the ***BiliTool***?

Answer 36

assess the risks of developing **hyperbilirubinemia or "jaundice"** in newborns over 35 weeks gestational age - tells you **treatment**

Question 37

What info do you enter in Bili Tool?

Answer 37

1. Newborn’s gestational age, 2. Birthday 3. Date and time of sampling and bilirubin levels.

Question 38

How to treat **mild jaundice,** but **not** a **candidate** for phottherapy?

Answer 38

**1. increase frequency of feeding** **2. Continue breast feeding**

Question 39

13. Explain how phototherapy decreases bilirubin levels in infants undergoing treatment for hyperbilirubinemia.

Answer 39

* Phototherapy isomerizes BR by exposing as much surface of BARE skin as possible, making it *water-soluble* so that you can get rid of it

Question 40

**Home phototherapy** can be used depending on?

Answer 40

1. BR levels 2. Home situation 3. Ability and willingness to follow up 4. Comfort level of parents

Question 41

What are 3 signs/symptoms of **Biliary Atresia (progressive and destructive inflammatory process that affects BOTH extra and intrahepatic biliary trea =\> jaundice in 1st few post-natal weeks)** in an infant?

Answer 41

1. **- Cholestatic jaundice (conjugated hyperbilirubinemia)** 2. **- Hepatomegaly** 3. **- Acholic stools**

Question 42

If there is **prolonged jaundice** in an **infant** and you are approaching 2 months, what should you start thinking about?

Answer 42

**Gilberts**

Question 43

**Crigler-Najjar Type 1** and **Type 2** differ in their response to what drug?

Answer 43

1. **Type 1** (total UDPGT deficiency) results in **SEVERE hyperbilirubinemia** with **high risk** of BIND/Kernicterus 2. **Type 2 (**partial UDPGT deficiency) results in **mild hyperbilirubinemia** with **low risk** of BIND/Kernicterus

Question 44

Extrahepatic biliary atresia occurs where in the hepatobilirary system?

Answer 44

Common bile duct