Case 8: Jaundice

Front 1

What is kernicterus?

Back 1

• staining of basal ganglia and cranial nerve nuclei by bilirubin
  
• toxic effects of high levels of unconjugated bilirubin
  

Front 2

Sequelae of jaundice?

Back 2

• lose of suck reflex
  
• become lethargic
  
• develop hyperirritability and seizures
  
• ultimately die
  

Front 3

What may happen to babies who survive kernicterus?

Back 3

• opisthotonus
  
• rigidity
  
• oculomotor paralysis
  
• tremors
  
• hearing loss
• ataxia

Front 4

Screening for kernicterus?

Back 4

screen for Rh incompatability

use RhoGAM

treatment of hyperbilirubinemia with phototherapy

Front 5

Newborn bilirubin physiology?

Back 5

most bili produced in healthy newborn comes from physiological breakdown of RBCs

in adults most bile metabolized by intestinal flora and excreeted in stool, however newborn infant lacks gastrointestinal flora to metabolize bile

Front 6

How do newborns absorb lots of bili?

Back 6

via enterohepatic circulation

Front 7

Types of jaundice?

Back 7

• Physiological jaundice
  
• Breastfeeding jaundice
  
• Breast milk jaundice
  
• Hemolysis
  
• Other causes
  

Front 8

Physiologic jaundice

Back 8

2nd or 3rd day of life in term baby

BR peaks at day 3 or four

Front 9

What causes physiological jaundice?

Back 9

Increased bilirubin production (from the breakdown of the short-lived fetalred cells)Relative deficiency of hepatocyte proteins and UDPGTLack of intestinal flora to metabolize bileHigh levels of β-glucuronidase in meconiumMinimal oral (enteral) intake in the first 2-4 days of life, resulting in slowexcretion of meconium (especially common with breastfed infants).

Front 10

Breastfeeding jaundice?

Back 10

first week of life

occurs when milk supply relatively or absolutely low resulting in limited enteral intake

"Lack of breast milk" jaundice

Front 11

Breastmilk jaundice?

Back 11

begins four to 7 days of life

may not peak to 10 to 14 days

can persist up to 12 weeks

cause not completely understood

one explanation is that β-glucuronidase present in breast milk deconjugates bilirubin in the intestinaltract; the unconjugated bilirubin is then reabsorbed via enterohepaticcirculation.

Front 12

How to detect hemolysis?

Back 12

Direct coombs or direct antibody test positive

Front 13

When is DAT positive?

Back 13

Rh incompatability

ABO incompatability

Incompatabilities with minor blood group antigens

antibody negative hemolysis in infants with red cell membrane defects or red cell enyzyme defects

Front 14

Rh incompatability

Back 14

mother is Rh-negative and baby is Rh-positive

Front 15

ABO incompatability

Back 15

(mother is type O and baby is type A or B)

Front 16

Non hemolytic red cell breakdown causes?

Back 16

• Extensive bruising from birth trauma
  
• Large cephalohematoma or other hemorrhage (e.g., intracranial)
  
• Polycythemia
• Swallowed blood (large amounts) during delivery.

Front 17

Metabolic errors?

Back 17

Crigler Najjar syndrome

Galactosemia

hypothyroidism

Front 18

Crigler Najjar syndrome

Back 18

hyperbilirubinemia results from decreased bilirubinclearance caused by deficient or completely absent UDPGT.

Front 19

Common ethnicities for jaundice?

Back 19

asian newborns

Front 20

What other factors contribute to hyperbilirubinemia?

Back 20

• prematurity
  
• bowel obstruction
  
• birth at high altitude
  

Front 21

What is the typical breastfeeding pattern in 24 hours?

Back 21

8-12 times in 24 hours

Front 22

Benefits of breastfeeding for infants?

Back 22

• Maternal-infant bonding
  
• Protection against some infections (e.g. otitis media, respiratory infections, diarrhea)
  
• Reduced rates of Sudden Infant Death Syndrome
• Reduced rates of some allergic reactions

Front 23

Benefits of breastfeeding for mother?

Back 23

• Decreased postpartum bleeding and more rapid uterine involution
  
• Lactational amenorrhea and delayed resumption of ovulation with increased child spacing
  
• Earlier return to pre-pregnant weight (compared with women who formula-feed)
  
• Improved bone remineralization postpartum with reduction in hip fractures in thepostmenopausal period
  
• Decreased cost, relative to formula
  

Ready availability without preparation time

Front 24

Common breastfeeding problems?

Back 24

• enlarge, tender breasts (engorgement, mastitis, plugged ducts
  
• improper latch, suckle
  
• prolonged feeds
  
• infants fall asleep before finishing feeds
  
• maternal inexperience/anxiety
  

Front 25

Major breast milk nutrients?

Back 25

carbs, fats, and proteins

Front 26

Carbs in breast milk?

Back 26

lactose, main

Front 27

Lipids in breast milk?

Back 27

approx 50% of calories in human milk come from lipids

increases in concentration as nursing proceeds, important to empty breast before going to the next breast

Front 28

Proteins

Back 28

whey proteins (70%) and casein (30%)

Front 29

Voiding patterns in newborn?

Back 29

day 3: 3-4 times per day

day 6: void 6-8 times per day

urine should be pale yellow

Front 30

Stooling patterns in newborn?

Back 30

Day 3: meconium should no longer appear in stool; BM start to be ellow

Day 6-7: 3-4 stools per day, many infants pass stool with every feed

little odour

Front 31

Acholic stool

Back 31

infant's stool loses colour

may be sign of biliary atresia

Front 32

Biliary atresia features

Back 32

jaundice

dark urine

acholic stools (between 3 and 6 weeks of age)

refer to ped gastro or ped surgeon

Front 33

Treatment of biliary atresia

Back 33

Kasai procedure

(anastomosis of the i ntrahepatic bile ducts to a loop of intestine to allow bile to drain directly intothe intestine)

Front 34

What is the prognosis of hyperbilirubinemia in newborn?

Back 34

rare to develop kernicterus

most jaundiced newborns dont have major risks for adverse outcomes

Front 35

Major risk factors for adverse outcomes

Back 35

• TSB or TcB in high risk zone
  
• jaundice in first 24 hours of life
  
• blood group incompatability
  
• GA 35-36 weeks
  
• previous sibling received photo
  
• cephalohematoma/bruising
  
• exclusive breastfeeding
  
• east asian race
  

Front 36

Minor risk factors for adverse outcomes?

Back 36

• Pre-discharge TSB or TcB level in the high intermediate-risk zone
  
• Gestational age 37-38 week
  
• Jaundice observed before discharge
  
• Previous sibling with jaundice
  
• Macrosomic infant of a diabetic mother
  
• Maternal age >25 y
• Male gender

Front 37

Decreased risk of adverse outcomes?

Back 37

• TSB or TcB level in the low-risk zone
  
• Gestational age 41 week
  
• Exclusive bottle feeding
  
• Black race
• Discharge from hospital after 72 hours

Front 38

Key physical findings on delivery that may alert you to bili?

Back 38

• cephalohematoma
  
• bruising
  

Front 39

risk factors for DD of hip?

Back 39

• Girls
  
• Infants born via breech presentations
  
• Caucasians
  
• Native Americans
  
• Infants with a family history of DDH
  

Front 40

How to screen for DDH?

Back 40

barlow and ortolani maneuvers

Front 41

evaluation of DDH

Back 41

imaging for all female infants born via breech delivery

US at 4-6 weeks or pelvis radiograph at 4 months

Front 42

therpy for ddh?

Back 42

corrected early

Front 43

Signs and symptoms of untreated congenital hypothyroidism?

Back 43

• Prolonged jaundice
  
• Lethargy
  
• Large fontanelles
  
• Macroglossia (enlargement of the tongue)
  
• Umbilical hernia
  
• Constipation
  
• Abdominal distention
• Severe developmental delay

Front 44

When to do neonatal screen?

Back 44

within 24 hours after birth

get PKU done

Front 45

How much weight might breastfed infnats lose in first four to five days of life?

Back 45

7-10%

regain by at least 2 weeks of age

Front 46

Three ways to evaluate for jaundice?

Back 46

inspection

serum bili measurement

transcutaneous bili measurement

Front 47

How to evaluate for etiology of hyperbilirubinemia in newborn?

Back 47

• Age at which jaundice begins
  
• Weight history
  
• Feeding history
  
• Pregnancy history
• Signs of illness in the newborn

Front 48

Age at which jaundice begins

Back 48

Can help determine the risk for severe hyperbilirubinemia and can direct you to specificcauses of jaundice, especially hemolysis.

Front 49

Pregnancy history

Back 49

• maternal infections may affect fetus in utero, resulting in IUGR
  
• consequence may be SGA infant with risk of direct hyperbilirubinemia
  

Front 50

DDx for jaundice in newborn

Back 50

• physiologic jaundice
  
• hemolysis
  
• hypothyroidism
  
• metabolic disease
  
• biliary atresia
  
• intrinsic liver disease
  
• birth trauma
  
• sepsis, TORCH
  
• Gilbert syndrome, Crigler Najjar syndrome
  

Front 51

Physiological jaundice

Back 51

Physiologic jaundice typically appears earlier than on day 4.The level of hyperbilirubinemia and the time course helps todistinguish physiologic from breast milk jaundice.

Front 52

Hemolysis

Back 52

Possible reasons for hemolysis include:ABO incompatibilityRh incompatibilityG6PD deficiency

Front 53

Hypothyroidism

Back 53

Typically detected by the neonatal screen.

Front 54

Metabolic disease

Back 54

Often children with inborn errors of metabolism-such as galactosemiaor urea cycle defects-present with liver dysfunction, including jaundice,in addition to other features (like seizures, sepsis, ascites) dependingon the defect.The newborn screen can help rule out these diagnoses.

Front 55

Biliary atresia

Back 55

Typically presents after 2 weeks of age with progressive jaundice andacholic stools.Causes a direct hyperbilirubinemia.

Front 56

Instrinsic liver disease

Back 56

Very rare cause of neonatal jaundice

Front 57

Birth trauma(cephalohematomaor other bruising)

Back 57

Reabsorption of blood and metabolism of red blood cells can causejaundice.

Front 58

Sepsis

Back 58

While sepsis can lead to jaundice, jaundice as the only sign of sepsisis rare.Breastfeeding offers some protection against infection, particularlyearly on when colostrum provides preformed antibodies, cells, andother anti-infective substances.

Front 59

TORCH infection

Back 59

In utero exposure to one of the TORCH infections can lead to jaundice.Physical findings may include hepatosplenomegaly, microcephaly,and/or rash.

Front 60

Gilbert syndrome

Back 60

Gilbert's syndrome (reduced activity of the enzymeglucuronyltransferase) is a relatively common cause of harmlessjaundice (~5% of the population).Final diagnosis usually does not occur until later in life, when it isfound that hyperbilirubinemia persists, with no other abnormalities.

Front 61

Crigler Najjar syndrome

Back 61

Due to the absence or low levels of UDP glucuronosyltransferase 1family, polypeptide A1.Can cause severe (type I) or mild/moderate (type II) jaundice.Also very rare.

Front 62

Tests to evaluate neonatal hyperbili?

Back 62

• maternal ABO & Rh screen for isoimmune Abs
  
• infant ABO and Rh typing, DAT
  
• G6PD screen
  
• TSB
  
• direct bili level
  
• CBC or HgB level
  
• retic count and blood smear
  
• neonatal screen
  
• Cultures, LP, CRP, CBC for sepsis
  
• TORCH screen
  

Front 63

Maternal ABO andRh typing andscreen for unusualisoimmuneantibodies

Back 63

During prenatal testing, this test identifies an Rh-sensitized motherwho could put the fetus at risk for Rh-isoimmune disease.

Front 64

Infant (cord blood)ABO and Rh typing,and direct Coombs'test

Back 64

When mother is Rh-negative (or prenatal testing has not beendone).Saving a sample of cord blood is encouraged for future testing ofblood type and Coombs' (particularly when the mother's blood typeis Group O).

Front 65

G6PD screen

Back 65

When the family history, the ethnic or geographic origin, or the timeof the onset of jaundice suggests the possibility of G6PD deficiency(particularly if late-onset jaundice).Similar criteria can be used to justify testing of an infant whosejaundice is caused by other specific hemolytic disorders.

Front 66

Total serum bilirubin(TSB)

Back 66

Infant has dark urine or light stools.Persistent jaundice (> 3 weeks).Infant is ill (there will be an increased direct bilirubin withsepsis/congenital infection)

Front 67

CBC/HgB level

Back 67

If there is a suspicion of hemolytic disease or anemia (e.g.,jaundice in the first day of life or TSB >14 mg/dL in the first 48hours).If anemia is found, an elevated reticulocyte count would be furtherevidence of hemolysis (some might obtain a reticulocyte count withthe CBC).

Front 68

Reticulocyte count and blood smear

Back 68

Consider if infant is anemic or there is a strong clinical suspicion ofhemolytic disease other than isoimmunization.

Front 69

Neonatal screening

Back 69

All infants need to have a neonatal screen.There is no uniform screening program throughout the U.S.; specific disorders tested by the screen vary from state to state.There is a movement to try to establish standards that specify thenumber and types of disorders that are screened.

Front 70

Tests for sepsis

Back 70

If the jaundiced infant is ill or has other clinical signs suggestingpossible infection.Jaundice as the only sign of sepsis would be rare.

Front 71

TORCH screen

Back 71

TORCHS titres test for congenital infections: TOxoplasmosis; Rubella; Cytomegalovirus; Herpes; andSyphilis.Obtain if the maternal history or infant's physical exam and clinicalcourse suggested a congenital infection

Front 72

Managing jaundice in the breastfed infant

Back 72

do NOT stop breastfeeding

(can do temporary stop)

Front 73

Management of chemical transfer into breast milk

Back 73

E.g. T3s

Codeine

Front 74

Supplementing with vitD

Back 74

supplement breastfeed infants within days of birth

Front 75

Iron supllementation

Back 75

infants exclusively breastfed should be started on iron rich foods

most formula has iron fortification

Front 76

Fluoride supplementation

Back 76

receive fluoride after 6 months if water supply lacks fluoride

Front 77

How to manage persistent jaundice?

Back 77

if jaundice at 2 weeks, or more, o check for either dark urine or acholicappearingstools that might signify the development of cholestasis.It is also reasonable to obtain total and direct bilirubin levels (also known as "fractionatedbilirubin") to be sure that the direct bilirubin is not beginning to climb.An increasing direct bilirubin at this age would lead to a new differential diagnosis thatincludes conditions such as biliary atresia and alpha-1 antitrypsin deficiency.