Neonatal Jaundice Joel Cadrin MD Candidate 2016, French Stream CHEO ~ Children’s Hospital of...
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Transcript of Neonatal Jaundice Joel Cadrin MD Candidate 2016, French Stream CHEO ~ Children’s Hospital of...
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Neonatal JaundiceJoel Cadrin
MD Candidate 2016, French StreamCHEO ~ Children’s Hospital of Eastern Ontario
Wednesday, June 3, 2015
Case Study
A full term male infant, born by Emergency Caesarean section following failed forceps-assisted delivery, is now 4 days old and is ready for discharge.
His birth weight was 3.3kg. He is being breastfed every 3 to 4 hours, which seems to be going well. This is the mother’s second child. The family is of Greek origin. You are called, because, on exam, the bedside nurse noticed the baby to be jaundiced. His mother thinks she noticed the jaundice at around 24 hours of life, but isn’t certain. It appears to be worse today. His weight is now 3.1kg. He is alert on exam. You notice a Band-Aid on his right heel. Jaundice is visible on his head, trunk, legs and sclerae. You can palpate his liver 2 cm below the right costal margin, and the spleen seems palpable. How do you approach this program?
Reflections
What particular characteristics do you find pertinent in this case?
How you would begin to approach this case?
Remember to keep this case study in mind as we proceed through the presentation
Objectives
To review the physiology of bilirubin metabolism and recognize the significance of jaundice in newborn infants.
To explore the differential diagnosis of jaundice according to timing of occurrence.
To review the important clues on history and important physical findings (antenatal, perinatal and postnatal) to assist with diagnosis
To elaborate an appropriate plan of management and discuss the currently available therapies
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Objective #1Review the physiology of bilirubin
metabolism and recognize the significance of jaundice in newborn infants.
Physiological Mechanism of Bilirubin Metabolism
UGT1A1 and Conjugation of Bilirubin
Uridine diphospate glucuronosyl transferase (UGT1A1) conjugates the unconjugated bilirubin in the liver
At 30 - 40 weeks’ gestation UGT1A1 is at ~1% of adult levels
At 14 weeks of age, the levels rise to adult concentration
The Pathways of Conjugated Bilirubin
Excreted into the intestine through the gallbladder and bile duct, and follows one of two following paths:
Pathway 1: Bilirubin excreted with the stool
Pathway 2: Bilirubin may be de-conjugated by bacteria and then reabsorbed into the blood via the enterohepatic circulation
Jaundice
What is jaundice?
Bilirubin deposition in skin and mucous membrane
Results in yellow color on the skin and mucous membranes
Cephalocaudal progression
Significance of Neonatal Jaundice
May be indicative of physiologic or pathologic origins
Most cases: Bilirubin deposition and according jaundice has little consequence on the neonate
However, there is potential for severe consequences as bilirubin may cross the BBB
Kernicterus
Severe jaundice may lead to kernicterus
Kernicterus is the staining of the basal ganglia with bilirubin
A rare, but preventable cause of athetoid cerebral palsy, and other neurological pathologies
Significance of Neonatal Jaundice
Factors to consider:
Many causes of jaundice
Timing of onset
Rate of rise of bilirubin
Maximum levels of bilirubin
Persistent jaundice
Acute Symptoms of Kernicterus
Lethargy
Hypotonia
Poor Suck
Hypertonia of extensor muscles (retrocolis, opisthotonus)
High-pitched cry
Fever
Seizures
Comas
Chronic Symptoms of Kerniterus
Athetoid cerebral palsy
Seizures
Developmental delay
Hearing deficits
Oculomotor disturbances
Dental Dysplasia
Mental deficiency
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Objective #2Explore the differential diagnosis of
jaundice according to timing of occurrence.
Differential diagnosis of jaundice according to timing
of occurrence
The differential diagnoses in neonatal jaundice has much to do with the timing of the onset of the jaundice.
Timing can indicate physiologic vs. pathologic jaundice
Timing can indicate the presence of unconjugated vs. conjugated bilirubin in the system
DDx
Hemolytic Disease: Isoimmune: ABO, Rh, Minor group incompatibility Structural or RBC abnormalities (Spherocytosis,
Glucose-6-Phosphate Dehydrogenase Deficiency) Hereditary of defects in bilirubin conjugation (Gilbert
Syndrome, Crogler-Najjar Syndrome)
DDx
Bacterial Sepsis
Breastfeeding jaundice
Breast milk jaundice
Congenital biliary atresia
Extrahepatic Biliary Obstruction
Neonatal Hepatitis (Bacterial, Viral, Not Specific)
Onset of Jaundice
Specific Timings of Onset to Consider:
First 24h of life
Between 24h to 2 weeks of age
After 2 weeks of age
Reflections
What do you think is significant about timing in the DDx of neonatal jaundice?
How do you think jaundice before 24h of life differs from jaundice after the 24h mark?
Requires IMMEDIATE Attention
Hemolysis
Rh incompatibility: Identified antenatally. Hydrops, anemia, Hepatosplenomegaly
ABO incompatibility: O women have IgG anti A or B that cross placenta
G6PD Deficiency: Mediterranean, Asian, Middle-Eastern, African American
Hemorrhage and Bruising
Sepsis
Congenital Infection
Bilirubin is conjugated & abnormal clinical signs
Growth restriction
Hepatosplenomegaly,
Thrombocytopenia purpura
First 24h of Life
Between 24h to 2 Weeks
Physiological jaundice (usually most noticeable around days 2-5)
Breast milk jaundice
Infection (UTI)
Hemolysis
Bruising
Polycythemia
Crigler-Najjar Syndrome (UTB1A1 deficiency)
After 2 Weeks of Age
Unconjugated
Physiological or breast milk jaundice
Infection (UTI)
Hypothyroidism
Hemolytic Anemia
High GI obstruction (i.e. pyloric stenosis)
Conjugated
Bile duct obstruction
Neonatal hepatitis
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Objective #3Review the important clues on history and
important physical findings (antenatal, perinatal and postnatal) to assist with
diagnosis
History
Newborn history
Delivery history
Risk Factors for Hyperbilirubinemia PreTerm Previous sibling with severe hyperbilirubinemia Bruising Cephalhematoma Male Asian or European background Dehydration Maternal blood type
Signs of cholestasis: Acholic stools, dark urine, weight loss
Back to the Case
A full term male infant, born by Emergency Caesarean section following failed forceps-assisted delivery, is now 4 days old and is ready for discharge.
His birth weight was 3.3kg. He is being breastfed every 3 to 4 hours, which seems to be going well. This is the mother’s second child. The family is of Greek origin. You are called, because, on exam, the bedside nurse noticed the baby to be jaundiced. His mother thinks she noticed the jaundice at around 24 hours of life, but isn’t certain. It appears to be worse today. His weight is now 3.1kg. He is alert on exam. You notice a Band-Aid on his right heel. Jaundice is visible on his head, trunk, legs and sclerae. You can palpate his liver 2 cm below the right costal margin, and the spleen seems palpable. How do you approach this program?
Pertinent points in the history
Full term : lower risk
Male sex: increased risk
Emergency C-Section following failed forceps: Trauma (cephalhematoma?)
4 days old and ready for discharge: 96 hours old
Breastfed every 3-4 hours, going well: exclusive breastfeeding
Greek origin: higher risk for G6PD deficiency in people of Mediterranean descent
Physical Exam
What should we be examining for?
Where would you start your physical exam in neonatal jaundice?
Physical Exam
Vital Signs
General appearance
Measurements
Respiratory, CVS
Abdo
Neuro
Findings on Physical Exam
Vital Signs are normal
Birth Weight was 3.3 kg, now 3.1 kg: Weight loss <10%
At Day 4, nurse finds baby to be jaundiced: 96 hours
Mother thinks it began at 24 h of life: Significance of 24h
Baby is on alert on exam, no abnormal neurological signs
Jaundice visible on head, trunk, legs, sclera: Moves in cephalocaudal direction
Band-Aid on right heel: metabolic screening underway
Hepatosplenomegaly : sign of possible intrauterine infection, Rh incompatibility (extravascular hemolysis)
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Objective #4To elaborate an appropriate plan of
management and discuss the currently available therapies
Plan of Management
When to measure bilirubin concentration? CPS recommendations
How do we predict hyperbilirubinemia?
How does phototherapy lower unconjugated bilirubin levels?
When should we consider exchange transfusions?
Investigations
TSB (or Transcutaneous measurement if available) Universal screening
CBC, Reticulocytes, Film, LDH, Haptoglobuline, DAT, Blood type
Electrolytes, Urea, Creatinine
LP
LFTs
Ultrasound (hepatobiliary anatomy)
CXR
Metabolic screen
Guideline 1
To be used to determine if phototherapy , follow up or if routine care is required.
Direct antiglobulin test (Coombs test) should be performed in infants with early jaundice and mother of blood group O in order to identify risk group (presently, in Ottawa this is done for all O mothers)
Guideline 2
To be used to determine if intensive phototherapy is required.
Guideline 3
Used to determine if exchange transfusion required
Immediate exchange tranfusion is recommended if signs of acute bilirubin encephalopathy (hypertonia, arching, retrocolis, fever, high pitched cry)
Available Therapies
Breastfeeding support programs
Phototherapy
Supplemental fluids (PO,IV)
IVIg
Exchange transfusion
Management of this case
If our patient’s bilirubin was measured to be 300 micromol/L, with a negative DAT (DAT performed since mother was O+), and no other risk factors
If our patient’s bilirubin was measured to be 400 micromol/L, with a positive DAT.
If our patient’s bilirubin was measured to be 350 micromol/L, with a positive DAT
Case 2
Female infant born at 39 weeks. Mother AB+
Spontaneous vaginal delivery, no complications
At 36 hours, weight is stable, baby is feeding well, she is alert on exam, she is ready for discharge.
No jaundice at present time, bilirubin at 96 micromol/L
What action is to be taken?