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Neonatal Jaundice

R2 Topic Presentation September 2012
by

Janet Ferguson

on 2 August 2014

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Transcript of Neonatal Jaundice

Janet Ferguson
PGY2 FRCPC-EM

Jaundice in Newborns
After this talk you should be able to...

generate a differential diagnosis for infants with jaundice
know the appropriate initial investigations to order for infants with jaundice
know the risk factors for severe hyperbilirubinemia
know when and how to treat hyperbilirubinemia
Objectives
MOST COMMON
Why should YOU care?
Heme Protein
Pathophysiology
Kernicterus
Acute bilirubin encephalopathy
Chronic bilirubin encephalopathy
Severe hyperbilirubinemia: TSB >340 µmol/L
Critical hyperbilirubinemia: TSB > 425 µmol/L
Definitions
CAUSES of hyperbilirubinemia
jaundice present <24 hrs or > 2 weeks
sibling who received phototherapy
pre-term infants <37 weeks
pre-discharge bilirubin in the "high-risk" zone
significant birth trauma (cephalohematoma)
east asian race
blood group incompatibility
exclusive breast feeding
maternal gestational diabetes

Source: Maisels MJ, Baltz RD, Bhutani V, et al. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 2004;114:297–316.
RisK FACTORS FOR SEVERE HYPERBILIRUBINEMIA
Biliverdin + Iron + CO
Heme Oxygenase
Unconj. Bilirubin + Albumin
Biliverdin Reductase
Ligandin
Unconj. Bilirubin
Glucuronic Acid
UDPGT
Conj. Bilirubin
B-glucuronidase
Unconj. Bilirubin
"glucuronyl transferase"
Conjugated
*Unconjugated*
Initial evaluation
Pre-natal history:
HISTORY
Physical examination
Looks Well:
Total and Fractionated Serum Bilirubin
Plot and proceed accordingly

Eventually....
Transcutaneous Bilirubinometer!
Investigations
Phototherapy
isomerization of bilirubin to a soluble form that can be excreted in bile and urine
TREATMENT

First nations female infant born at 38 5/7 weeks via SVD with vaccuum assist to 19 yo G1P1 mother, no complications in pregnancy, blood group A pos,
Discharged on day 2, presents to the ED on day 4 of life because mother is having trouble with the breastfeeding latch and is worried about jaundice.
Baby's level at 24 hrs on post-partum was 100 umol/L.
Your experienced peds nurse states, "the baby is fine, it's not jaundiced at all"
Cases
SUMMARY
Neonatal jaundice is common.
Chronic bilirubin encephalopathy, although rare, is irreversible.
The majority of cases will be due to physiologic and breast feeding jaundice.
Other etiologies should be considered based on history, physical exam and results of investigations (let conjugated vs unconjugated bilirubin levels guide your differential)
Visual estimation of bilirubin levels are not accurate!
Watch for transcutaneous bilirubinometers in the near future.
Risk factors for severe hyperbilirubinemia include jaundice within 24 hours or > 2 weeks, pre-term infants <37 weeks, sibling who received phototherapy, exclusive breast-feeding, east Asian descent, maternal diabetes, blood group incompatibility, significant birth trauma, pre-discharge bilirubin in the high zone.
Use total serum bilirubin levels on the nomograms to guide therapy.
Mainstays of treatment of unconjugated hyperbilirubinemia are phototherapy, IVIg (in iso-immune hemolytic disease), and exchange transfusion.
Questions?
*>20% of total serum bilirubin
"always pathologic"
think cholestasis
IRREVERSIBLE
*PREVENTABLE
http://neuropathology-web.org/chapter3/chapter3eBilirubinencephalopathy.html
glowm.com
http://pediatrics.aappublications.org/content/suppl/2005/11/04/116.5.1226.DC1/term_infant_kernicterus-1.mov
Exchange Transfusion
indications: as per nomogram, >425 umol/L or if clinical signs of acute bilirubin encephalopathy
Other:
IvIg
- 500 mg/kg - 1g/kg, indicated in iso-immune hemolytic disease, has been show to greatly decrease need for exchange transfusion
Phenobarbital
- increases ligandin concentrations to promote uptake of bilirubin into hepatocytes for conjugation, increases glucuronyl transferase activity, not routinely used
Synthetic Metalloporphyrins (SnMP)
- inhibits heme oxygenase and therefore formation of bilirubin, not FDA approved,
bilitool.org
Birth History:
HPI:
CHALLENGING: "The prevention, detection and management of jaundice in otherwise healthy term and late preterm newborn infants remains a challenge..." (CPS Position Statement, 2007)
JAUNDICE = COMMON
KERNICTERUS=RARE
INCREASED PRODUCTION
HEMOLYSIS
isoimmune-mediated (ABO/Rh)
rbc membrane defects (sphero/ellipto-cytosis)
erythrocyte enzyme defects (G6PD and PK deficiency)
sepsis
polycythemia (delayed cord clamping, maternal diabetes)
birth trauma (cephalohematoma)
Family History:
WELL VS UNWELL
Pallor
Jaundice (will start cephalad and move caudad)
Mucous Membranes
Cephalohematoma/Bruising
In Severe Hemolysis > Heart Failure
2 big (Heart, Liver)
2 fast (HR, RR)
Splenomegaly
Neuro Exam:
Level of Consciousness
Cry
Tone
Gross motor movement
Primitive Reflexes
Looks Unwell or Risk Factors Present:
Febrile? Full Septic Workup

If afebrile:
CBC
Total and Fractionated Serum Bilirubin
DAT
Mom and Baby: Blood Group and Rh status
Peripheral Blood Smear
Reticulocyte count
G6PD deficiency screen (if Asian, African, Middle Eastern or Mediterrenean background)
Thyroid function tests

If conjugated bilirubin high:
Liver Enzymes
Liver Function (Albumin, Coags)
Urinalysis and Urine Culture
+/- ultrasound
Population:
healthy infants >35 weeks (14,000 in each group)
Intervention:
TcB program in the neonatal nursery and in the community by public health nurses
Comparison:
TSB levels in the severe, extreme and hazardous ranges, number of TSB draws, phototherapy rate, age of readmission for phototherapy, duration of phototherapy readmission,
Outcome:
incidence of severe neonatal hyperbilirubinemia and measures of laboratory, hospital, and nursing resource utilization.
Pediatrics, January 2012
Transcutaneous Bilirubinometry coming soon to these locations!
NICU*
POST-PARTUM
PUBLIC HEALTH NURSES
Healthy and home program
Post-partum, 24 hours old, TSB draw on ALL babies
Healthy and Home PHN, day 0-4, TSB draw if clinically suspicious
Results to family doctor, if high, call to Pediatrician on call, decides if re-admission or workup in ED more appropriate
It's all about the nomograms...
IMPAIRED CONJUGATION
decreased activity of glucuronyl transferase
Gilbert's Syndrome
Crigler-Najjar Syndrome
maternal diabetes
congenital hypothyroidism
galactosemia*
INCREASED ENTEROHEPATIC CIRCULATION
breastfeeding jaundice (because of decreased intake, slowed intestinal transit time)
breast milk jaundice
impaired intestinal motility (obstruction eg. pyloric stenosis)
Limitations of TcB:
-unreliable after initiation of phototherapy
-may be unreliable with changes in skin color and thickness
-more accurate at lower levels
OBSTRUCTION
biliary atresia, hepatitis
INFECTION
urinary tract infxns, TORCH
METABOLIC/GENETIC
galactosemia, cystic fibrosis, alpha-1 antitrypsin (AAT) deficiency, tyrosinemia, fructosemia, glycogen storage disease type IV (Andersen disease), lipid storage diseases (Niemann-Pick disease, Gaucher's disease, Wolman's disease), cerebrohepatorenal syndrome (Zellweger syndrome), familial idiopathic cholestasis (Byler's disease), hemochromatosis, idiopathic hypopituitarism, hypothyroidism
TOXIC
medications, TPN
MOST COMMON (source: uptodate.com)
Extrahepatic biliary atresia
Idiopathic neonatal hepatitis
Infectious hepatitis
Alpha-1-antitrypsin deficiency
Alagille syndrome
Progressive familial intrahepatic cholestasis
Parenteral nutrition-associated
SCREENING
PHOTOTHERAPY
EXCHANGE TRANSFUSION
"CPS POSITION STATEMENT: Guidelines for detection, management and prevention of hyperbilirubinemia in term and late preterm newborn infants"
KJ Barrington, K Sankaran; Posted: Jun 1 2007 Reaffirmed: Feb 1 2011

"Neonatal Jaundice". M. Jeffrey Maisels. Pediatrics in Review Vol. 27 No. 12 December 1, 2006 pp. 443 -454

"Impact of a Transcutaneous Bilirubinometry Program on Resource Utilization and Severe Hyperbilirubinemia"
Stephen Wainer, Seema M. Parmar, Donna Allegro, Yacov Rabi and Martha E. Lyon Pediatrics Vol. 129 No. 1 January 1, 2012 pp. 77 -86

uptodate articles:
Pathogenesis and etiology of unconjugated hyperbilirubinemia in the newborn
Approach to neonatal cholestasis
Treatment of unconjugated hyperbilirubinemia in term and late preterm infants
Evaluation of unconjugated hyperbilirubinemia in term and late preterm infants
Causes of neonatal cholestasis
Clinical manifestations of unconjugated hyperbilirubinemia in term and late preterm infants

Dynamed: "Neonatal hyperbilirubinemia"

Emedicine: "Neonatal jaundice" http://emedicine.medscape.com/article/974786-overview

Rosen's Emergency Medicine
Chapter 170 Pediatrics> Gastrointestinal Disorders

Tintinalli's Emergency Medicine: A Comprehensive Study Guide
Chapter 111. Neonatal Emergencies and Common Neonatal Problems> GI Tract Symptoms >Jaundice (Hyperbilirubinemia)
Sources
Def'n: TSB >95th percentile on the hour-specific Bhutani nomogram
normally peaks on day 2-4
focus of today's talk
Question 1: Do you believe your nurse?
Question 2: You decide to draw a total serum bilirubin level. The baby is now 84 hours old and your level is 300 umol/L. What is your next step in management?
Case 1
Asian male infant born at 36 5/7 weeks via C-section to 32 yo G3P2 mother, no complications in pregnancy, blood group O neg, formula fed.
Discharged on Day 4 of life, noted to be jaundiced in hospital, mother lost bilirubin nomogram and can't remember what the levels were.
Presents to ED on Day 6 of life because not feeding well and looks like he is more jaundiced. He has only had 2 wet diapers today.
Case 2
Greek male infant born at 40 4/7 weeks via cervidil induction and subsequent vaginal delivery, vacuum + forceps + episiotomy, to a 27 yo G2P2 mother, blood group B pos, pregnancy complicated by gestational diabetes controlled with diet, PROM with intrapartum maternal fever, breast and bottle feeding
Discharged on day 3 of life, mother states 24 hour bilirubin was in the intermediate zone. Healthy and Home nurse visit on day 4 of life and re-draws bilirubin. Mother receives a phone call 4 hours later that the levels are high and she needs to come in for an assessment.
Your nurse comes out after her initial assessment and says the infant is "full of bruises" and that it "must have a been a rough delivery". Also notes a temperature to be 37.9.
Case 3
Caucasian female infant born at 37 3/7 weeks via SVD to a 29 year old G2P1 mother, no complications in pregnancy, blood group O pos.
Discharged 26 hours after birth, mother states 24 hour level was in "low-risk" zone, no concerns from her family physician at the 2 week follow up, exclusively breastfeeding.
Presents to ED when baby is 3 weeks old because mother-in-law came for a visit and says the baby looks yellow. Mother insists the baby is fine.
case 4
Question 1: Name 2 risk factors for severe hyperbilirubinemia, other than the ones listed in the stem.
Question 2: You decide to draw a total serum bilirubin level. The baby is now 150 hours old and your level is 375 umol/L. What is your next step in management?
Question 1: What is your initial approach to management of this infant?
Question 2: You decide to draw a total serum bilirubin level. The baby is now 100 hours old and your level is 260 umol/L. What is your next step in management?
Question 1: What investigations would you like to order?
Question 2: You diagnose breast-milk jaundice after your panel of blood work comes back normal and your serum bilirubin is noted to be 200 umol/L. The patient's mother is asking you if she should stop breast-feeding?
Thanks!
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