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My baby still appears jaundice at one month
1. My baby still appears
jaundice at 1 month old
Chris Cheung
MBBS V O&G Rotation 5
2. Causes of neonatal jaundice
<24 hours of age 24 hours to 2 weeks of age > 2 weeks of age (persistent
neonatal jaundice)
Congenital infection
Hemolytic disorders
• Rhesus incompatibility
• ABO incompatibility
• G6PD deficiency
• Spherocytosis
• Pyruvate kinase deficiency
Physiological jaundice
Breast milk jaundice
Dehydration
Infections (esp. UTI)
Rarer causes: Crigler-Najjar
syndrome
Unconjugated:
- Breast milk jaundice
- Infection (esp. UTI)
- Hypothyroidism
- Hemolytic anemia
- High GI obstruction (e.g.
pyloric stenosis)
Conjugated:
- Bile duct obstruction (biliary
atresia, choledochal cyst)
- Neonatal hepatitis
3. Clinical assessment of persistent
neonatal jaundice
History
Onset of jaundice
Dietary and bowel habits
Urine and stool color
Any gestation problems
Any other symptoms of the baby
Fever
Respiratory distress
Family history
4. Clinical assessment of persistent
neonatal jaundice
Physical examination
Vitals: respiratory rate, temperature
Petechiae?
Hepatosplenomegaly
Assess severity of jaundice:
Press skin to assess jaundice
Progresses from head to limbs
Assess urine
Dark color?
Purulent?
5. Laboratory investigations for persistent
neonatal jaundice
Hematology:
ABO + Rh testing
CBC: look for anemia and infections
Direct Coombs Test
LFT
Clotting profile
Bilirubin measurements:
Ways to measure:
Mild: transcutaneous bilirubinometry
Moderate to severe: Serum bilirubin
Look for: bilirubin fractions and levels
6. Imaging in persistent neonatal jaundice
USG: liver and bile ducts
Radionuclide scanning (HIDA):
Indicated if extrahepatic biliary atresia is suspected
7. Neonatal liver disease
URGENCY to diagnose – early diagnosis and management improves prognosis
Persistent neonatal jaundice
Raised conjugated bilirubin (>20 umol/L)
Common clinical features:
Pale stool
Dark urine
Bleeding tendency
Failure to thrive
8. Biliary atresia
Normal birth-weight but fails to thrive as disease progresses
Clinical:
Picture of obstructive jaundice
Hepatosplenomegaly
Ultrasound features:
Triangular cord sign
Large hepatic arterial caliber
Gall bladder ghost triad:
Atretic gallbladder, length less than 19 mm
Irregular or lobular contour
Lack of smooth/complete echogenic mucosal lining with an indistinct wall
9. Kasai classification
Type I: obliteration of common bile duct
(patent cystic and common hepatic duct)
Type II
IIa: obliteration of common hepatic duct
(patent cystic and common bile duct),
sometimes with a cyst at hilum hence
termed cystic biliary atresia
IIb: obliteration of common hepatic duct,
cystic and common bile duct
Type III: obliteration of left and right
main hepatic ducts at the level of porta
hepatis (most common, 90%)
10. Kasai procedure
Surgical bypass of the fibrotic ducts +
hepatoportoenterostomy
Success rate ~80% if done within 60 days
after birth
Success rate declines with increasing age
Liver transplant if failed procedure
Main post-operative complications:
Malabsorption of fats and fat-soluble
vitamins
Cholangitis
11. Take-home message
Conjugated and unconjugated causes of persistent neonatal jaundice
Identify causes through multi-system clinical features and laboratory tests
Counsel parents of prognosis
12. References
Illustrated textbook of Paediatrics 2012 - P.168-171, P.353-355
Humphrey TM, Stringer MD. Biliary atresia: US diagnosis. Radiology. 2007;244 (3): 845-51.
doi:10.1148/radiol.2443061051
Lee MS, Kim MJ, Lee MJ et-al. Biliary atresia: color doppler US findings in neonates and
infants. Radiology. 2009;252 (1): 282-9. doi:10.1148/radiol.2522080923
Kanegawa K, Akasaka Y, Kitamura E et-al. Sonographic diagnosis of biliary atresia in pediatric
patients using the "triangular cord" sign versus gallbladder length and contraction. AJR Am J
Roentgenol. 2003;181 (5): 1387-90. AJR Am J Roentgenol
Tan Kendrick AP, Phua KB, Ooi BC et-al. Biliary atresia: making the diagnosis by the
gallbladder ghost triad. Pediatr Radiol. 2003;33 (5): 311-5. doi:10.1007/s00247-003-0867-z
Lee HJ, Lee SM, Park WH et-al. Objective criteria of triangular cord sign in biliary atresia on
US scans. Radiology. 2003;229 (2): 395-400. doi:10.1148/radiol.292020472
Sinha CK, Davenport M. Biliary atresia. J Indian Assoc Pediatr Surg. 2008;13 (2): 49-56.
doi:10.4103/0971-9261.43015
Editor's Notes
Congenital infection: bilirubin is conjugated and the infants have other abnormal clinical signs, such as growth restriction, hepatosplenomegaly
Rhesus hemolytic disease – usually identified antenatally and monitored and treated if necessary
ABO incompatibility – nowadays more common, most ABO antibodies are IgM and they cannot go across the placenta, but some group O women have an IgG anti-A-hemolysin in the blood which can cross the placenta and hemolyse the red cells of group A infant. However, it is less severe than Rhesus disease
Neonatal hepatitis syndrome: can be caused by inborn errors of metabolism, such as a1-antitrypsin deficiency, cystic fibrosis, congenital infections
Gestation: lower treatment threshold if baby is pre-term
Gestation: lower treatment threshold if baby is pre-term
Usually, a total serum bilirubin level test is the only one required in an infant with moderate jaundice who presents on the typical second or third day of life without a history and physical findings suggestive of a pathologic process. Measurement of bilirubin fractions (conjugated vs unconjugated) in serum is not usually required in infants who present as described above. However, in infants who have hepatosplenomegaly, petechiae, thrombocytopenia, or other findings suggestive of hepatobiliary disease, metabolic disorder, or congenital infection, early measurement of bilirubin fractions is suggested. The same may apply to infants who remain jaundiced beyond the first 7-10 days of life, and to infants whose total serum bilirubin levels repeatedly rebound following treatment.