Case:
Your next patient is a 17 day old presenting for follow-up. During the visit you note that the baby’s eyes and skin are yellow. Labs show a total bilirubin of 7 mg/dL, with direct component being 5mg/dl. What are your next steps?
What is Jaundice?
- A yellowish discoloration of the skin and sclera due to elevated bilirubin levels in the blood.
- Associated findings include: pale stool, dark urine
- Becomes clinically apparent at total levels >2.5mg/dL (Fawaz et al, 2016)
- Very common finding; found in up 15% of infants at 2 week visit (Kelly et al, 1995)
What is Bilirubin?
- Breakdown product of heme metabolism
- Conjugated in the Liver
- Excreted in feces (majority) and urine
Approach to A Jaundiced Neonate (adapted from NASPGHAN guidelines; Moyer et al, 2004)
Step 1: Is the baby sick?
- Differential includes: infection (especially urinary tract), inborn errors of metabolism, acute hemolysis, etc
- Most important: manage acute illness
Step 2: What type of hyperbilirubinemia?
- Direct
- Defined as a serum conjugated bilirubin >1.0mg/dl if serum total bilirubin is <5.0 mg/dl or >20% of total bilirubin if >5 mg/dL. (Shulman et al, 2016)
Figure 1: Most Common Etiologies of Neonatal Cholestasis
Etiology | Number of Cases (Total: 1692) | % of Cases |
Idiopathic Neonatal Hepatitis | 440 | 26 |
Extrahepatic Biliary Atresia | 438 | 25.9 |
Infection | 194 | 11.5 |
Metabolic Disease | 74 | 4.4 |
Adapted from Gottesman et al, 2015
2.) Indirect Hyperbilirubinemia
- Most commonly due breast milk jaundice, though larger differential exists. Will not be discussed during this post.
Step 3: Other Labs and Studies
- Important Imaging Studies
- Ultrasound- wide estimates of sensitivity
- 73-100% for absent gallbladder and 83-100% for “triangular cord” sign depending on study cited.
- Ultrasound- wide estimates of sensitivity
- Other labs/blood to obtain
- Review Newborn Screen: Congenital hypothyroidism and galactosemia can present with cholestasis.
- GGTP, LFTs, CBC, Coags
- α-1 antitrypsin (If low, order Pi Typing)
Next Steps
- Next steps largely depend on findings of workup listed above
- As biliary atresia (BA) can cause neonatal cholestasis, this MUST be ruled out
- Outcome of patients diagnosed with biliary atresia is inversely correlated with age of diagnosis (specifically the age at which they undergo the Kasai hepatoportoenterostomy (Balistreri et al, 1996)
- If ultrasound non-diagnostic, hepatobiliary scintigrapy (HDS), magnetic resonance cholangiopancreatography (MRCP), liver biopsy may need to be undertaken to rule out BA
- Note: Gold standard is intra-op cholangiogram (Shulman et al, 2016)
Faculty Reviewer: Jason Shapiro, MD
Resident Reviewer: Meg Gibson, MD
References
- Balistreri WF et al. “Biliary Atresia: Current Concepts and Research Directions.” Hepatology. 1996;23(6)1682-1692.
- Fawaz R et al. “Guideline for the Evaluation of Cholestatic Jaundice in Infants: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition.” Journal of Pediatric Gastroenterology and Nutrition. 2016; (Published ahead of print)
- Gottesman LE, Del Vecchio MT, Aronoff SC. “Etiologies of conjugated hyperbilirubinemia in infancy: A systematic review of 1692 subjects.” BMC Pediatrics 2015; 15:192
- Kelly DA et al. “Jaundice in Babies: Implications for Community Screening for Biliary Atresia.” The British Medical Journal. 1995;310(6988)1172-1173.
- Moyer M et al. “Guideline for the Evaluation of Cholestatic Jaundice in Infants: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition.”Journal of Pediatric Gastroenterology and Nutrition. 2004;39(2)115-128
- Shulman RJ et al. “Approach to Neonatal Cholestasis.” http://www.uptodate.com/contents/approach-to-neonatal-cholestasis. Accessed July 5th, 2016.
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