Table 2

Summary of recommendations and evidence base for strategies to improve outcome of patients with neonatal cholestasis

RECOMMENDATIONSTRATEGYEVIDENCE
Test at 2 weeks (fractionated bilirubin levels at 2–3 weeks of age)Early identification of neonatal cholestasisLevel III: Guidelines from professional organizations (NASPGHAN, CPS, AAP)
Application of diagnostic algorithm and early referral to pediatric gastroenterologistAccurate diagnosis of cause of cholestasis enables early initiation of treatmentLevel III: Case series, guidelines from professional organizations (NASPGHAN, CPS, AAP)
Immediate intervention to address the underlying condition if cholestasis is identifiedEarly intervention improves outcomes of biliary atresia, congenital hypothyroidism, galactosemia, tyrosinemia, congenital CMV infectionLevel II and III: Retrospective cohort studies, guidelines from professional organizations (NASPGHAN)
Further investigation of efficacy and feasibility of screening for neonatal cholestasisEarly identification of neonatal cholestasis results in early interventionLevel II: Prospective cohort studies
  • AAP—American Academy of Pediatrics, CMV—cytomegalovirus, CPS—Canadian Paediatric Society, NASPGHAN—North American Society for Pediatric Gastroenterology, Hepatology and Nutrition.