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peds.biliary-atresia.v1

Biliary atresia

pediatricssubacutepediatricneonatal
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Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Confirm cholestatic jaundice: total bilirubin elevated + direct bilirubin > 1.0 mg/dL (> 17 micromol/L) in infant beyond 2 weeks of age (Fawaz NASPGHAN 2017 PMID 27429428)

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Advance rule
Set
Advance when

Direct hyperbilirubinemia confirmed + pediatric hepatology referral initiated

Patient inputs (19)

All dosing (ursodiol, fat-soluble vitamins, phenobarbital) weight-based (AAP Red Book 2024-2027, Lexicomp Peds)

Preterm biliary atresia presents later; differential of TPN cholestasis broader in NICU graduates (Fawaz NASPGHAN 2017)

Breastfeeding jaundice can mimic; persistence beyond 2 weeks always requires direct bilirubin (Fawaz NASPGHAN 2017)

Alagille syndrome (autosomal dominant JAG1 / NOTCH2), alpha-1 antitrypsin deficiency, progressive familial intrahepatic cholestasis (Fawaz NASPGHAN 2017)

CMV, syphilis, toxoplasmosis, rubella, herpes — cholestasis mimics (Fawaz NASPGHAN 2017)

Kasai window < 60 days for best native-liver survival; significant decline 60-90 days; very poor > 90 days (Hartley Lancet 2009 PMID 19914515)

Acholic / clay-colored stools red flag; stool-color cards distributed at well-child visits per NASPGHAN (Fawaz NASPGHAN 2017)

Direct bilirubin > 1.0 mg/dL (> 17 micromol/L) defines cholestasis; total bilirubin also tracks (Fawaz NASPGHAN 2017)

GGT often markedly elevated in BA; AST/ALT modest; alkaline phosphatase elevated (Fawaz NASPGHAN 2017)

Vitamin-K-dependent coagulopathy from cholestasis-induced fat malabsorption (Venkat 2014 PMID 25419594)

Anemia + thrombocytopenia from splenic sequestration if late presentation with cirrhosis (Fawaz NASPGHAN 2017)

Alpha-1 antitrypsin deficiency is differential (Fawaz NASPGHAN 2017)

Hypothyroidism can cause cholestasis (Fawaz NASPGHAN 2017)

UTI can cause cholestasis (Fawaz NASPGHAN 2017)

CMV PCR, RPR, toxoplasmosis IgM, rubella IgM, HSV PCR if vesicular lesions (Fawaz NASPGHAN 2017)

Cystic fibrosis can cause cholestasis (Fawaz NASPGHAN 2017)

Triangular cord sign, absent or abnormal gallbladder, polysplenia syndrome — first-line imaging (Hartley Lancet 2009)

Phenobarbital priming 5 mg/kg/day PO × 5 days then HIDA scan; no excretion into bowel by 24 h supports biliary atresia (Hartley Lancet 2009)

Bile-duct proliferation, fibrosis, bile plugs — histologic confirmation (Fawaz NASPGHAN 2017)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (9)

9 need judgement
  • informationallife_threateningkasai_window_under_60_days
    Suspected biliary atresia with age < 60 days — TIME-CRITICAL Kasai window for best native-liver survival (Hartley Lancet 2009 PMID 19914515)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateninglate_presentation_with_cirrhosis
    Late presentation with established cirrhosis features (ascites, splenomegaly, coagulopathy, hypoalbuminemia, portal hypertension) — primary transplant pathway (Hartley Lancet 2009)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepersistent_jaundice_over_2_weeks_infant
    Persistent jaundice in infant > 2 weeks of age (Fawaz NASPGHAN 2017 PMID 27429428 — mandates direct bilirubin evaluation)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveredirect_hyperbilirubinemia_confirmed
    Direct bilirubin > 1.0 mg/dL (> 17 micromol/L) in infant — cholestatic jaundice confirmed (Fawaz NASPGHAN 2017)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverekasai_60_to_120_days_window_closing
    Suspected biliary atresia with age 60-120 days — Kasai window closing; outcomes worse but Kasai still recommended; consider primary transplant pathway (Hartley Lancet 2009)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepost_kasai_cholangitis_concern
    Post-Kasai fever + change in stool color + LFT elevation — cholangitis concern (leading post-Kasai complication per Hartley Lancet 2009)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverefailed_kasai_transplant_acceleration
    Total bilirubin remains elevated at 3-6 mo post-Kasai OR progressive cirrhosis post-Kasai — failed Kasai → transplant pathway (Hartley Lancet 2009)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatefat_soluble_vitamin_deficiency_concern
    Post-Kasai persistent cholestasis with concern for fat-soluble vitamin deficiency — total bilirubin elevation parallels vitamin deficiency (Venkat 2014 PMID 25419594)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmildavoid_routine_post_kasai_steroids
    Routine post-Kasai steroids — Alonso START 2018 PMID 30244988 showed no benefit + significant growth impairment (length, weight, head circumference) for > 6 mo post-Kasai
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

Biliary atresia — Kasai hepatoportoenterostomy < 60 days + post-Kasai medical adjuncts (Fawaz NASPGHAN 2017 PMID 27429428; Hartley Lancet 2009 PMID 19914515)
axis: peds_biliary_atresia_surgical_mainstay_plus_medical_adjuncts
Selected axis "Biliary atresia — Kasai hepatoportoenterostomy < 60 days + post-Kasai medical adjuncts (Fawaz NASPGHAN 2017 PMID 27429428; Hartley Lancet 2009 PMID 19914515)" by default fallback (first axis)
  • Kasai hepatoportoenterostomy < 60 days of life — surgical mainstay
    first line
    pediatric_hepatobiliary_surgery
    Surgical procedure < 60 days of life ideally; performed at pediatric liver center with high case volume
    triggers: confirmed_biliary_atresia_age_under_60_days_to_90_days, no_contraindications_to_surgery
    Hartley Lancet 2009 PMID 19914515 — Kasai < 60 days associated with best native-liver survival (~ 80% reach adolescence with successful drainage); 60-90 days significant decline; > 90 days very poor; performed at high-volume pediatric liver center
  • ursodiol
    first line
    bile_acid_choleresis
    10-20 mg/kg/day PO divided BID-TID, may titrate up to 30 mg/kg/day if tolerated • PO • BID-TID (max: max 30 mg/kg/day per AAP Lexicomp Peds)
    triggers: post_Kasai_cholestasis_continued_OR_unresectable_cholestasis
    Hartley Lancet 2009 PMID 19914515 + Fawaz NASPGHAN 2017 PMID 27429428 — ursodeoxycholic acid for choleresis post-Kasai (also used in other neonatal cholestatic syndromes); improves bile flow + reduces hepatocyte injury (AAP Red Book 2024-2027, Lexicomp Peds)
    rxcui 11065
  • vitamin A (retinol) supplementation
    first line
    fat_soluble_vitamin
    5,000-25,000 IU/day PO titrate per vitamin A level • PO • daily
    triggers: post_Kasai_persistent_cholestasis_with_fat_malabsorption
    Venkat 2014 PMID 25419594 — fat-soluble vitamin deficiency parallels total bilirubin elevation; supplementation per protocol (AAP Lexicomp Peds; NASPGHAN biliary atresia nutrition guidance) — listed as non_pharm because formulations + RxCUI vary by product (retinol palmitate 11246 verified but pediatric liquid formulations differ)
  • cholecalciferol (vitamin D3)
    first line
    fat_soluble_vitamin
    800-5,000 IU/day PO titrate per 25-OH-vitamin D level (target > 30 ng/mL) • PO • daily (max: max 10,000 IU/day per AAP Lexicomp Peds)
    triggers: post_Kasai_persistent_cholestasis_with_fat_malabsorption
    Venkat 2014 PMID 25419594 — vitamin D deficiency parallels total bilirubin in biliary atresia; targets 25-OH-D > 30 ng/mL (AAP Lexicomp Peds; NASPGHAN biliary atresia nutrition)
    rxcui 2418
  • ergocalciferol (vitamin D2)
    second line
    fat_soluble_vitamin
    alternative to cholecalciferol: 50,000 IU PO once-weekly OR daily dosing per protocol • PO • daily or weekly (max: max per AAP Lexicomp Peds)
    triggers: post_Kasai_alternative_to_cholecalciferol_or_pediatric_preference
    Venkat 2014 PMID 25419594 — alternative vitamin D form; cholecalciferol generally preferred (AAP Lexicomp Peds)
    rxcui 4018
  • vitamin E
    first line
    fat_soluble_vitamin_antioxidant
    25-100 IU/kg/day PO titrate per vitamin E:total lipid ratio (TPGS form preferred for cholestasis-induced fat malabsorption) • PO • daily (max: max 100 IU/kg/day per AAP Lexicomp Peds)
    triggers: post_Kasai_persistent_cholestasis_with_fat_malabsorption
    Venkat 2014 PMID 25419594 — vitamin E deficiency parallels total bilirubin in biliary atresia; TPGS (tocopheryl polyethylene glycol succinate) form preferred for cholestatic infants (AAP Lexicomp Peds; NASPGHAN biliary atresia nutrition)
    rxcui 11256
  • phytonadione (vitamin K1)
    first line
    fat_soluble_vitamin_coagulation_factor
    2.5-5 mg PO 2-7×/week (titrate per INR); for acute coagulopathy use 0.3 mg/kg IV/IM/SC slow IV (max 10 mg single dose) • PO maintenance, IV/IM/SC acute • 2-7×/week PO; single dose IV/IM/SC for acute reversal (max: max 10 mg single dose IV per AAP Red Book 2024-2027 / Lexicomp Peds)
    triggers: post_Kasai_coagulopathy_OR_INR_elevated_OR_active_bleeding
    Venkat 2014 PMID 25419594 + Fawaz NASPGHAN 2017 — vitamin K deficiency in cholestasis causes hypoprothrombinemia + bleeding risk; oral maintenance + IV/IM/SC for acute reversal (AAP Red Book 2024-2027, Lexicomp Peds)
    rxcui 8308
  • phenobarbital
    add on
    barbiturate_hida_priming
    5 mg/kg/day PO × 5 days for HIDA scan priming ONLY (NOT for routine post-Kasai choleresis) • PO • daily × 5 days pre-HIDA (max: max 5 mg/kg/day for HIDA priming protocol; total course 5 days only)
    triggers: HIDA_scan_indicated_for_biliary_atresia_workup
    Fawaz NASPGHAN 2017 PMID 27429428 — phenobarbital priming pre-HIDA increases biliary excretion sensitivity; NOT used for routine post-Kasai choleresis (no evidence of benefit) (AAP Red Book 2024-2027, Lexicomp Peds)
    rxcui 8134
  • methylprednisolone
    contraindication substitute
    corticosteroid_AVOID_routine_use
    DO NOT USE ROUTINELY post-Kasai (Alonso START 2018 PMID 30244988 — no benefit + significant growth impairment) • PO/IV • not routinely used (max: see contraindication_rules — START trial showed no benefit + growth impairment)
    triggers: investigational_per_clinical_trial_only
    Alonso START 2018 PMID 30244988 — Steroids in Biliary Atresia Randomized Trial showed no benefit on biliary drainage + significant impairment of length, weight, head circumference growth for > 6 mo post-Kasai (especially in successful drainage subset); routine post-Kasai steroids NOT recommended
    rxcui 6902
  • liver transplant evaluation + listing if Kasai fails
    rescue
    pediatric_liver_transplant
    Refer at diagnosis to pediatric liver transplant center; list when total bilirubin remains elevated at 3-6 mo post-Kasai OR progressive cirrhosis OR Kasai not performed (late presentation > 100-120 days)
    triggers: failed_Kasai_or_late_presentation_with_established_cirrhosis_or_progressive_synthetic_dysfunction
    Hartley Lancet 2009 PMID 19914515 — liver transplantation is life-saving for failed Kasai or late-presenting biliary atresia; transplant center referral at diagnosis (regardless of Kasai plan) ensures readiness
  • trimethoprim-sulfamethoxazole post-Kasai cholangitis prophylaxis (institutional protocol)
    comorbidity specific
    cholangitis_prophylaxis
    Per institutional protocol: some centers use TMP-SMX 2-5 mg/kg/day PO BID × 6-12 months post-Kasai; evidence is limited • PO • BID
    triggers: post_Kasai_per_institutional_cholangitis_prevention_protocol
    Hartley Lancet 2009 PMID 19914515 — cholangitis is leading post-Kasai complication; prophylactic antibiotic regimen varies by center (no consensus); listed as non_pharm because regimen + RxCUI varies by institution (TMP-SMX, neomycin, others)

outpatient playbook — drug actions (1)

  1. 1. no outpatient medication initiated for suspected biliary atresia — TIME-CRITICAL referral to pediatric hepatology for inpatient workup
    N/A • N/A • N/A
    trigger: Suspected biliary atresia at outpatient visit
    Fawaz NASPGHAN 2017 — time-critical Kasai window < 60 days; refer immediately for inpatient diagnostic workup + surgical evaluation

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Persistent jaundice in infant > 2 weeks of age — direct bilirubin evaluation mandated (Fawaz NASPGHAN 2017 PMID 27429428); Acholic / clay-colored stools in infant — biliary atresia red flag (Fawaz NASPGHAN 2017; NASPGHAN stool color cards); Direct bilirubin > 1.0 mg/dL (> 17 micromol/L) in infant — cholestatic jaundice (Fawaz NASPGHAN 2017).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Biliary atresia** (peds.biliary-atresia.v1).
Phenotype framing: Biliary atresia (25-40% of neonatal cholestasis) vs Alagille syndrome (JAG1 / NOTCH2) vs progressive familial intrahepatic cholestasis 1/2/3 vs alpha-1 antitrypsin deficiency vs cystic fibrosis vs galactosemia vs tyrosinemia vs neonatal hemochromatosis vs TPN-associated cholestasis vs TORCH infections vs neonatal sepsis with cholestasis (Fawaz NASPGHAN 2017)
Scope: Confirm cholestatic jaundice: total bilirubin elevated + direct bilirubin > 1.0 mg/dL (> 17 micromol/L) in infant beyond 2 weeks of age (Fawaz NASPGHAN 2017 PMID 27429428)

No severity triggers fired against current inputs.

Plan

Regimen axis: **Biliary atresia — Kasai hepatoportoenterostomy < 60 days + post-Kasai medical adjuncts (Fawaz NASPGHAN 2017 PMID 27429428; Hartley Lancet 2009 PMID 19914515)**.
1. Kasai hepatoportoenterostomy < 60 days of life — surgical mainstay Surgical procedure < 60 days of life ideally; performed at pediatric liver center with high case volume (pediatric_hepatobiliary_surgery, first line) — Hartley Lancet 2009 PMID 19914515 — Kasai < 60 days associated with best native-liver survival (~ 80% reach adolescence with successful drainage); 60-90 days significant decline; > 90 days very poor; performed at high-volume pediatric liver center
2. ursodiol 10-20 mg/kg/day PO divided BID-TID, may titrate up to 30 mg/kg/day if tolerated PO BID-TID (bile_acid_choleresis, first line) — Hartley Lancet 2009 PMID 19914515 + Fawaz NASPGHAN 2017 PMID 27429428 — ursodeoxycholic acid for choleresis post-Kasai (also used in other neonatal cholestatic syndromes); improves bile flow + reduces hepatocyte injury (AAP Red Book 2024-2027, Lexicomp Peds)
3. vitamin A (retinol) supplementation 5,000-25,000 IU/day PO titrate per vitamin A level PO daily (fat_soluble_vitamin, first line) — Venkat 2014 PMID 25419594 — fat-soluble vitamin deficiency parallels total bilirubin elevation; supplementation per protocol (AAP Lexicomp Peds; NASPGHAN biliary atresia nutrition guidance) — listed as non_pharm because formulations + RxCUI vary by product (retinol palmitate 11246 verified but pediatric liquid formulations differ)
4. cholecalciferol (vitamin D3) 800-5,000 IU/day PO titrate per 25-OH-vitamin D level (target > 30 ng/mL) PO daily (fat_soluble_vitamin, first line) — Venkat 2014 PMID 25419594 — vitamin D deficiency parallels total bilirubin in biliary atresia; targets 25-OH-D > 30 ng/mL (AAP Lexicomp Peds; NASPGHAN biliary atresia nutrition)
5. ergocalciferol (vitamin D2) alternative to cholecalciferol: 50,000 IU PO once-weekly OR daily dosing per protocol PO daily or weekly (fat_soluble_vitamin, second line) — Venkat 2014 PMID 25419594 — alternative vitamin D form; cholecalciferol generally preferred (AAP Lexicomp Peds)
6. vitamin E 25-100 IU/kg/day PO titrate per vitamin E:total lipid ratio (TPGS form preferred for cholestasis-induced fat malabsorption) PO daily (fat_soluble_vitamin_antioxidant, first line) — Venkat 2014 PMID 25419594 — vitamin E deficiency parallels total bilirubin in biliary atresia; TPGS (tocopheryl polyethylene glycol succinate) form preferred for cholestatic infants (AAP Lexicomp Peds; NASPGHAN biliary atresia nutrition)
7. phytonadione (vitamin K1) 2.5-5 mg PO 2-7×/week (titrate per INR); for acute coagulopathy use 0.3 mg/kg IV/IM/SC slow IV (max 10 mg single dose) PO maintenance, IV/IM/SC acute 2-7×/week PO; single dose IV/IM/SC for acute reversal (fat_soluble_vitamin_coagulation_factor, first line) — Venkat 2014 PMID 25419594 + Fawaz NASPGHAN 2017 — vitamin K deficiency in cholestasis causes hypoprothrombinemia + bleeding risk; oral maintenance + IV/IM/SC for acute reversal (AAP Red Book 2024-2027, Lexicomp Peds)
8. phenobarbital 5 mg/kg/day PO × 5 days for HIDA scan priming ONLY (NOT for routine post-Kasai choleresis) PO daily × 5 days pre-HIDA (barbiturate_hida_priming, add on) — Fawaz NASPGHAN 2017 PMID 27429428 — phenobarbital priming pre-HIDA increases biliary excretion sensitivity; NOT used for routine post-Kasai choleresis (no evidence of benefit) (AAP Red Book 2024-2027, Lexicomp Peds)
9. methylprednisolone DO NOT USE ROUTINELY post-Kasai (Alonso START 2018 PMID 30244988 — no benefit + significant growth impairment) PO/IV not routinely used (corticosteroid_AVOID_routine_use, contraindication substitute) — Alonso START 2018 PMID 30244988 — Steroids in Biliary Atresia Randomized Trial showed no benefit on biliary drainage + significant impairment of length, weight, head circumference growth for > 6 mo post-Kasai (especially in successful drainage subset); routine post-Kasai steroids NOT recommended
10. liver transplant evaluation + listing if Kasai fails Refer at diagnosis to pediatric liver transplant center; list when total bilirubin remains elevated at 3-6 mo post-Kasai OR progressive cirrhosis OR Kasai not performed (late presentation > 100-120 days) (pediatric_liver_transplant, rescue) — Hartley Lancet 2009 PMID 19914515 — liver transplantation is life-saving for failed Kasai or late-presenting biliary atresia; transplant center referral at diagnosis (regardless of Kasai plan) ensures readiness
11. trimethoprim-sulfamethoxazole post-Kasai cholangitis prophylaxis (institutional protocol) Per institutional protocol: some centers use TMP-SMX 2-5 mg/kg/day PO BID × 6-12 months post-Kasai; evidence is limited PO BID (cholangitis_prophylaxis, comorbidity specific) — Hartley Lancet 2009 PMID 19914515 — cholangitis is leading post-Kasai complication; prophylactic antibiotic regimen varies by center (no consensus); listed as non_pharm because regimen + RxCUI varies by institution (TMP-SMX, neomycin, others)

Setting playbook (outpatient) — Recognize cholestatic jaundice in infant > 2 weeks (direct bilirubin > 1.0 mg/dL) per Fawaz NASPGHAN 2017, urgent referral to pediatric hepatology, prepare for inpatient workup + Kasai surgery, family education on stool color cards + jaundice red flags
12. no outpatient medication initiated for suspected biliary atresia — TIME-CRITICAL referral to pediatric hepatology for inpatient workup N/A N/A N/A — Suspected biliary atresia at outpatient visit (Fawaz NASPGHAN 2017 — time-critical Kasai window < 60 days; refer immediately for inpatient diagnostic workup + surgical evaluation)

Non-pharmacologic actions:
- Same-day or next-day pediatric hepatology referral (Fawaz NASPGHAN 2017)
- Stool color card education for caregivers (NASPGHAN)
- Jaundice education — caregivers expected to track jaundice resolution + report persistent jaundice > 2 weeks (Fawaz NASPGHAN 2017)
- Refer to pediatric liver transplant center for diagnostic workup + Kasai consideration (Hartley Lancet 2009)
- Track time from symptom onset to referral (lag time) (Hartley Lancet 2009)

AVOID / contraindication checks:
- DO_NOT_USE_ROUTINE_POST_KASAI_STEROIDS_no_benefit_growth_impairment (Alonso START 2018 PMID 30244988)
- Vitamin_K_slow_IV_anaphylactoid_risk (Lexicomp Peds)
- Vitamin_D_titrate_per_25_OH_D_avoid_toxicity_hypercalcemia (Lexicomp Peds)
- Fat_soluble_vitamin_titration_per_total_bilirubin_trend (Venkat 2014 PMID 25419594)
- Phenobarbital_NOT_for_routine_post_Kasai_choleresis_HIDA_priming_only (Fawaz NASPGHAN 2017)
- Kasai_age_window_under_60_days_best_outcome_under_90_days_acceptable_above_120_days_likely_transplant_primary (Hartley Lancet 2009)
- Any_post_Kasai_fever_requires_cholangitis_evaluation (Hartley Lancet 2009)
- Transplant_referral_at_diagnosis_regardless_of_Kasai_plan (Hartley Lancet 2009)

Monitoring

Regimen monitoring:
- Total bilirubin q1-2 wk × 3 mo post-Kasai (cessation of cholestasis is treatment goal) (Hartley Lancet 2009)
- LFTs + GGT + alkaline phosphatase serial (Hartley Lancet 2009)
- Fat-soluble vitamin levels (A, 25-OH-D, E, INR for K) per total bilirubin trend (Venkat 2014 PMID 25419594)
- Growth (length, weight, head circumference) at every visit (Alonso START 2018 PMID 30244988)
- Daily weight + nutrition + MCT-enriched formula intake (NASPGHAN biliary atresia nutrition)
- Cholangitis surveillance — fever + change in stool color + LFT elevation (Hartley Lancet 2009)
- Portal hypertension surveillance — splenomegaly + thrombocytopenia + variceal screening (Hartley Lancet 2009)
- Annual screening: AFP, ultrasound for hepatocellular carcinoma after age 1-2 yr (Hartley Lancet 2009)

Setting (outpatient) monitoring:
- Direct bilirubin trend (Fawaz NASPGHAN 2017)
- Growth + stool color + urine color at every visit (NASPGHAN)

Follow-up plan: Pediatric hepatology + transplant team; surgery; nutrition; cholangitis prevention (some centers use prophylactic trimethoprim-sulfamethoxazole post-Kasai); transplant evaluation if total bilirubin remains elevated at 3-6 mo post-Kasai or progressive cirrhosis (Hartley Lancet 2009)
- Close-out criterion: Follow-up + nutritional support + transplant evaluation plan documented

Monitoring phase: Total bilirubin trend post-Kasai (clears to normal at 3 mo in ~ 60% of Kasai successes per Hartley 2009 Lancet); fat-soluble vitamin levels per total bilirubin (Venkat 2014 PMID 25419594); growth + nutrition; signs of portal hypertension + cirrhosis; cholangitis surveillance (fever + bile-leak post-op) (Fawaz NASPGHAN 2017)

Disposition

Current setting: outpatient — Recognize cholestatic jaundice in infant > 2 weeks (direct bilirubin > 1.0 mg/dL) per Fawaz NASPGHAN 2017, urgent referral to pediatric hepatology, prepare for inpatient workup + Kasai surgery, family education on stool color cards + jaundice red flags

Disposition criteria:
- Refer urgently to pediatric hepatology / liver transplant center for inpatient diagnostic workup + Kasai evaluation (Fawaz NASPGHAN 2017)

Escalation triggers (move to higher acuity):
- Direct bilirubin > 1.0 mg/dL → urgent pediatric hepatology referral (Fawaz NASPGHAN 2017)
- Acholic / clay-colored stools → urgent pediatric hepatology referral (Fawaz NASPGHAN 2017)
- Coagulopathy / failure to thrive → ED + urgent admit (Fawaz NASPGHAN 2017)
- Age approaching 60 days with suspected BA → urgent inpatient workup + surgical evaluation (Hartley Lancet 2009)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Suspected biliary atresia with age < 60 days — TIME-CRITICAL Kasai window for best native-liver survival (Hartley Lancet 2009 PMID 19914515)
- [LIFE_THREATENING] Late presentation with established cirrhosis features (ascites, splenomegaly, coagulopathy, hypoalbuminemia, portal hypertension) — primary transplant pathway (Hartley Lancet 2009)
- [SEVERE] Persistent jaundice in infant > 2 weeks of age (Fawaz NASPGHAN 2017 PMID 27429428 — mandates direct bilirubin evaluation)

Citations

- Fawaz NASPGHAN/ESPGHAN 2017 PMID 27429428 (canonical guideline for evaluation of cholestatic jaundice in infants — biliary atresia accounts for 25-40% of neonatal cholestasis) + Hartley Lancet 2009 PMID 19914515 (biliary atresia clinical review + Kasai outcomes) + Alonso START 2018 PMID 30244988 (steroids in biliary atresia trial) + Venkat 2014 PMID 25419594 (fat-soluble vitamin deficiency monitoring) [PMID:27429428](https://pubmed.ncbi.nlm.nih.gov/27429428/)
- Cited evidence (PMID 19914515) [PMID:19914515](https://pubmed.ncbi.nlm.nih.gov/19914515/)
- Cited evidence (PMID 30244988) [PMID:30244988](https://pubmed.ncbi.nlm.nih.gov/30244988/)
- Cited evidence (PMID 25419594) [PMID:25419594](https://pubmed.ncbi.nlm.nih.gov/25419594/)

Last reconciled with current guidelines: 2026-05-26.
References
  • Fawaz NASPGHAN/ESPGHAN 2017 PMID 27429428 (canonical guideline for evaluation of cholestatic jaundice in infants — biliary atresia accounts for 25-40% of neonatal cholestasis) + Hartley Lancet 2009 PMID 19914515 (biliary atresia clinical review + Kasai outcomes) + Alonso START 2018 PMID 30244988 (steroids in biliary atresia trial) + Venkat 2014 PMID 25419594 (fat-soluble vitamin deficiency monitoring) — PMID:27429428
  • Cited evidence (PMID 19914515) — PMID:19914515
  • Cited evidence (PMID 30244988) — PMID:30244988
  • Cited evidence (PMID 25419594) — PMID:25419594