This handout is for biliary atresia. Your care team identified this based on: persistent jaundice in infant > 2 weeks of age — direct bilirubin evaluation mandated (fawaz naspghan 2017 pmid 27429428).
Other reasons your team may use this plan: 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); dark urine + pale stools in infant — obstructive cholestasis pattern (hartley lancet 2009 pmid 19914515).
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| Kasai hepatoportoenterostomy < 60 days of life — surgical mainstay | Surgical procedure < 60 days of life ideally; performed at pediatric liver center with high case volume | — | — | 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 | 10-20 mg/kg/day PO divided BID-TID, may titrate up to 30 mg/kg/day if tolerated | PO | BID-TID | 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) |
| vitamin A (retinol) supplementation | 5,000-25,000 IU/day PO titrate per vitamin A level | PO | daily | 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) | 800-5,000 IU/day PO titrate per 25-OH-vitamin D level (target > 30 ng/mL) | PO | daily | 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) |
| ergocalciferol (vitamin D2) | alternative to cholecalciferol: 50,000 IU PO once-weekly OR daily dosing per protocol | PO | daily or weekly | Venkat 2014 PMID 25419594 — alternative vitamin D form; cholecalciferol generally preferred (AAP Lexicomp Peds) |
| 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 | 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) |
| 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 | 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) |
| 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 | 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) |
| methylprednisolone | DO NOT USE ROUTINELY post-Kasai (Alonso START 2018 PMID 30244988 — no benefit + significant growth impairment) | PO/IV | not routinely used | 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 |
| 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) | — | — | 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) | 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 | 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) |
Plan: Biliary atresia — Kasai hepatoportoenterostomy < 60 days + post-Kasai medical adjuncts (Fawaz NASPGHAN 2017 PMID 27429428; Hartley Lancet 2009 PMID 19914515)
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
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)
Guideline: 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)