Neonatal hepatitis - Revision history

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Ostermayer: Created page with "Neonatal hepatitis is a clinical syndrome of hepatic inflammation and '''conjugated (direct) hyperbilirubinemia''' in the newborn, representing over 100 possible underlying etiologies ranging from benign self-limited conditions to life-threatening emergencies.Neonatal Cholestasis. ''Pediatr Rev''. 2014;35(10):436-443. doi:10.1542/pir.35-10-436 The critical task for the emergency physician is to '''recognize conjugated hyperbilirubinemia as alway..."

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Created page with "Neonatal hepatitis is a clinical syndrome of hepatic inflammation and '''conjugated (direct) hyperbilirubinemia''' in the newborn, representing over 100 possible underlying etiologies ranging from benign self-limited conditions to life-threatening emergencies.<ref name="NeoChol">Neonatal Cholestasis. ''Pediatr Rev''. 2014;35(10):436-443. doi:10.1542/pir.35-10-436</ref> The critical task for the emergency physician is to '''recognize conjugated hyperbilirubinemia as alway..."

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Neonatal hepatitis is a clinical syndrome of hepatic inflammation and '''conjugated (direct) hyperbilirubinemia''' in the newborn, representing over 100 possible underlying etiologies ranging from benign self-limited conditions to life-threatening emergencies.<ref name="NeoChol">Neonatal Cholestasis. ''Pediatr Rev''. 2014;35(10):436-443. doi:10.1542/pir.35-10-436</ref> The critical task for the emergency physician is to '''recognize conjugated hyperbilirubinemia as always pathologic''', distinguish it from benign physiologic jaundice, initiate a systematic workup, and urgently identify time-sensitive diagnoses — especially '''[[Biliary atresia|biliary atresia]]''' (which requires surgery before 60 days of life) and '''fulminant causes''' (sepsis, HSV, galactosemia, [[Tyrosinemia|tyrosinemia]]).<ref name="NASPGHAN">Guideline for the Evaluation of Cholestatic Jaundice in Infants. Joint NASPGHAN/ESPGHAN. ''J Pediatr Gastroenterol Nutr''. 2017;64(1):154-168.</ref>

==Background==
*Neonatal cholestasis occurs in approximately '''1 in 2,500 live births'''<ref name="Frontiers">Götze T, et al. Neonatal Cholestasis – Differential Diagnoses, Current Diagnostic Procedures, and Treatment. ''Front Pediatr''. 2015;3:43. doi:10.3389/fped.2015.00043</ref>
*'''Conjugated (direct) hyperbilirubinemia is NEVER physiologic and NEVER normal''' — it always requires evaluation<ref name="NeoChol"/>
*Definition of conjugated hyperbilirubinemia:
**Direct bilirubin '''> 1 mg/dL''' (regardless of total bilirubin), OR
**Direct bilirubin '''> 20% of total bilirubin''' (if total > 5 mg/dL)<ref name="NASPGHAN"/>
*The term "neonatal hepatitis" historically referred to '''"idiopathic neonatal hepatitis"''' (a diagnosis of exclusion after all identifiable causes are ruled out) — this accounts for ~26% of cases<ref name="Gottesman">Gottesman LE, et al. Etiologies of conjugated hyperbilirubinemia in infancy: a systematic review of 1692 subjects. ''BMC Pediatr''. 2015;15:192. doi:10.1186/s12887-015-0506-5</ref>
*Most common identifiable causes:<ref name="Gottesman"/>
**'''[[Biliary atresia]]''' (~26%) — the most time-sensitive surgical diagnosis
**'''Infections''' (~11%) — CMV is the most common infectious cause
**'''TPN-associated cholestasis''' (~6%)
**'''Alpha-1 antitrypsin deficiency''' (~4%)
**'''Metabolic diseases''' (~4%) — [[Galactosemia|galactosemia]], [[Tyrosinemia|tyrosinemia type 1]], [[Cystinosis|cystinosis]]
**'''Perinatal hypoxia/ischemia''' (~4%)

===EM critical rule===
*'''Any infant jaundiced beyond 2 weeks of age (3 weeks if breastfed and otherwise well) must have a fractionated bilirubin''' — this single step prevents the most consequential missed diagnosis<ref name="NASPGHAN"/>

==Clinical features==
===Cardinal triad of neonatal cholestasis===
*'''Jaundice''' (persisting beyond 2 weeks)
*'''Acholic (pale/clay-colored) stools''' — absent bile pigment indicates biliary obstruction
*'''Dark urine''' (conjugated bilirubin excreted renally)

===Features suggesting specific etiologies===

====Biliary atresia (most time-sensitive)====
*Term infant, often appears well initially
*Progressive jaundice with '''acholic stools''' (single most important clue)
*Hepatomegaly (firm liver)
*May have associated '''situs inversus''' or polysplenia (biliary atresia splenic malformation syndrome)
*'''No''' hepatic synthetic failure early (unlike metabolic/infectious causes)
*'''Kasai hepatoportoenterostomy must be performed before 60 days of age''' for best outcomes; delays worsen prognosis dramatically<ref name="NASPGHAN"/>

====Infectious causes (may present as sepsis)====
*'''[[Neonatal HSV|Neonatal herpes simplex (HSV)]]''' — fulminant liver failure, DIC, vesicular rash (may be absent), seizures; '''acyclovir must be started empirically if suspected'''
*'''[[TORCH infections|TORCH]]''' — toxoplasmosis, rubella, CMV, HSV, syphilis; may present with hepatosplenomegaly, petechiae, microcephaly, chorioretinitis, intracranial calcifications
*'''Bacterial [[Sepsis|sepsis]]''' — UTI (especially ''E. coli'') is a common cause of cholestasis in young infants; gram-negative sepsis
*'''Enterovirus, adenovirus, parvovirus B19'''

====Metabolic emergencies====
*'''[[Galactosemia]]''' — vomiting, poor feeding, hepatomegaly after introduction of lactose-containing formula/breast milk; '''E. coli sepsis''' association; cataracts; reducing substances in urine; '''STOP galactose-containing feeds immediately'''
*'''[[Tyrosinemia|Tyrosinemia type 1]]''' — liver failure with '''coagulopathy out of proportion to transaminases'''; very high AFP; "boiled cabbage" odor; [[Fanconi syndrome]]
*'''Hereditary fructose intolerance''' — hepatic failure after introduction of fructose/sucrose to diet
*'''[[Neonatal hemochromatosis]]''' (gestational alloimmune liver disease; GALD) — severe liver failure present at birth or within hours; high ferritin; requires exchange transfusion/IVIG
*'''Hypothyroidism''' — may present with prolonged jaundice; check TSH
*'''Cystic fibrosis''' — meconium ileus history; may present with cholestasis

====Other causes====
*'''Alpha-1 antitrypsin deficiency''' — most common genetic cause of liver disease in children; may present as neonatal cholestasis or later as cirrhosis
*'''Alagille syndrome''' — characteristic facies (broad forehead, pointed chin, deep-set eyes), butterfly vertebrae, cardiac murmur (peripheral pulmonic stenosis), posterior embryotoxon on eye exam
*'''Progressive familial intrahepatic cholestasis (PFIC)''' — various subtypes; severe pruritus (may not be apparent in neonates)
*'''TPN-associated cholestasis''' — in premature infants receiving prolonged parenteral nutrition
*'''Choledochal cyst''' — may present as a palpable RUQ mass with jaundice

===General examination findings===
*Hepatomegaly (nearly universal in cholestasis)
*Splenomegaly (suggests portal hypertension, storage disease, or infection)
*Growth failure, poor feeding
*Coagulopathy/bleeding (vitamin K deficiency from fat malabsorption, or hepatic synthetic failure)
*'''Stool color is critically important''' — ask caregivers and inspect stools directly

==Differential diagnosis==
===Must-not-miss (time-sensitive or life-threatening)===
*'''[[Biliary atresia]]''' — surgery before 60 days
*'''[[Neonatal HSV]]''' — acyclovir immediately
*'''Bacterial [[Sepsis|sepsis]]/UTI''' — antibiotics immediately
*'''[[Galactosemia]]''' — stop lactose immediately
*'''[[Tyrosinemia|Tyrosinemia type 1]]''' — start nitisinone
*'''[[Neonatal hemochromatosis]]''' (GALD) — exchange transfusion/IVIG

===Common causes===
*Idiopathic neonatal hepatitis (diagnosis of exclusion)
*TORCH infections (especially CMV)
*TPN-associated cholestasis (premature infants)
*Alpha-1 antitrypsin deficiency
*Alagille syndrome

===Conditions that mimic cholestasis (unconjugated)===
*Physiologic jaundice (unconjugated; resolves by 2 weeks)
*Breast milk jaundice (unconjugated; benign; may persist 3+ weeks)
*Hemolytic disease (ABO/Rh incompatibility)
*[[Gilbert syndrome]], [[Crigler-Najjar syndrome]]
*'''These are distinguished by fractionated bilirubin — the direct fraction will be normal'''

==Evaluation==
===ED workup===
*'''Fractionated (total and direct) bilirubin''' — the single most important initial test; identifies conjugated hyperbilirubinemia<ref name="NASPGHAN"/>
*'''Hepatic function panel:''' AST, ALT, GGT, alkaline phosphatase, albumin
**GGT is particularly useful: markedly elevated in biliary atresia and bile duct disorders; normal/low in PFIC
*'''Coagulation studies (PT/INR):''' hepatic synthetic function; also assesses vitamin K deficiency
*'''CBC with differential:''' infection, anemia, thrombocytopenia
*'''Blood glucose:''' hypoglycemia (metabolic diseases, liver failure)
*'''BMP:''' electrolytes, renal function
*'''Blood cultures, urinalysis, urine culture''' — sepsis/UTI workup in any ill-appearing infant
*'''Newborn screening results''' — review if available; many metabolic causes are screened

===Directed second-tier testing (initiate from ED or arrange urgently)===
*'''Abdominal ultrasound:''' evaluate for biliary atresia (absent/atretic gallbladder, triangular cord sign), choledochal cyst, gallstones; does '''not''' reliably exclude biliary atresia
*'''TORCH titers/PCR:''' CMV urine PCR, HSV PCR, rubella, toxoplasmosis, syphilis (RPR/VDRL)
*'''Urine reducing substances:''' positive in galactosemia (but not specific; false negatives occur)
*'''TSH, free T4:''' hypothyroidism
*'''Alpha-1 antitrypsin level and phenotype'''
*'''Urine succinylacetone:''' pathognomonic for [[Tyrosinemia|tyrosinemia type 1]]
*'''Serum ferritin, transferrin saturation:''' neonatal hemochromatosis (ferritin often massively elevated)
*'''AFP (alpha-fetoprotein):''' markedly elevated in tyrosinemia; elevated in normal neonates but should be declining
*'''Sweat chloride or immunoreactive trypsinogen:''' if cystic fibrosis suspected
*'''Galactose-1-phosphate uridylyltransferase (GALT) activity:''' confirms galactosemia

===When to suspect biliary atresia===
*Full-term infant with '''persistent acholic stools + conjugated hyperbilirubinemia + hepatomegaly'''
*GGT markedly elevated
*Absent or atretic gallbladder on ultrasound (though ultrasound sensitivity is imperfect)
*'''Refer urgently to pediatric surgery/hepatology''' — do not wait for complete workup if clinical suspicion is high; intraoperative cholangiogram is the gold standard

==Management==
===Immediate ED management===
*'''Vitamin K:''' administer '''1 mg IM (neonatal dose)''' to any infant with cholestasis and coagulopathy — fat-soluble vitamin malabsorption causes vitamin K deficiency and bleeding risk; this can be life-saving<ref name="Merck">Neonatal Cholestasis. ''Merck Manual Professional Edition''. 2025.</ref>
*'''NPO if acutely ill''' until stabilized; IV dextrose-containing fluids to prevent hypoglycemia
*'''Empiric antibiotics''' if sepsis suspected (ampicillin + gentamicin or cefotaxime per neonatal protocol)
*'''Empiric acyclovir''' if '''any''' suspicion for neonatal HSV (do not wait for HSV PCR results; disseminated HSV with hepatitis carries >80% mortality without treatment)<ref name="NASPGHAN"/>
*'''Stop galactose-containing feeds''' (breast milk and standard lactose-based formula) if galactosemia suspected — switch to soy-based formula
*'''Stop fructose/sucrose''' if hereditary fructose intolerance suspected
*'''Correct coagulopathy''' with vitamin K; FFP if active bleeding
*'''Correct hypoglycemia''' with IV dextrose

===Supportive care===
*Fat-soluble vitamin supplementation (A, D, E, K) — arrange through pediatric GI
*For formula-fed infants: MCT-enriched formula (medium-chain triglycerides are absorbed without bile salts)
*Ursodeoxycholic acid (UDCA) — choleretic agent; may be started by GI/hepatology for some causes of intrahepatic cholestasis

===Disease-specific management (arrange via specialist)===
*'''Biliary atresia:''' Kasai hepatoportoenterostomy (ideally before day 45-60 of life)
*'''Tyrosinemia type 1:''' nitisinone + low-tyrosine diet (see [[Tyrosinemia]])
*'''Galactosemia:''' strict galactose-free diet
*'''Neonatal hemochromatosis (GALD):''' exchange transfusion + IVIG (consult neonatology/hepatology emergently)
*'''Alpha-1 antitrypsin deficiency:''' supportive; liver transplant if progressive
*'''Hypothyroidism:''' levothyroxine

==Disposition==
*'''All infants with confirmed conjugated hyperbilirubinemia should be admitted''' or have '''same-day/next-day urgent pediatric GI/hepatology follow-up''' arranged from the ED<ref name="NASPGHAN"/>
*'''Admit to hospital (often NICU/PICU):'''
**Any ill-appearing infant
**Suspected sepsis, HSV, or metabolic emergency
**Coagulopathy or active bleeding
**Hepatic failure (elevated INR, hypoglycemia, encephalopathy)
**Suspected biliary atresia — urgent surgical consultation
*'''Urgent outpatient referral (if well-appearing, stable, feeding well):'''
**Conjugated hyperbilirubinemia confirmed but infant is clinically well
**Pediatric GI/hepatology appointment within '''24-48 hours'''
**Ensure caregivers understand return precautions: fever, pale stools, increasing jaundice, bleeding, poor feeding, lethargy
*'''Communicate stool color education:''' provide caregivers with a '''stool color card''' if available (used in some countries for biliary atresia screening); instruct them to report acholic/pale stools immediately
*'''Do not attribute persistent jaundice beyond 2 weeks to "breast milk jaundice" without fractionating bilirubin''' — this is the most common cause of delayed diagnosis of biliary atresia and other serious conditions

==See Also==
*[[Neonatal jaundice]]
*[[Biliary atresia]]
*[[Neonatal HSV]]
*[[Galactosemia]]
*[[Tyrosinemia]]
*[[Fanconi syndrome]]
*[[Cystinosis]]
*[[Sepsis]]
*[[TORCH infections]]
*[[Neonatal hemochromatosis]]

==External Links==
*[https://www.naspghan.org/files/documents/pdfs/position-papers/Guideline_for_the_Evaluation_of_Cholestatic.23.pdf NASPGHAN/ESPGHAN — Guideline for the Evaluation of Cholestatic Jaundice in Infants (2017)]
*[https://pmc.ncbi.nlm.nih.gov/articles/PMC3827866/ Pediatr Rev — Neonatal Cholestasis (2014)]
*[https://pmc.ncbi.nlm.nih.gov/articles/PMC4654877/ BMC Pediatr — Etiologies of conjugated hyperbilirubinemia in infancy (2015)]
*[https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2015.00043/full Front Pediatr — Neonatal Cholestasis: Differential Diagnoses, Current Diagnostic Procedures, and Treatment (2015)]
*[https://www.merckmanuals.com/professional/pediatrics/gastrointestinal-disorders-in-neonates-and-infants/neonatal-cholestasis Merck Manual — Neonatal Cholestasis]

==References==
<references/>

[[Category:Pediatrics]]
[[Category:GI]]

← Older revision		Revision as of 09:30, 22 March 2026
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	==Background==		==Background==
	*Neonatal hepatitis is a clinical syndrome of hepatic inflammation and conjugated (direct) hyperbilirubinemia in the newborn, representing over 100 possible underlying etiologies ranging from benign self-limited conditions to life-threatening emergencies.<ref name="NeoChol">Neonatal Cholestasis. ''Pediatr Rev''. 2014;35(10):436-443. doi:10.1542/pir.35-10-436</ref> The critical task for the emergency physician is to recognize conjugated hyperbilirubinemia as always pathologic, distinguish it from benign physiologic jaundice, initiate a systematic workup, and urgently identify time-sensitive diagnoses — especially [[Biliary atresia\|biliary atresia]] (which requires surgery before 60 days of life) and fulminant causes (sepsis, HSV, galactosemia, [[Tyrosinemia\|tyrosinemia]]).<ref name="NASPGHAN">Guideline for the Evaluation of Cholestatic Jaundice in Infants. Joint NASPGHAN/ESPGHAN. ''J Pediatr Gastroenterol Nutr''. 2017;64(1):154-168.</ref>		*Neonatal hepatitis is a clinical syndrome of hepatic inflammation and conjugated (direct) hyperbilirubinemia in the newborn, representing over 100 possible underlying etiologies ranging from benign self-limited conditions to life-threatening emergencies.<ref name="NeoChol">Neonatal Cholestasis. ''Pediatr Rev''. 2014;35(10):436-443. doi:10.1542/pir.35-10-436</ref> The critical task for the emergency physician is to recognize conjugated hyperbilirubinemia as always pathologic, distinguish it from benign physiologic jaundice, initiate a systematic workup, and urgently identify time-sensitive diagnoses — especially [[Biliary atresia\|biliary atresia]] (which requires surgery before 60 days of life) and fulminant causes (sepsis, HSV, galactosemia, [[Tyrosinemia\|tyrosinemia]]).<ref name="NASPGHAN">Guideline for the Evaluation of Cholestatic Jaundice in Infants. Joint NASPGHAN/ESPGHAN. ''J Pediatr Gastroenterol Nutr''. 2017;64(1):154-168.</ref>
	*Neonatal cholestasis occurs in approximately ~~'''~~1 in 2,500 live births~~'''~~<ref name="Frontiers">Götze T, et al. Neonatal Cholestasis – Differential Diagnoses, Current Diagnostic Procedures, and Treatment. ''Front Pediatr''. 2015;3:43. doi:10.3389/fped.2015.00043</ref>		*Neonatal cholestasis occurs in approximately 1 in 2,500 live births<ref name="Frontiers">Götze T, et al. Neonatal Cholestasis – Differential Diagnoses, Current Diagnostic Procedures, and Treatment. ''Front Pediatr''. 2015;3:43. doi:10.3389/fped.2015.00043</ref>
	*'''Conjugated (direct) hyperbilirubinemia is NEVER physiologic and NEVER normal''' — it always requires evaluation<ref name="NeoChol"/>		*'''Conjugated (direct) hyperbilirubinemia is NEVER physiologic and NEVER normal''' — it always requires evaluation<ref name="NeoChol"/>
	*Definition of conjugated hyperbilirubinemia:		*Definition of conjugated hyperbilirubinemia:
	**Direct bilirubin ~~'''~~> 1 mg/dL~~'''~~ (regardless of total bilirubin), OR		**Direct bilirubin > 1 mg/dL (regardless of total bilirubin), OR
	**Direct bilirubin ~~'''~~> 20% of total bilirubin~~'''~~ (if total > 5 mg/dL)<ref name="NASPGHAN"/>		**Direct bilirubin > 20% of total bilirubin (if total > 5 mg/dL)<ref name="NASPGHAN"/>
	*The term "neonatal hepatitis" historically referred to ~~'''~~"idiopathic neonatal hepatitis"~~'''~~ (a diagnosis of exclusion after all identifiable causes are ruled out) — this accounts for ~26% of cases<ref name="Gottesman">Gottesman LE, et al. Etiologies of conjugated hyperbilirubinemia in infancy: a systematic review of 1692 subjects. ''BMC Pediatr''. 2015;15:192. doi:10.1186/s12887-015-0506-5</ref>		*The term "neonatal hepatitis" historically referred to "idiopathic neonatal hepatitis" (a diagnosis of exclusion after all identifiable causes are ruled out) — this accounts for ~26% of cases<ref name="Gottesman">Gottesman LE, et al. Etiologies of conjugated hyperbilirubinemia in infancy: a systematic review of 1692 subjects. ''BMC Pediatr''. 2015;15:192. doi:10.1186/s12887-015-0506-5</ref>
	*Most common identifiable causes:<ref name="Gottesman"/>		*Most common identifiable causes:<ref name="Gottesman"/>
	** [[Biliary atresia]] (~26%) — the most time-sensitive surgical diagnosis		** [[Biliary atresia]] (~26%) — the most time-sensitive surgical diagnosis
Line 28:		Line 28:
	====Biliary atresia (most time-sensitive)====		====Biliary atresia (most time-sensitive)====
	*Term infant, often appears well initially		*Term infant, often appears well initially
	*Progressive jaundice with ~~'''~~acholic stools~~'''~~ (single most important clue)		*Progressive jaundice with acholic stools (single most important clue)
	*Hepatomegaly (firm liver)		*Hepatomegaly (firm liver)
	*May have associated ~~'''~~situs inversus~~'''~~ or polysplenia (biliary atresia splenic malformation syndrome)		*May have associated situs inversus or polysplenia (biliary atresia splenic malformation syndrome)
	* No hepatic synthetic failure early (unlike metabolic/infectious causes)		* No hepatic synthetic failure early (unlike metabolic/infectious causes)
	*'''Kasai hepatoportoenterostomy must be performed before 60 days of age''' for best outcomes; delays worsen prognosis dramatically<ref name="NASPGHAN"/>		*'''Kasai hepatoportoenterostomy must be performed before 60 days of age''' for best outcomes; delays worsen prognosis dramatically<ref name="NASPGHAN"/>
Line 42:		Line 42:
	====Metabolic emergencies====		====Metabolic emergencies====
	* [[Galactosemia]] — vomiting, poor feeding, hepatomegaly after introduction of lactose-containing formula/breast milk; '''E. coli sepsis''' association; cataracts; reducing substances in urine; '''STOP galactose-containing feeds immediately'''		* [[Galactosemia]] — vomiting, poor feeding, hepatomegaly after introduction of lactose-containing formula/breast milk; '''E. coli sepsis''' association; cataracts; reducing substances in urine; '''STOP galactose-containing feeds immediately'''
	* [[Tyrosinemia\|Tyrosinemia type 1]] — liver failure with ~~'''~~coagulopathy out of proportion to transaminases~~'''~~; very high AFP; "boiled cabbage" odor; [[Fanconi syndrome]]		* [[Tyrosinemia\|Tyrosinemia type 1]] — liver failure with coagulopathy out of proportion to transaminases; very high AFP; "boiled cabbage" odor; [[Fanconi syndrome]]
	* Hereditary fructose intolerance — hepatic failure after introduction of fructose/sucrose to diet		* Hereditary fructose intolerance — hepatic failure after introduction of fructose/sucrose to diet
	* [[Neonatal hemochromatosis]] (gestational alloimmune liver disease; GALD) — severe liver failure present at birth or within hours; high ferritin; requires exchange transfusion/IVIG		* [[Neonatal hemochromatosis]] (gestational alloimmune liver disease; GALD) — severe liver failure present at birth or within hours; high ferritin; requires exchange transfusion/IVIG
Line 98:		Line 98:

	===Directed second-tier testing (initiate from ED or arrange urgently)===		===Directed second-tier testing (initiate from ED or arrange urgently)===
	* Abdominal ultrasound: evaluate for biliary atresia (absent/atretic gallbladder, triangular cord sign), choledochal cyst, gallstones; does ~~'''~~not~~'''~~ reliably exclude biliary atresia		* Abdominal ultrasound: evaluate for biliary atresia (absent/atretic gallbladder, triangular cord sign), choledochal cyst, gallstones; does not reliably exclude biliary atresia
	* TORCH titers/PCR: CMV urine PCR, HSV PCR, rubella, toxoplasmosis, syphilis (RPR/VDRL)		* TORCH titers/PCR: CMV urine PCR, HSV PCR, rubella, toxoplasmosis, syphilis (RPR/VDRL)
	* Urine reducing substances: positive in galactosemia (but not specific; false negatives occur)		* Urine reducing substances: positive in galactosemia (but not specific; false negatives occur)
Line 110:		Line 110:

	===When to suspect biliary atresia===		===When to suspect biliary atresia===
	*Full-term infant with ~~'''~~persistent acholic stools + conjugated hyperbilirubinemia + hepatomegaly~~'''~~		*Full-term infant with persistent acholic stools + conjugated hyperbilirubinemia + hepatomegaly
	*GGT markedly elevated		*GGT markedly elevated
	*Absent or atretic gallbladder on ultrasound (though ultrasound sensitivity is imperfect)		*Absent or atretic gallbladder on ultrasound (though ultrasound sensitivity is imperfect)
Line 117:		Line 117:
	==Management==		==Management==
	===Immediate ED management===		===Immediate ED management===
	* Vitamin K: administer ~~'''~~1 mg IM (neonatal dose)~~'''~~ to any infant with cholestasis and coagulopathy — fat-soluble vitamin malabsorption causes vitamin K deficiency and bleeding risk; this can be life-saving<ref name="Merck">Neonatal Cholestasis. ''Merck Manual Professional Edition''. 2025.</ref>		* Vitamin K: administer 1 mg IM (neonatal dose) to any infant with cholestasis and coagulopathy — fat-soluble vitamin malabsorption causes vitamin K deficiency and bleeding risk; this can be life-saving<ref name="Merck">Neonatal Cholestasis. ''Merck Manual Professional Edition''. 2025.</ref>
	* NPO if acutely ill until stabilized; IV dextrose-containing fluids to prevent hypoglycemia		* NPO if acutely ill until stabilized; IV dextrose-containing fluids to prevent hypoglycemia
	* Empiric antibiotics if sepsis suspected (ampicillin + gentamicin or cefotaxime per neonatal protocol)		* Empiric antibiotics if sepsis suspected (ampicillin + gentamicin or cefotaxime per neonatal protocol)
Line 140:		Line 140:

	==Disposition==		==Disposition==
	* All infants with confirmed conjugated hyperbilirubinemia should be admitted or have ~~'''~~same-day/next-day urgent pediatric GI/hepatology follow-up~~'''~~ arranged from the ED<ref name="NASPGHAN"/>		* All infants with confirmed conjugated hyperbilirubinemia should be admitted or have same-day/next-day urgent pediatric GI/hepatology follow-up arranged from the ED<ref name="NASPGHAN"/>
	* Admit to hospital (often NICU/PICU):		* Admit to hospital (often NICU/PICU):
	**Any ill-appearing infant		**Any ill-appearing infant
Line 149:		Line 149:
	* Urgent outpatient referral (if well-appearing, stable, feeding well):		* Urgent outpatient referral (if well-appearing, stable, feeding well):
	**Conjugated hyperbilirubinemia confirmed but infant is clinically well		**Conjugated hyperbilirubinemia confirmed but infant is clinically well
	**Pediatric GI/hepatology appointment within ~~'''~~24-48 hours~~'''~~		**Pediatric GI/hepatology appointment within 24-48 hours
	**Ensure caregivers understand return precautions: fever, pale stools, increasing jaundice, bleeding, poor feeding, lethargy		**Ensure caregivers understand return precautions: fever, pale stools, increasing jaundice, bleeding, poor feeding, lethargy
	* Communicate stool color education: provide caregivers with a '''stool color card''' if available (used in some countries for biliary atresia screening); instruct them to report acholic/pale stools immediately		* Communicate stool color education: provide caregivers with a '''stool color card''' if available (used in some countries for biliary atresia screening); instruct them to report acholic/pale stools immediately