approach to cholestasis

Approach to cholestasis

Neonatal conjugated hyperbilirubinemiaDirect bilirubin > 1mg/dl if TSB < 5mg/dlDirect bilirubin > 20% if TSB > 5mg/dl

Etiology Extrahepatic disorders

Biliary biliary atresia

bile duct stricture/sclerosing cholangitis

anomalies of PD junction

choledochal cyst

spontaneous perforation of bileduct

inspissated bile

•MassIntraductular - stone rhabdomyosarcoma

Extraductular – hepatoblastoma, neuroblastoma

Intrahepatic disorders •IdiopathicNeonatal hepatitis

Intrahepatic cholestasis persistent

severe intrahepatic cholestasis with progressive hepatocellular disease

Alagille syndrome

•Intrahepatic cholestasis recurrent benign recurrent cholestasis Aagenes syndrome

AnatomicCongenital hepatic fibrosisCaroli disease

Metabolic/endocrine

Aminoacid metabolism tyrosinemia

Lipid metabolismWolmans disease

Niemann pick

Gauchers disease

•Carbohydrate metabolismGalactosemiaFructose intoleranceGlycogen storage disease type 4

Disorders of bileacid metabolismprimary

•Enzyme defe

Disorders of bileacid metabolism Secondary

Zellwager syndromeRotor syndromeDubin johnsonMitochondrial hepatopathies

Other metabolic defects

•Cystic fibrosis•Hypopituitarism•Hypothyroidism•Neonatal storage disease•Menkes disease

Toxic

•TPNAL•Fetal alcohol syndrome

InfectionsTORCHListeriosisHep BHivParvovirus

Chromosomal

•Trisomy 18 21

VascularBudd chiariPerinatal asphyxiaMultiple hemangiomataCardiac insufficiency

Miscellaneous•Shock•Intestinal obstruction•Neonatsl lupus

HISTORY

•INFECTIONS•In mother•Infant

STOOLSPale/clay coloured stoolsPersistent acholic stoolsDark urine

•Irritability / vomitingMetabolic disease lethary seizures

Sepsis

Hypothhyroidism

Family historY•Early childhood deaths•Jaundice PFIC, Cystic

fibrosis,alpha antitrypsin

PNALD

PHYSICAL EXAMINATION•Biliary atresia•Alagille syndrome is healthy

Metabolic disease is sick

Jaundiced infant 2 to 8 week old

Rx acute illnessUTI

GALACTOSEMIAFRUCTO

TYROSINEMIANISD

HEMOLYSISMETABOLIC

HYPOPITUATARISM

↑DIRECT BILIRUBIN

Acutely ill?

DIRECT ABNORMA

L

CHOESTATIC JAUNDICE

DIRECT NORMAL

UNCONJUGATED HYPERBILURUBINEMI

A

•CHOESTATIC JAUNDICE

HISTORY PHYSICAL EXAM CUE

SPECIFIC DISEASE EVALUATE & TREAT TO REPEAT AFTER 6 WKS

NO SPECIFIC DISEASE

+ FOR GALACTOSEMIA,

HYPOTHYROIDISM

FURTHER MANAGEMENT

CBCLFT

PLATELET COUNTPROTHROMBIN

ALBUMINALPHA 1 ANTITRYPSIN

URINE REDUCING SUBSTANCES

USG ABDOMEN

LOW ALPHA ANTI TRYPSIN

YESFURTHER

MANAGEMENT

NOLIVER BIOPSYSCINTISCANDUODENAL ASPIRATE

ERCP

CHOLEDOCHAL CYST

YESSURGERY

NOLIVER BIOPSYSCINTISCAN

DUODENAL ASPIRATEERCP

NO BILIARY OBSTRUCTION

MEDICAL EVALUATIONINFECTION

METABOLIC DISEASEGENETIC DISORDERS

BILIARY OBSTRUCTION

SURGERY

LIVER BIOPSYSCINTISCANDUODENAL ASPIRATE

ERCP