Approach to cholestasis
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Transcript of 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