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Approach to evaluating neonatal cholestasis
- 2. Neonatal conjugated hyperbilirubinemia Direct bilirubin > 1mg/dl if TSB < 5mg/dl Direct bilirubin > 20% if TSB > 5mg/dl
- 3. Etiology Extrahepatic disorders Biliary biliary atresia bile duct stricture/sclerosing cholangitis anomalies of PD junction choledochal cyst spontaneous perforation of bileduct inspissated bile
- 4. • Mass Intraductular - stone rhabdomyosarcoma Extraductular – hepatoblastoma, neuroblastoma
- 5. Intrahepatic disorders • Idiopathic Neonatal hepatitis Intrahepatic cholestasis persistent severe intrahepatic cholestasis with progressive hepatocellular disease Alagille syndrome
- 6. • Intrahepatic cholestasis recurrent benign recurrent cholestasis Aagenes syndrome Anatomic Congenital hepatic fibrosis Caroli disease
- 7. Metabolic/endocrine Aminoacid metabolism tyrosinemia Lipid metabolism Wolmans disease Niemann pick Gauchers disease
- 8. • Carbohydrate metabolism Galactosemia Fructose intolerance Glycogen storage disease type 4
- 9. Disorders of bileacid metabolism primary • Enzyme defe Disorders of bileacid metabolism Secondary Zellwager syndrome Rotor syndrome Dubin johnson Mitochondrial hepatopathies
- 10. Other metabolic defects • Cystic fibrosis • Hypopituitarism • Hypothyroidism • Neonatal storage disease • Menkes disease
- 11. Toxic • TPNAL • Fetal alcohol syndrome Infections TORCH Listeriosis Hep B Hiv Parvovirus
- 12. Chromosomal • Trisomy 18 21 Vascular Budd chiari Perinatal asphyxia Multiple hemangiomata Cardiac insufficiency
- 13. Miscellaneous • Shock • Intestinal obstruction • Neonatsl lupus
- 14. HISTORY • INFECTIONS • In mother • Infant STOOLS Pale/clay coloured stools Persistent acholic stools Dark urine
- 15. • Irritability / vomiting Metabolic disease lethary seizures Sepsis Hypothhyroidism
- 16. Family historY • Early childhood deaths • Jaundice PFIC, Cystic fibrosis,alpha antitrypsin PNALD
- 17. PHYSICAL EXAMINATION • Biliary atresia • Alagille syndrome is healthy Metabolic disease is sick
- 18. Jaundiced infant 2 to 8 week old Rx acute illness UTI GALACTOSEMIA FRUCTO TYROSINEMIA NISD HEMOLYSIS METABOLIC HYPOPITUATARISM ↑DIRECT BILIRUBIN Acutely ill?
- 20. • CHOESTATIC JAUNDICE HISTORY PHYSICAL EXAM CUE SPECIFIC DISEASE EVALUATE & TREAT TO REPEAT AFTER 6 WKS
- 21. NO SPECIFIC DISEASE + FOR GALACTOSEMIA, HYPOTHYROIDISM FURTHER MANAGEMENT CBC LFT PLATELET COUNT PROTHROMBIN ALBUMIN ALPHA 1 ANTITRYPSIN URINE REDUCING SUBSTANCES USG ABDOMEN
- 22. LOW ALPHA ANTI TRYPSIN YES FURTHER MANAGEMENT NO LIVER BIOPSY SCINTISCAN DUODENAL ASPIRATE ERCP CHOLEDOCHAL CYST YES SURGERY NO LIVER BIOPSY SCINTISCAN DUODENAL ASPIRATE ERCP
- 23. NO BILIARY OBSTRUCTION MEDICAL EVALUATION INFECTION METABOLIC DISEASE GENETIC DISORDERS BILIARY OBSTRUCTION SURGERY LIVER BIOPSY SCINTISCAN DUODENAL ASPIRATE ERCP