Approach to cholestasis

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Approach to cholestasis

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

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