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Approach to cholestasis
Neonatal conjugated hyperbilirubinemia
Direct bilirubin > 1mg/dl if TSB < 5mg/dl
Direct bilirubin > 20% if TSB > 5mg/dl
Etiology Extrahepatic disorders
Biliary biliary atresia
bile duct stricture/sclerosing cholangitis
anomalies of PD junctio...
• Mass
Intraductular - stone
rhabdomyosarcoma
Extraductular – hepatoblastoma, neuroblastoma
Intrahepatic disorders
• Idiopathic
Neonatal hepatitis
Intrahepatic cholestasis persistent
 severe intrahepatic cholestas...
• Intrahepatic cholestasis recurrent
benign recurrent cholestasis
Aagenes syndrome
Anatomic
Congenital hepatic fibrosis
Ca...
Metabolic/endocrine
Aminoacid metabolism
tyrosinemia
Lipid metabolism
Wolmans disease
Niemann pick
Gauchers disease
• Carbohydrate metabolism
Galactosemia
Fructose intolerance
Glycogen storage disease type 4
Disorders of bileacid metabolism
primary
• Enzyme defe
Disorders of bileacid metabolism
Secondary
Zellwager syndrome
Rotor...
Other metabolic defects
• Cystic fibrosis
• Hypopituitarism
• Hypothyroidism
• Neonatal storage disease
• Menkes disease
Toxic
• TPNAL
• Fetal alcohol syndrome
Infections
TORCH
Listeriosis
Hep B
Hiv
Parvovirus
Chromosomal
• Trisomy 18
21
Vascular
Budd chiari
Perinatal asphyxia
Multiple hemangiomata
Cardiac insufficiency
Miscellaneous
• Shock
• Intestinal obstruction
• Neonatsl lupus
HISTORY
• INFECTIONS
• In mother
• Infant
STOOLS
Pale/clay coloured stools
Persistent acholic stools
Dark urine
• Irritability / vomiting
Metabolic 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 illness
UTI
GALACTOSEMIA
FRUCTO
TYROSINEMIA
NISD
HEMOLYSIS
METABOLIC
HYPOPITUATA...
DIRECT
ABNORMAL
CHOESTATIC JAUNDICE
DIRECT
NORMAL
UNCONJUGATED
HYPERBILURUBINEMIA
• CHOESTATIC JAUNDICE
HISTORY
PHYSICAL EXAM
CUE
SPECIFIC DISEASE EVALUATE & TREAT
TO REPEAT AFTER 6 WKS
NO SPECIFIC DISEASE
+ FOR GALACTOSEMIA,
HYPOTHYROIDISM
FURTHER
MANAGEMENT
CBC
LFT
PLATELET COUNT
PROTHROMBIN
ALBUMIN
ALPHA...
LOW ALPHA
ANTI TRYPSIN
YES
FURTHER
MANAGEMENT
NO
LIVER BIOPSY
SCINTISCAN
DUODENAL ASPIRATE
ERCP
CHOLEDOCHAL
CYST
YES
SURGE...
NO BILIARY
OBSTRUCTION
MEDICAL EVALUATION
INFECTION
METABOLIC DISEASE
GENETIC DISORDERS
BILIARY OBSTRUCTION
SURGERY
LIVER ...
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Approach to cholestasis

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Transcript of "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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