management of neonatala cholestasis

1. MANAGEMENT OF NEONATAL
CHOLESTASIS
PRESENTED BY:Dr.Divya

2. NEONATAL CHOLESTASIS-Definition
• Prolonged elevation of conj.bilirubin beyond 14
days of life
• Serum direct bilirubin greater than 1.0mg/dl if the
total serum bilirubin (TSB) is <5.0 mg/dl or
• > 20% of TSB if the TSB is >5.0 mg/dl
• Any newborn with jaundice and dark yellow urine
with or without pale stools should be strongly
suspected to have NC.

3. NEONATAL CHOLESTASIS-causes
INTRAHEPATIC EXTRAHEPATIC
• Infections EHBA
• Metabolic ds Choledochal cyst
• Genetic ds Cholelithiasis-TPN
• Endocrine ds Drugs
• Paucity of bile ducts
• Familial syndromes

4. INTRAHEPATIC CAUSES
• Inf.-TORCHES
• Hepatitis B,C
• Septicemia
• HIV
• TB
• Listeriosis
• Metabolic-Galactosemia
• Hereditary fructose intolerance
• GSD 4
• Gauchers ds
• Niemann pick ds
• Hereditary tyrosinemia
• Alpha 1 antitrypsin def
• Cystic fibrosis
• NISD

5. INTRAHEPATIC
• Endocrine-Hypothyroidism
• Hypopituitarism
• Genetic-Down syndrome
• Edward syndrome
• Paucity of bile ducts-Watson alagille syndrome
• Familial syn-PFIC
• Aagenes syn
• Carolis ds
• BRIC

6. ETIOLOGICAL PROFILE OF NC IN INDIA

7. • Hepatocellular causes constitute 45% to 69%
• obstructive causes account for 19% to 55% of all
cases

8. CLINICAL PRESENTATION
• Jaundice
• Hepatomegaly
• Acholic stools
• Dark urine
• Other signs and symptoms depend on specific
disease process

9. Differentiation-BA v/s NH-AIIMS SCORE
• Age - <6wks -score 2
• >6wks -score 1
• Icterus-Fluctuating,mild to mod.-score 2
• Severe >8mg% -score 1
• Urine –Normal/yellow coloured –score 2
• High coloured -score 1
• Stool – N./Light yellow -score 2
• Clay coloured -score 1
• Liver –Soft -score 4
• Firm-score 1
• Score >10 – NH
• <10 - BA

10. INVESTIGATIONS
• LFT-Markedly elevated S.ALP+min.elevated SGPT
& SGOT s/o BA
• TFT
• SEPSIS SCREEN

11. Radiological evaluation
Ultrasonography
Findings s/o BA
• triangular cord sign
• gallbladder not visualized
• length <1.9 cm
• To r/o choledochal cyst & cholelithiasis

13. • HIDA scan
• limited role in evaluation of NC
• Performing a HIDA scan is optional and one may
go for a liver biopsy straightaway

14. Liver biopsy
• Early recognition of BA by liver biopsy can avoid
unnecessary laparotomy
• F/O BA-marked bile duct proliferation
• Min. giant cell proliferation
• bile plugs in ducts
• fibrosis and lymphocytic infiltrates in the
portal tracts
• Intra-operative cholangiogram (IOC) remains the
gold standard for diagnosis of BA.

15. Metabolic workup
• Urine exmn-Reducing sugar-Galactosemia,HFI
• Succinyl acetone-Tyrosinemia
• RBC GALT levels- Galactosemia
• AAT levels
• Sweat chloride level
• Aldolase B enz assay in liver biopsy
• S.Ferritin level

17. MANAGEMENT-General Management
• Nutritional support-Caloric req. 125% of RDA
• Breast feed-MCT oil @ 1-2ml/kg/d
• Veg.prot-2-3g/kg/d
• Supplementation of fat soluble vitamins
• Vitamin supplementation should be continued
till 3 months after resolution of jaundice
• Pruritus-UDCA @20mg/kg/d
• Rifampicin @ 5-10mg/kg/d
• Phenobarbitone @ 5-10mg/kg/d

18. SUGGESTED DAILY REQUIREMENTS

20. SPECIFIC TREATMENT
• Special infant formula and diets are
recommended for children with specific diagnosis
(galactosemia,fructosemia and tyrosinemia)
• Nitisinone (1 mg/kg/d)-Tyrosinemia
• Specific therapy -CMV (associated neurological
involvement), herpes and toxoplasmosis related
cholestasis.

21. SURGICAL
• KASAI’s Procedure
• Choledochal cyst
• (PFIC) without decompensated cirrhosis,
external and internal biliary diversion

22. KASAI PROCEDURE
• Performed for biliary atresia that is not
surgically correctable with excision of a distal
atretic segment.
• Roux-en-Y portoenterostomy
• Bile flow re-established in 80-90% if performed
prior to 8 weeks-old.
• Bile flow re-established in less than 20% if
performed after 12 weeks-old

24. LIVER TRANSPLANTATION
• standard therapy for decompensated cirrhosis due
to any cause
• bilirubin >6 mg/dL, three months after kasai’s
surgery
• BA who present with decompensated cirrhosis
(low albumin, prolonged INR, ascites) are not
likely to improve with a Kasai PE and should be
referred for liver transplantation

25. CONCLUSIONS
• NC constitutes almost one-third of children with
chronic liver disease in major hospitals in India.
• BA,NH and metabolic causes are the most
important causes in India
• Early identification of the cause is essential for a
favorable outcome
• Urine and stool color assessment(minimum 3 stool
samples) by the mother and physician in a stool
color card incorporated in all well baby
cards(Indian Academy of Pediatrics and the
Government of India cards)