1. Neonatal hepatitis syndrome
Jeevan Gyawali

2. Introduction
Neonatal hepatitis syndrome is one of the most frequent cause of conjugated hyperbiliubinemia in the small infant.
Diagnosed by its plurietiologic character:
onset in the first 3 months
subacute or chronic
potentially cirrhogenous
conjugated hyperbilirubinemia
mainly gigantocellular hepatic transformation, the essential characterstic of the diagnosis

3. Any infant who is jaundiced at 2-4 weeks old needs to have the serum conjugated bilirubin measured, even if he/she looks otherwise well.
If conjugated hyperbilirubinaemia is present, a methodical and comprehensive diagnostic investigation should be performed. Early diagnosis is critical for the best outcome. In particular, palliative surgery for extrahepatic biliary atresia has the best chance of success if performed before the infant is 8 weeks old.

4. Causes Infective cause 2. Anatomical and structural 3. metabolic
Viral infection
septicemia
Syphilis
Toxoplasmosis
Rubella
Parvovirus B19 infection
Cytomegalovirus
Herpes simplex virus infection
Enterovirus
2. Anatomical and structural
3. metabolic
4. Genetic
5. Neoplastic
6. Vascular
7. Toxin
8. Immue mediated and idiopathy

5. Clinical features Asymptomatic Urine and stool: Bile stained
Urine dark or brown colored
Acholic or clay colored stool intermittently
Hepatosplenomegaly
Intrauterine infections are associated with growth retardation, meningoencephalitis, ophthalmic manifestation, purpura, anemia, Increased cord IgM level
Infants with herpes simplex, coxsackie-B and echovirus infections manifest with fulminant hepatic necrosis leading to early death.
May fail to gain weight or grow normally
Note- Hepatitis B virus may be acquired in utero, during delivery or post-natally by maternal contact. Mother is usually asymptomatic hepatitis B carrier positive for e antigen or may have suffered from hepatitis within 2 months before or after delivery.

6. Laboratory findings
Moderate elevation of serum bilirubin—slight preponderance of direct reacting bilirubin
Transaminase grossly elevated
Etiological diagnosis in small percentage of people by:
Specific IgM fluorescent antibodies
Rising titer of Hemagglunitation inhibition
Neutralizing
Complement fixation antibodies
Small no of inflammatory cells in the portal tracts

7. treatment
palliative surgery for extrahepatic biliary atresia has the best chance of success if performed before the infant is 8 weeks old.
Corticosteroid given
Alternatively, liver transplantation may be life saving.
Supportive care, especially with attention to nutritional needs, is important for all infants with neonatal hepatitis syndrome.

8. Difference between Neonatal Hepatitis and Extra Hepatic Biliary Atresia (EHBA)
Features
Neonatal Hepatitis
EHBA
Onset
Any time during neonatal period
End of first week
Jaundice
Mild-moderate
Moderate-severe
Stools
Variable in color
Clay colored
Activity and feeding
Normal or slow
Normal
Hepatosplenomegaly
Early
Late
Urinary urobilin + ­-
Sterchobilin in stools -
Serum alkaline phosphatase ++
SGOT and SGPT
Severe dearrangement
Mild to moderate dearrangement
Serum alpha fetoprotein
May be raised
Absent
Alpha 1-anti-trypsin
May be deficient
Generally normal
Australian antigens
+ or -
Tc 99 m-para isopropyl IDA (iminodiacetic acid hepatobilary scintigraphy)
Radioactivity seen in intestine
No radioactivity seen in intestine
Liver biopsy
Giant cell predominate
Hypoplasia and dilation of bile canaliculi predominate
Operative cholangiogram
Block may be visualised

9. cholestasis
prolonged elevation of the serum levels of conjugated bilirubin beyond the 1st 14 days of life.
Jaundice that appears after 2 wk of age, progresses after this time, or does not resolve at this time should be evaluated.
biliary atresia is the prototypic obstructive abnormality. Functional impairment of bile secretion can result from congenital defects or damage to liver cells or to the biliary secretory apparatus.

10. Cont.... Cholestasis in a newborn can be due to
infectious: viral, bacterial ,parasitic
Bile duct abnormality: EHBA
Neoplasia: neonatal leukaemia
metabolic disorder: glycogen storage disease
Chromosomal disorder: trisomy 18,21,13
giving rise to mechanical obstruction of bile flow or to functional impairment of hepatic excretory function and bile secretion

12. Clinical features Jaundice- conjugated hyperbilirubinemia
Yellow urine and/or hypopigmented (Acholic/pale/clay colored)
With EHBA--- infant often full term, look apparently healthy,
Neonatal hepatitis---- infant often small for date or growth retardation, increase association with infections and genetic abnormalities

13. Management Treatment of cause Supportive treatment Nutritional support
Supplementation of fat soluble vitamins
Adequate proteins intake---2-3g/kg/day
Drugs
Ursodeoxycholic acid (UDCA)
Hydrophilic bile acid
Dose mg/kg/day in two divided dose
Along with that---- high dose of fat soluble vitamins with vitamin E and two times RDA of water soluble vitamins should be supplemented
Weekly dose of vitamin K and D in severe cholestasis

14. Cont... Surgery EHBA and cholesdochal cyst ---- surgically corrected
Kasai’s portoenterostomy for EHBA --- out come good if surgery done before 2 month of age
Patients developing---- portal HTN, ascites, infection and hepatic encephalopathy should be treated accordingly

15. Reference
on 2015

16. Thank you