Neonatal hepatitis syndrome Jeevan Gyawali

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

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.

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

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.

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

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.

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

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.

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

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

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---- 20-30mg/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

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

Reference http://journals.lww.com/jpgn/Abstract/1994/02000/Clinical_and_Biochemical_Findings_in_Progressive.3.aspx http://www.ncbi.nlm.nih.gov/pubmed/2128714 on 2015

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