Neonatal Cholestasis Syndrome: Aetiological Spectrum and Outcome Analysis-Single Center Study

• Main Authors: Ann Mary Thomas, Sophy Korula, Leenath Thomas, Santhanam Sridhar, John Mathai, Julie Hephzibah
• Format: Article
• Language: English
• Published: JCDR Research and Publications Private Limited 2019-11-01
• Series: Journal of Clinical and Diagnostic Research
• Subjects: aetiology; follow-up study; growth; liver function; neonatal hepatitis
• Online Access: https://jcdr.net/articles/PDF/13261/42699_CE[Ra1]_F(SHU)_PF1(PoG_OM)_PN(SL).pdf

Introduction: Neonatal Cholestasis syndrome is a common condition among infants that needs to be evaluated in detail to establish aetiology. Although Idiopathic neonatal hepatitis is common, clear outcome for same has not been established. Aim: To assess the clinical profile, aetiological spectrum of infants with Neonatal Cholestasis Syndrome (NCS) followed-up over 10 years. Materials and Methods: It was a retrospective study; data were collected from online records Jan 2008-Dec 2018. Total of 300 infants (210 males) were studied. Clinical outcome was assessed in non-surgical patients with a minimum of 6 month follow-up period. Chi-square test was used for statistical analysis. Results: Intrahepatic NCS was identified in 232 (77%)-majority 164 (54.6%) were idiopathic hepatitis. Remaining 68 (23%) had Extrahepatic NCS and Biliary Atresia (BA) was confirmed in 64 (21.3%). Mean age of presentation was 2.4 months in the intrahepatic group and 3 months in Extrahepatic NCS. All underwent blood tests and ultrasound. Liver biopsy was done in 72 (24%) -35 neonatal hepatitis (2 with cirrhosis), 24 BA, 11 Progressive Familial Intrahepatic Cholestasis, 1 bile duct paucity. All received multivitamin therapy till 1 year of age. Follow-up analysis done in 95 subjects at a mean age of 23.5 months (range 6-90) showed significant improvement in growth (weight centile from 8.9 to 27.7 and height centile from 16.7 to 22.4) and in liver function. Hepatobliary scan had a sensitivity of 90% in diagnosing Biliary Atresia. Conclusion: Idiopathic neonatal hepatitis is the most common aetiology in Neonatal hepatitis. Intrahepatic NCS has good outcome in terms of growth and normalisation of liver function.