Neonatal Cholestasis Syndrome: Aetiological Spectrum and Outcome Analysis- Single Center Study
Published: November 1, 2019 | DOI: https://doi.org/10.7860/JCDR/2019/42699.13261
Ann Mary Thomas, Sophy Korula, Leenath Thomas, Santhanam Sridhar, John Mathai, Julie Hephzibah
1. Paediatric Trainee, Department of Paediatric Unit 1, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
2. Associate Professor, Department of Paediatric Unit 1, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
3. Assistant Professor, Department of Paediatric Unit 1, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
4. Professor, Department of Neonatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
5. Professor, Department of Paediatric Surgery, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
6. Professor, Department of Nuclear Medicine, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
Correspondence
Dr. Sophy Korula,
Paediatric Unit 1, ISSCC Building, CMC Hospital, Vellore, Tamil Nadu, India.
E-mail: jsophyhr@yahoo.co.in
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.
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