Clinical profile and outcome of conjugated neonatal hyper bilirubinimia in a tertiary pediatric hospital

Introduction: Neonatal cholesstasis is a silent emergency (National Biliary Atresia Registry, 1976). In a tertiary care hospital we can diagnose many cases but outcome depends on post natal age, if post natal age crosses 8 weeks of age prognosis is guarded. Very few studies captured clinical profile and outcome which can alert a practicing pediatrician about the nature of severity and long term follow up (Ohkohchi 1989). Aims and objectives: Outline the etiology and clinical profile of different cases of Conjugated neonatal hyperbilirubinemia and their outcome over 1year follow up. Materials and Method: Babies with conjugated hyperbilirubinimiamore than 20% of total bilirubin presenting at more than 14 days of age at OPD or emergency of Institute of Child Health, Kolkata were admitted in the hospital and started investigation as per standered protocol. Those diagnosed as billary atresia were referred to pediatric surgery and kasai’s operation was done as early as possible preferably within 8 weeks of life. Those presenting later than 10 to12 weeks where chance of failed kasai was very high were put on palliative care after discussion with parents.In this prospective cohort study of 46 patients of conjugated hyperbilirubinemia in a neonate is followed up over 12 months and analysed the morbidity and mortality. Result and analysis: Ideopathic neonatal hepatitis14 (30.5%) was very high followed by billary atresia BA 11(24%), sepsis 8(17.39%) and hypothyroidism 4 (8.69%). Down syndrome and infant of diabetic mother constitute 6.5% (3) each followed by CMV2 (4.3%) and Galactosemia1 (2.17%).Out of 46 cases 17 cases were died within the follow up period of 8 months. No death is reported within 8 to 12 months. Among 17 cases of death BA were 6 ,r est 11 were due to medical cause. Conclusion: The prognosis for intants with neonatal cholestasis syndromespecially due to biliary atresia remains dismal, particularly in our country. It is unfortunate that 35 years after Morio Kasai’s first reported success with his portoenterostomy operation our patients still not benefited from the same. Due to delay in diagnosis most babies undergo surgery late and ultimately need a liver transplant.