Published online Sep 18, 2017. doi: 10.4254/wjh.v9.i26.1108
Peer-review started: March 23, 2017
First decision: June 30, 2017
Revised: July 6, 2017
Accepted: September 5, 2017
Article in press: September 7, 2017
Published online: September 18, 2017
To describe the etiology and characteristics of early-onset conjugated hyperbilirubinemia (ECHB) presenting within 14 d of life in term neonates.
Retrospective review was performed of term infants up to 28-d-old who presented with conjugated hyperbilirubinemia (CHB) at a tertiary center over a 5-year period from January 2010 to December 2014. CHB is defined as conjugated bilirubin (CB) fraction greater than 15% of total bilirubin and CB greater or equal to 25 μmol/L. ECHB is defined as CHB detected within 14 d of life. “Late-onset” CHB (LCHB) is detected at 15-28 d of life and served as the comparison group.
Total of 117 patients were recruited: 65 had ECHB, 52 had LCHB. Neonates with ECHB were more likely to be clinically unwell (80.0% vs 42.3%, P < 0.001) and associated with non-hepatic causes (73.8% vs 44.2%, P = 0.001) compared to LCHB. Multifactorial liver injury (75.0%) and sepsis (17.3%) were the most common causes of ECHB in clinically unwell infants, majority (87.5%) had resolution of CHB with no progression to chronic liver disease. Inborn errors of metabolism were rare (5.8%) but associated with high mortality (100%) in our series. In the subgroup of clinically well infants (n = 13) with ECHB, biliary atresia (BA) was the most common diagnosis (61.5%), all presented initially with normal stools and decline in total bilirubin but with persistent CHB.
Secondary hepatic injury is the most common reason for ECHB. BA presents with ECHB in well infants without classical symptoms of pale stools and deep jaundice.
Core tip: Conjugated hyperbilirubinemia (CHB) is not routinely checked before 14-21 d of life, hence incidence and etiology of early-onset CHB (ECHB) before 14 d are not well-documented. Nearly three-quarters of ECHB have non-hepatic cause and are expected to recover with supportive treatment, while biliary atresia and metabolic disorders are important etiologies associated with significant morbidity. In our study, BA presenting before 14 d were detected solely from low levels of CHB without pale stools or worsening jaundice. Further studies are needed to determine if CHB screening before 14 d would lead to improved detection and outcome in neonatal liver disorders.