CC-BY-NC-ND 4.0 · AJP Rep 2018; 08(02): e95-e98
DOI: 10.1055/s-0038-1649339
Case Report
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Liver Failure and Conjugated Hyperbilirubinemia in a Preterm Neonate: Role of Early IVIG and Exchange Transfusion

Jayasree Nair
Department of Pediatrics, John R. Oishei Children's Hospital, Buffalo, New York
,
Vasantha H.S. Kumar
Department of Pediatrics, John R. Oishei Children's Hospital, Buffalo, New York
› Author Affiliations
Source of Funding None.
Further Information

Publication History

31 August 2017

04 March 2018

Publication Date:
14 May 2018 (online)

Abstract

Neonatal liver failure (NLF) is a rare diagnosis but carries with it significant risks of mortality and morbidity. Common etiologies for NLF include metabolic causes, gestational alloimmune liver disease (GALD or neonatal hemochromatosis), and viral infections. We report a case of liver failure in a premature infant with abnormal iron profile within 48 hours of birth. Lack of accepted guidelines for the initial management of severe jaundice with a high direct component in the first week after birth made treatment challenging. The infant underwent intensive phototherapy along with four exchange transfusions (ET) and two courses of intravenous immunoglobulins (IVIG). The clinical goals were to keep total bilirubin values ≤ 20 mg/dL in this premature neonate and to minimize the risk of bilirubin-induced neurologic dysfunction and decompensated liver failure. Abnormal iron studies and later magnetic resonance imaging were suggestive of GALD. Liver functions improved over time with normal neurodevelopmental assessment at 3 years of age. To conclude, in infants with NLF soon after birth, earlier consideration of IVIG/ET in the first few days may be beneficial. Larger multicenter data analyses are required to formulate treatment guidelines and indications for phototherapy, ET, and IVIG in sick neonates with NLF.