Risk factors and outcomes for hyperbilirubinaemia after heart surgery in children

Introduction:Liver dysfunction, associated with morbidity and mortality, is common in patients with CHD. We investigate risk factors for and outcomes of hyperbilirubinaemia in neonates and infants after cardiac surgery.Materials and methods:In a retrospective analysis of neonates and infants undergoing cardiac surgery at our institution between January 2013 and December 2017, we identified those with post-operative conjugated hyperbilirubinaemia. We tested various demographic and surgical risk factors, and use of post-operative interventions, for an association with conjugated hyperbilirubinaemia. We also tested hyperbilirubinaemia for association with post-operative mortality and prolonged length of stay.Results:We identified 242 post-operative admissions, of which 45 (19%) had conjugated hyperbilirubinaemia. The average conjugated bilirubin level in this group was 2.0 mg/dl versus 0.3 mg/dl for peers without hyperbilirubinaemia. The post-operative use of both extracorporeal membrane oxygenation (OR 4.97, 95% CI 1.89–13.5, p = 0.001) and total parenteral nutrition (OR 2.98, 95% CI 1.34–7.17, p = 0.010) was associated with conjugated hyperbilirubinaemia. No demographic variable analysed was found to be a risk factor. Hyperbilirubinaemia was associated with higher odds of mortality (OR 3.74, 95% CI 2.69–13.8, p = 0.005) and prolonged length of stay (OR 2.87, 95% CI 2.02–7.97, p = 0.005), which were independent of other risk factors.Discussion:We identified the post-operative use of total parenteral nutrition and extracorporeal membrane oxygenation as risk factors for hyperbilirubinaemia. These patients were more likely to experience morbidity and mortality than control peers. As such, bilirubin may be marker for elevated risk of poor post-operative outcomes and should be more frequently measured after cardiac surgery.

Prolonged cholestasis after acute viral hepatitis: successfully treated with oral steroid

Cholestasis is a rare distinct complication of hepatitis A infection. Usually it runs a long indolent course with significant pruritus and malabsorption lasting for few months. A 9-year-old boy presented with yellowish discolouration of eye for 1 month. Liver function test showed conjugated hyperbilirubinaemia. Serology was positive for hepatitis A IgM antibody. Liver biopsy showed features of hepatitis with cholestasis. Child successfully treated with oral steroids.

A suspected case of Progressive Familial Intra-hepatic Cholestasis in a Six-Year-Old Nigerian Child

Progressive Familial Intra-hepatic Cholestasis (PFIC) is a group of heterogeneous, autosomal recessive disorders characterized by cholestasis, jaundice and mutilating pruritus, mostly in infancy. The incidence of PFIC ranges from 1:50,000 to 1:100,000. There are three subtypes; Types 1 and 2 typically present in the neonatal period and early infancy while Type 3 can present in early infancy, childhood or adolescence. This report is about a 6-year old Nigerian girl who presented with jaundice and severe pruritus of one-month duration and abdominal pain of a week duration. The symptoms were preceded by ingestion of Atropine meant for ocular examination two days earlier. She was well-nourished, deeply icteric, had generalized healing scratch marks and hepatomegaly. The laboratory findings included conjugated hyperbilirubinaemia, moderately elevated liver transaminases and Gamma-Glutamyltransferase enzymes. She was managed for PFIC3 using oral ursodeoxycholic acid with complete resolution of the disease.

Metabolic problems and jaundice

This chapter covers problems of glycaemic control in neonates (including investigation and management of hypoglycaemia and hyperglycaemia); management of babies of women with diabetes (infant of the diabetic mother); the physiological basis and rational approach to the treatment of metabolic acidosis in the newborn; the presentation, investigation, and management of inborn errors of metabolism presenting in the newborn; and metabolic bone disease (also known as osteopenia or rickets of prematurity). There is an overview of the investigation and treatment of neonatal jaundice, including physiological jaundice, aetiology, and investigation of non-physiological jaundice and aims of treatment of this; with guidelines for the use of phototherapy, exchange transfusion, and intravenous immunoglobulin. The importance of assessment of prolonged jaundice with reference to conjugated hyperbilirubinaemia is also covered in this section.

Neonatal jaundice

Epidemiology 340Unconjugated hyperbilirubinaemia 340Specific conditions 342Conjugated hyperbilirubinaemia 343Idiopathic neonatal hepatitis 347• 30–50% of normal term newborns are jaundiced after birth.• Physiological and breast milk jaundice account for the majority of cases.• 1 in 2500 infants has conjugated hyperbilirubinaemia....