Jaundice, or icterus, is a yellow-green discoloration of the skin, eyes, mucous membranes, and body fluids that results from excessive bilirubin. Jaundice is apparent in infants with a total serum bilirubin (TSB) concentration greater than 5 mg/dL and in older children with a TSB concentration greater than 2 to 3 mg/dL.1 Total serum bilirubin reflects the sum of unconjugated (ie, indirect) and conjugated (ie, direct) fractions. Conjugated hyperbilirubinemia, also called cholestasis, is defined as a conjugated bilirubin concentration greater than 1 mg/dL or more than 20% of the TSB concentration; it is always pathologic.2 Early detection of cholestatic jaundice and a timely, accurate diagnosis are important for successful treatment and a favorable prognosis.
Although jaundice is a common finding in children, it also can be the presenting sign for a number of other disorders, hepatic as well as extrahepatic, reflecting either excessive bilirubin production or decreased excretion by the liver or biliary system. Binding to albumin prolongs the half-life of direct bilirubin, and even though the results of other liver tests may be normalizing, jaundice may be protracted. Jaundice itself is harmful only in newborns, in whom the accumulation of unconjugated bilirubin may lead to kernicterus in the immature brain.3 The differential diagnosis is age specific. Neonatal jaundice occurs in up to 60% of term infants in the first week of life. Approximately 2% of newborns reach TSB levels in excess of 20 mg/dL.4
The differentiation between nonpathologic and pathologic jaundice begins in the emergency department (ED). In recent years, a large body of evidence and updated guidelines have helped elucidate the pathophysiology of jaundice and outlined appropriate management options for children with cholestasis and related illnesses. This issue of Pediatric Emergency Medicine Practice provides the results of these evidencebased studies and the most up-to-date guidelines to assist the emergency clinician in caring for pediatric patients presenting with jaundice. These recommendations are general guidelines and are not intended as a substitute for clinical judgment or as a protocol for the care of all patients who present with jaundice.
A 2-month-old infant presents to the emergency department with a chief complaint of jaundice. History reveals that the infant was full-term, had a normal birth, and was discharged from the nursery after 2 days. The infant is breastfed. During the last month, the parents have noticed progressive jaundice, and the infant’s stools seem lighter in color. The physical examination reveals a well-appearing, icteric infant without hepatosplenomegaly. The screening bilirubin test from the pediatrician’s office reveals that the infant’s total bilirubin level is elevated, but fractionation was not done. You have seen many jaundiced infants, especially among breastfed patients. Does this infant warrant further evaluation, or is this a case of physiologic/breast milk jaundice? Is there something in the history that suggests this jaundice is worrisome? Should you discharge the infant for follow-up with the primary physician, or should you proceed with emergency department evaluation? If so, what is the next step in examining this patient? Does this infant have acute liver failure that warrants admission to the intensive care unit? Does he have an obstruction of the biliary tree or other surgical emergency? Is this a case of infectious hepatitis, and can the patient be discharged home after proper hydration?
All studies, review articles, and meta-analyses were identified via MEDLINE® and Ovid MEDLINE® (eg, the Cochrane Database of Systematic Reviews, the ACP Journal Club®, the Database of Abstracts of Reviews of Effects, the Cochrane Central Register of Controlled Trials, the Cochrane Methodology Register, the Health Technology Assessment Database, and the National Health Service Economic Evaluation Database). Terms used in the search included jaundice, cholestasis, neonate, infant, pediatric, child, guideline, and emergency department/room. More than 200 articles published from January 1991 to July 2008 were analyzed, providing the background for further review.
Although most population-based data come from well-designed, large-sample studies of infants with jaundice, only a few studies have been prospective in nature. In addition, differences in interhospital bilirubin testing and frequency of identified jaundice are significant.5 This has led to the development of benchmarking models that adjust for race, feeding method, and gestational age in order to compare incidence of hyperbilirubinemia between hospitals.6 Guidelines have been issued by the American Academy of Pediatrics (AAP)7 and the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN)2 using the definitions for quality of evidence and balance of benefits and harm established by the AAP/NASPGHAN Steering Committee on Quality Improvement Management. These guidelines have undergone extensive peer review by committees, outside organizations, and experts in the field.
Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report.