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"Biliary atresia (BA) is a common cause of jaundice in infancy. There is increasing evidence that newborn screening with direct or conjugated bilirubin leads to earlier diagnosis. Although the Kasai portoenterostomy is the primary treatment, there are scientific advances in adjuvant therapies. As pediatric patients transition to adult care, multidisciplinary care is essential, given the complexity of this patient population.", (Copyright © 2021 Elsevier Inc. All rights reserved.)

Hyperbilirubinemia, or jaundice, is common in the ICU, with incidence up to 40% among critically ill patients. Unfortunately, it is poorly understood in the critically ill, and too often presents a diagnostic dilemma to the ICU physician. Causes of jaundice in the ICU are multiple; the etiology in any given patient, multifactorial. Acute jaundice can be a harbinger or marker of sepsis, multisystem organ failure (MSOF), or a reflection of transient hypotension (shock liver), right-sided heart failure, the metabolic breakdown of red blood cells, or pharmacologic toxicity. Acute ICU jaundice is best divided into obstructive and nonobstructive. This stratification directs subsequent management and therapeutic decisions.

Cholestatic jaundice defined as conjugated hyperbilirubinemia is a typical feature of neonatal liver disease. Biliary atresia is the most common disorder producing cholestasis during the first 2 months of life. Syndromic and non-syndromic paucity of the intralobular bile ducts and choledochal cysts can also present with cholestasis during early life. Liver dysfunction from obstruction of the biliary tree must be differentiated from numerous disorders affecting hepatocytes such as congenital infection and inborn errors of metabolism. Early recognition and a stepwise diagnostic evaluation of the cholestatic infant are essential in successfully treating many metabolic and infectious liver diseases of the infant as well as surgically relieving obstruction in patients with biliary atresia.

A 43-year-old male developed abdominal pain and jaundice after the administration of erythromycin estolate. The diagnosis was strongly suspected on clinical grounds, but ultimate confirmation depends upon the demonstration of biochemical and morphological alterations after challenge with the drug.

The need for prognostic and biochemical monitors of cellular function becomes increasingly evident with the further elucidation of the metabolic pattern secondary to disease. Thus far studies have shown that lactic acid may be one of these biochemical parameters. Investigations are now in the process of defining other biochemical indicators of cellular dysfunction.