Management options for cholestatic liver disease in children

Expert Rev Gastroenterol Hepatol. 2017 Nov;11(11):1019-1030. doi: 10.1080/17474124.2017.1359538. Epub 2017 Jul 28.

Abstract

Due to a peculiar age-dependent increased susceptibility, neonatal cholestasis affects the liver of approximately 1 in every 2500 term infants. A high index of suspicion is the key to an early diagnosis, and to implement timely, often life-saving treatments. Even when specific treatment is not available or curative, prompt medical management and optimization of nutrition are of paramount importance to survival and avoidance of complications. Areas covered: The present article will prominently focus on a series of newer diagnostic and therapeutic options of cholestasis in neonates and infants blended with consolidated established paradigms. The overview of strategies for the management reported here is based on a systematic literature search published in English using accessible databases (PubMed, MEDLINE) with the keywords biliary atresia, choleretics and neonatal cholestasis. References lists from retrieved articles were also reviewed. Expert commentary: A large number of uncommon and rare hepatobiliary disorders may present with cholestasis during the neonatal and infantile period. Potentially life-saving disease-specific pharmacological and surgical therapeutic approaches are currently available. Advances in hepatobiliary transport mechanisms have started clarifying fundamental aspects of inherited and acquired cholestasis, laying the foundation for the development of possibly more effective specific therapies.

Keywords: Biliary atresia; choleretics; diagnosis; jaundice; management; neonatal cholestasis; treatment; ursodeoxycholic acid.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Bile / metabolism
  • Biliary Atresia / diagnosis
  • Biliary Atresia / metabolism
  • Biliary Atresia / physiopathology
  • Biliary Atresia / therapy*
  • Cholagogues and Choleretics / adverse effects
  • Cholagogues and Choleretics / therapeutic use*
  • Cholestasis / diagnosis
  • Cholestasis / metabolism
  • Cholestasis / physiopathology
  • Cholestasis / therapy
  • Digestive System Surgical Procedures
  • Humans
  • Infant
  • Infant Nutritional Physiological Phenomena
  • Infant, Newborn
  • Jaundice, Neonatal / diagnosis
  • Jaundice, Neonatal / metabolism
  • Jaundice, Neonatal / physiopathology
  • Jaundice, Neonatal / therapy*
  • Nutritional Status
  • Nutritional Support* / adverse effects
  • Risk Factors
  • Treatment Outcome

Substances

  • Cholagogues and Choleretics