Intestinal hypoganglionosis leading to intestinal failure and the compassionate use of OmegavenTM

Racha Khalaf, Sara Karjoo, Paul Danielson, Michael Wilsey, Fauzia Shakeel

Research output: Contribution to journalArticlepeer-review

Abstract

Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to OmegavenTM, a fat emulsion comprised of omega-3 fatty acids. OmegavenTM has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with OmegavenTM was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and complete resolution of cholestasis with a direct bilirubin of zero within two weeks of initiation of OmegavenTM. No current guidelines for the diagnosis and management of hypoganglionosis are available. We recommend a multidisciplinary approach and the use of novel therapies such as fat emulsions composed of omega-3 fatty acids for improved patient outcomes. Appropriate compassionate use protocols should be obtained from the Food and Drug Administration prior to initiation of OmegavenTM.

Original languageEnglish (US)
Pages (from-to)55-60
Number of pages6
JournalPediatric Gastroenterology, Hepatology and Nutrition
Volume20
Issue number1
DOIs
StatePublished - Mar 1 2017

Keywords

  • Hirschsprung disease
  • Liver diseases
  • Omega-3 fatty acids
  • Total parenteral nutrition

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hepatology
  • Gastroenterology

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