title: Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of Omegaven™.
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 Omegaven™, a fat emulsion comprised of omega-3 fatty acids. Omegaven™ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with Omegaven™ 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 Omegaven™. 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 Omegaven™.
authors.affiliation: Department of Medical Education, Johns Hopkins All Children's Hospital, St. Petersburg, FL, USA.
authors.forename: Racha
authors.initials: R
authors.lastname: Khalaf
authors.affiliation: Department of Gatroenterology, Hepatology and Nutrition, Johns Hopkins All Children's Hospital, St. Petersburg, FL, USA.
authors.forename: Sara
authors.initials: S
authors.lastname: Karjoo
authors.affiliation: Department of Surgery, Johns Hopkins All Children's Hospital, St. Petersburg, FL, USA.
authors.forename: Paul
authors.initials: P
authors.lastname: Danielson
authors.affiliation: Department of Gatroenterology, Hepatology and Nutrition, Johns Hopkins All Children's Hospital, St. Petersburg, FL, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Wilsey
authors.affiliation: Department of Neonatology, Johns Hopkins All Children's Hospital, St. Petersburg, FL, USA.
authors.forename: Fauzia
authors.initials: F
authors.lastname: Shakeel
keywords: Hirschsprung disease; Liver diseases; Omega-3 fatty acids; Total parenteral nutrition