Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
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Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of OmegavenTM

  • Khalaf, Racha (Department of Medical Education, Johns Hopkins All Children's Hospital) ;
  • Karjoo, Sara (Department of Gatroenterology, Hepatology and Nutrition, Johns Hopkins All Children's Hospital) ;
  • Danielson, Paul (Department of Surgery, Johns Hopkins All Children's Hospital) ;
  • Wilsey, Michael (Department of Gatroenterology, Hepatology and Nutrition, Johns Hopkins All Children's Hospital) ;
  • Shakeel, Fauzia (Department of Neonatology, Johns Hopkins All Children's Hospital)
  • Received : 2016.02.25
  • Accepted : 2016.05.07
  • Published : 2017.03.30
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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 $Omegaven^{TM}$, a fat emulsion comprised of omega-3 fatty acids. $Omegaven^{TM}$ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with $Omegaven^{TM}$ 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 $Omegaven^{TM}$. 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 $Omegaven^{TM}$.

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References

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