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 language | English (US) |
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Pages (from-to) | 55-60 |
Number of pages | 6 |
Journal | Pediatric Gastroenterology, Hepatology and Nutrition |
Volume | 20 |
Issue number | 1 |
DOIs | |
State | Published - 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