Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of Omegaven™ / 대한소아소화기영양학회지
Pediatric Gastroenterology, Hepatology & Nutrition
;
: 55-60, 2017.
Article
in English
| WPRIM
| ID: wpr-28079
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™.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Aspartate Aminotransferases
/
United States Food and Drug Administration
/
Bilirubin
/
Fatty Acids, Omega-3
/
Cholestasis
/
Parenteral Nutrition, Total
/
Diagnosis
/
Alanine Transaminase
/
Alkaline Phosphatase
/
Empathy
Type of study:
Diagnostic study
/
Practice guideline
Limits:
Humans
/
Infant, Newborn
Language:
English
Journal:
Pediatric Gastroenterology, Hepatology & Nutrition
Year:
2017
Type:
Article
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