Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of Omegaven™ / 대한소아소화기영양학회지
Pediatric Gastroenterology, Hepatology & Nutrition
;
: 55-60, 2017.
Artículo
en Inglés
| 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™.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Aspartato Aminotransferasas
/
United States Food and Drug Administration
/
Bilirrubina
/
Ácidos Grasos Omega-3
/
Colestasis
/
Nutrición Parenteral Total
/
Diagnóstico
/
Alanina Transaminasa
/
Fosfatasa Alcalina
/
Empatía
Tipo de estudio:
Estudio diagnóstico
/
Guía de Práctica Clínica
Límite:
Humanos
/
Recién Nacido
Idioma:
Inglés
Revista:
Pediatric Gastroenterology, Hepatology & Nutrition
Año:
2017
Tipo del documento:
Artículo
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