Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of Omegaven™ / 대한소아소화기영양학회지
Pediatric Gastroenterology, Hepatology & Nutrition
;
: 55-60, 2017.
Artigo
em Inglês
| 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:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Aspartato Aminotransferases
/
United States Food and Drug Administration
/
Bilirrubina
/
Ácidos Graxos Ômega-3
/
Colestase
/
Nutrição Parenteral Total
/
Diagnóstico
/
Alanina Transaminase
/
Fosfatase Alcalina
/
Empatia
Tipo de estudo:
Estudo diagnóstico
/
Guia de Prática Clínica
Limite:
Humanos
/
Recém-Nascido
Idioma:
Inglês
Revista:
Pediatric Gastroenterology, Hepatology & Nutrition
Ano de publicação:
2017
Tipo de documento:
Artigo
Similares
MEDLINE
...
LILACS
LIS