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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한소아소화기영양학회 Pediatric Gastroenterology, Hepatology & Nutrition Pediatric Gastroenterology, Hepatology & Nutrition 제20권 제1호
- 발행연도
- 2017.1
- 수록면
- 55 - 60 (6page)
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초록· 키워드
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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 hypo-ganglionosis 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.
목차
등록된 정보가 없습니다.
참고문헌 (11)
참고문헌 신청
Calhoun AW / 2009 / Omegaven for the treatment of parenteral nutrition associated liver disease: a case study / J Ky Med Assoc 107 : 55 ~ 57
Cooper A / 1984 / Morbidity and mortality of shortbowel syndrome acquired in infancy: an update / J Pediatr Surg 19 : 711 ~ 718
Dingemann J / 2010 / Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect / Pediatr Surg Int 26 : 1111 ~ 1115
Kelly DA / 1998 / Liver complications of pediatric parenteral nutrition--epidemiology / Nutrition 14 : 153 ~ 157
Lacaille F / 2015 / Intestinal failure-associated liver disease:a position paper of the ESPGHAN working group of intestinal failure and intestinal transplantation / J Pediatr
Cooper A / 1984 / Morbidity and mortality of shortbowel syndrome acquired in infancy: an update / J Pediatr Surg 19 : 711 ~ 718
Dingemann J / 2010 / Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect / Pediatr Surg Int 26 : 1111 ~ 1115
Kelly DA / 1998 / Liver complications of pediatric parenteral nutrition--epidemiology / Nutrition 14 : 153 ~ 157
Lacaille F / 2015 / Intestinal failure-associated liver disease:a position paper of the ESPGHAN working group of intestinal failure and intestinal transplantation / J Pediatr
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