Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of OmegavenTM
0
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
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.
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 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.
참고문헌 (11)
- Schäppi MG, "A practical guide for the diagnosis of primary enteric nervous system disorders", J Pediatr Gastroenterol Nutr, 57, pp.677~686 (2013)
- Dingemann J, "Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect", Pediatr Surg Int, 26, pp.1111~1115 (2010)
- Le HD, "Parenteral fishoil-based lipid emulsion improves fatty acid profiles and lipids in parenteral nutrition-dependent children", Am J Clin Nutr, 94, pp.749~758 (2011)
- Park HW, "Parenteral fish oil-containing lipid emulsions may reverse parenteral nutrition-associated cholestasis in neonates:a systematic review and meta-analysis", J Nutr, 145, pp.277~283 (2015)
- Raptis DA, "GPR120 on Kupffer cells mediates hepatoprotective effects of ω3-fatty acids", J Hepatol, 60, pp.625~632 (2014)
- Venick RS, "The impact of intravenous fish oil emulsions on pediatric intestinal failure-associated liver disease", Curr Opin Organ Transplant, 16, pp.306~311 (2011)
- Kelly DA, "Liver complications of pediatric parenteral nutrition--epidemiology", Nutrition, 14, pp.153~157 (1998)
- Lacaille F, "Intestinal failure-associated liver disease:a position paper of the ESPGHAN working group of intestinal failure and intestinal transplantation", J Pediatr Gastroenterol Nutr, 60, pp.272~283 (2015)
- Calhoun AW, "Omegaven for the treatment of parenteral nutrition associated liver disease: a case study", J Ky Med Assoc, 107, pp.55~57 (2009)
- Cooper A, "Morbidity and mortality of shortbowel syndrome acquired in infancy: an update", J Pediatr Surg, 19, pp.711~718 (1984)
- Sondheimer JM, "Infection and cholestasis in neonates with intestinal resection and long-term parenteral nutrition", J Pediatr Gastroenterol Nutr, 27, pp.131~137 (1998)