Parenteral nutrition-associated liver disease

Two infants with intestinal failure and parenteral nutrition-associated liver disease were given an intravenous fat emulsion containing primarily omega-3 fatty acids instead of the conventional emulsion [30 ]. Biochemical tests of liver function improved significantly. One child was removed from the liver transplantation list because of improved hepatic function, and the second child had complete resolution of cholestasis while solely on parenteral nutrition. 

Cober MP, Teitelbaum DH. Prevention of parenteral nutrition-associated liver disease lipid minimization. Curr Opin Organ Transplant 2010 15(3) 330-3. 

Le HD, de Meijer VE, Zurakowski D, Meisel JA, Gura KM, Puder M. Parenteral fish oil as monotherapy improves lipid profiles in children with parenteral nutrition-associated liver disease. JPEN J Parenter Enteral Nutr 2010 34(5) 477-84. 

K. M. Gura, S. Lee, C. Valim, J. Zhou, S. Kim, B. P. Modi, D. A. Arsenault, R. A. Strijbosch, S. Lopes, C. Duggan and M. Puder, Safety and efficacy of a fish-oil-based fat emulsion in the treatment of parenteral nutrition-associated liver disease. 

In a prospective cohort study including 24 infants, the incidence and risk factors of parenteral nutrition-associated liver disease (PNALD) was determined. Eight infants developed PNALD. The concluded that the duration of enteral starvation, gastrointestinal surgery, duration of enteral nutrition, maximum caloric and carbohydrate intakes were significant risks of PNALD in newborn infants [lob ll. In a retrospective review of the safety and efficacy of PN among 105 paediatric patients with bum injuries (>30% total-body sxuface area), no respiratory or blood infections were observed with the use of parenteral nutrition, and the overall mortality rate was 4% [107 ]. 

Death Parenteral-nutrition-associated liver disease after intestinal perforation, with resulting/afaZifi/ has been... 

RoUins MD, Ward RM, Jackson WD, Mulroy CW, Spencer CP, Ying J, et al. Effect of decreased parenteral soybean lipid emulsion on hepatic function in infants at risk for parenteral nutrition-associated liver disease a pilot study. J Pediatr Surg Jime 2013 48(6) 1348-56. 

Kubota A, Mochizuki N, Shiraishi J, NakayamaM, Kawahara H, YonedaA, et al. Parenteral-nutrition-associated liver disease after intestinal perforation in extremely low-birthweight infants consequent lethal portal hypertension. Pediatr Int February 2013 55(l) 39-43. 

In a prospective prevalence study of liver disease in 90 patients with permanent intestinal failure receiving parenteral nutrition hver biopsy was performed in 57 (95). Chronic cholestasis developed in 58 patients after a median of 6 (range 3-132) months, and 37 developed comphcated liver disease after a median of 17 (range 2-155) months. Chronic cholestasis was significantly associated with a risk of liver disease independent of parenteral nutrition, a bowel remnant shorter than 50 cm, and a lipid intake of 1 g/kg/day or more hver disease related to parenteral nutrition was significantly associated with chronic cholestasis and a parenteral hpid intake of 1 g/kg/day or more. The authors concluded that the prevalence of hver disease increased with the duration of parenteral nutrition and was one of the main causes of death in patients with permanent intestinal failure. Parenteral intake of long-chain hpid emulsion should be restricted to less than 1 g/kg/day. 

A fish oil-based intravenous lipid emulsion in the treatment of liver disease associated with parenteral nutrition has been compared with soybean oil in an open study in 42 infants with short bowel syndrome who developed cholestasis [35 ]. There were three deaths and one liver transplantation in those who received the fish oil, compared with 12 deaths and 6 transplants in those who received soybean oil The fish oil was not associated with hypertriglyceridemia, coagulopathy, or deficiency of essential fatty acids. 

Santra S, McKiernan P, Lander A, Dalzell AM, Baillie C, Beath S, Gupte GL. Ischemic hepatitis is a risk factor for progression of liver disease associated with parenteral nutrition in intestinal failure. J Pediatr Gastroenterol Nutr 2008 47(3) 367-9. 

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