Liver failure parenteral nutrition

The indication for administering BCAA in patients with hepatic encephalopathy to compensate amino-acid imbalance was proposed by J.E. Fischer et al. in 1974, and implemented parenterally. However, oral application of BCAA for an adequate treatment period also has beneficial effects on cirrhosis and HE (7.) improvement in protein tolerance and the nutritional condition, (2.) improvement in cerebral functions (II8, 122), probably due to an amelioration of liver function, (2.) stimulation of ammonia detoxification with a positive nitrogen balance (118), (4.) reduction in or normalization of AAA levels, and (5.) promotion of glutamine synthesis with a favourable effect on the cells of the immune system and on renal function. By means of BCAA, it was possible to prolong the survival time and delay the occurrence of liver failure in rats with CC -induced cirrhosis. (123, 126) However, there are diverging results, which need further clarification. In principle, the use of BCAA is considered to be a necessary form of supplementary treatment for catabolic metabolism in cirrhosis (124,125, 127, 128, 130-132), in (also latent) HE and after curative resection of hepatocellular carcinoma. (I2l) (s. p. 280)... 

Alpers, D.H. Liver complications and failure in patients on home parenteral nutrition. Gastroenterology 2001 17 147—149... 

Intestinal transplantation is combined with liver transplantation in 46% of cases, because of terminal liver failure (93). Of 78 patients who had received parenteral nutrition for more than 2 years n — 66) and/ or had short bowel syndrome and could not be weaned from parenteral nutrition (n = 12), 58 developed chronic cholestasis and 37 developed one or more severe liver complication (serum bilirubin concentration 60 pmol/l, factor V (proaccelerin) 50%, portal hypertension, encephalopathy, ascites, bleeding from the gastrointestinal tract, or histological findings consisting of extensive fibrosis and cirrhosis) after 6 (3-132) months and 17 (2-155) months respectively. Liver disease was responsible for deaths in 6.5% of the patients (22% of deaths). 

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. 

In a retrospective study of the incidence of cholestasis and liver failure in 42 patients with intestinal resection in the neonatal period who subsequently became dependent on parenteral nutrition support, the effect of various associated clinical factors on the incidence and severity of cholestasis was determined (103). Cholestasis developed in 28 while they were receiving parenteral nutrition. In 21 patients, the raised direct bilirubin concentration returned to normal while they continued to receive parenteral nutrition. Seven patients progressed to liver failure. Patients without cholestasis had been dependent on parenteral nutrition for longer than patients with cholestasis. It was clear from this study that cholestasis in neonates with intestinal resection is not simply a function of the duration of exposure to intravenous nutrition. 

Fortunately, bed rest, rehydration, parenteral nutrition, and therapy directed at decreasing the production of toxins that result from bacterial degradation of nitrogenous substrates in the gut lumen (e.g., administration of lactulose, which reduces gut ammonia levels by a variety of mechanisms, the use of enemas and antibiotics to decrease the intestinal flora, a low-protein diet) prevented Percy Veere from progressing to the later stages of hepatic encephalopathy. As with most patients who survive an episode of fulminant hepatic failure, recovery to his previous state of health occurred over the next 3 months. Percy s liver function studies returned to normal, and a follow-up liver biopsy showed no histologic abnormalities. 

Liver damage is a common complication of parenteral nutrition and has been reviewed [25 ]. For example, it develops in 40-60% of infants who require long-term parenteral nutrition for intestinal failure. The clinical spectrum includes hepatic steatosis,... 

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. 

In contrast, in a retrospective analysis of 292 neonates who received parenteral nutrition with lipid emulsions containing omega-3 fatty acids for more than 1 day, 104 (36%) developed cholestasis after a mean of 22 days, with a conjugated bilirubin concentration over 34 pmol/l 31 had a serum conjugated bilirubin concentration over 100 pmoUl and 13 developed liver failure 4 underwent transplantation and 5 died of hepatic disease [385]. The authors suggested that in the absence of definitive evidence of efficacy, as well as increased costs, it is difficult to justify the routine use of lipid... 

Progression of liver disease during parenteral nutrition in two infants with intestinal failure was rapidly exacerbated by ischemic liver damage [67 ]. 

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. 

Liver The effect of weaning patients off PN and its relationship to liver histology has been evaluated in a study including 38 patients with paediatric intestinal failure. Abnormal histology was foxmd in 77% of patients weaned of parenteral nutrition. Portal inflammation and fibrosis were also observed [117 ]. In a prospective cohort study to determine the incidence and risk factors for development of abnormal LFTs in patients on short-term PN (8-54 days mean 15.9 days), 57.5% of 80 patients had abnormal LFTs [118 ]. Septic shock, hyperglycaemia, soybean lipid and absolute diet were associated with an increased risk of abnormal LFTs. 

Mutanen A, Lohi J, Heikkila P, Koivusalo Al, Rintala RJ, Pakarinen MP. Persistent abnormal liver fibrosis after weaning off parenteral nutrition in pediatric intestinal failure. Hepatology August 2013 58(2) 729-38. 

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