BACKGROUND: Parenteral nutrition-associated liver disease (PNALD) has been common in patients who require long-term parenteral nutrition. PNALD develops in 40%-60% of infants on long-term parenteral nutrition compared...BACKGROUND: Parenteral nutrition-associated liver disease (PNALD) has been common in patients who require long-term parenteral nutrition. PNALD develops in 40%-60% of infants on long-term parenteral nutrition compared with 15%-40% of adults on home parenteral nutrition for intestinal failure. The pathogenesis of PNALD is multifactorial and remains unclear There is no specific treatment. Management strategies for its prevention and treatment depend on an understanding of many risk factors. This review aims to provide an update on the pathogenesis and treatment of this disease. DATA SOURCES: A literature search was performed on the MEDLINE and Web of Science databases for articles published up to October 2011, using the keywords: parenteral nutrition associated liver disease, intestinal failure associated liver disease lipid emulsions and fish oil. The available data reported in the relevant literatures were analyzed. RESULTS: The literature search provided a huge amount of evidence about the pathogenesis and management strategies on PNALD. Currently, lack of enteral feeding, extended duration of parenteral nutrition, recurrent sepsis, and nutrient deficiency or excess may play important roles in the pathogenesis of PNALD. Recent studies found that phytosterols present as contaminants in soy-based lipid emulsions, are also an important factor in the pathogenesis. Moreover, the treatment of PNALD is discussed. CONCLUSIONS: The use of lipid emulsions, phytosterols in particular, is associated with PNALD. Management strategies for the prevention and treatment of PNALD include consideration of early enteral feeding, the use of specialized lipid emulsions such as fish oil emulsions, and isolated small bowel or combined liver and small bowel transplantation. A greater understanding of the pathogenesis of PNALD has led to promising interventions to prevent and treat this condition. Future work should aim to better understand the mechanisms of PNALD and the long-term outcomes of its treatment.展开更多
Intestinal failure is characterized by loss of enteral function to absorb necessary nutrients and water to sustain life.Parenteral nutrition(PN)is a lifesaving therapeutic modality for patients with intestinal failure...Intestinal failure is characterized by loss of enteral function to absorb necessary nutrients and water to sustain life.Parenteral nutrition(PN)is a lifesaving therapeutic modality for patients with intestinal failure.Lifelong PN is also needed for patients who have short bowel syndrome due to extensive resection or a dysmotility disorder with malabsorption.However,prolonged PN is associated with short-term and long-term complications.Parenteral nutrition-associated liver disease(PNALD)is one of the long-termcomplications associated with the use of an intravenous lipid emulsion to prevent essential fatty acid deficiency in these patients.PNALD affects 30–60%of the adult population on long-term PN.Further,PNALD is one of the indications for isolated liver or combined liver and intestinal transplantation.There is no consensus on how to manage PNALD,but fish oil-based lipid emulsion(FOBLE)has been suggested to play an important role both in its prevention and reversal.There is significant improvement in liver function in those who received FOBLE as lipid supplement compared with those who received soy-based lipid emulsion.Studies have also demonstrated that FOBLE reverses hepatic steatosis and reduces markers of inflammation in patients on long-term PN.Future prospective studies with larger sample sizes are needed to further strengthen the positive role of FOBLE in PNALD.展开更多
文摘BACKGROUND: Parenteral nutrition-associated liver disease (PNALD) has been common in patients who require long-term parenteral nutrition. PNALD develops in 40%-60% of infants on long-term parenteral nutrition compared with 15%-40% of adults on home parenteral nutrition for intestinal failure. The pathogenesis of PNALD is multifactorial and remains unclear There is no specific treatment. Management strategies for its prevention and treatment depend on an understanding of many risk factors. This review aims to provide an update on the pathogenesis and treatment of this disease. DATA SOURCES: A literature search was performed on the MEDLINE and Web of Science databases for articles published up to October 2011, using the keywords: parenteral nutrition associated liver disease, intestinal failure associated liver disease lipid emulsions and fish oil. The available data reported in the relevant literatures were analyzed. RESULTS: The literature search provided a huge amount of evidence about the pathogenesis and management strategies on PNALD. Currently, lack of enteral feeding, extended duration of parenteral nutrition, recurrent sepsis, and nutrient deficiency or excess may play important roles in the pathogenesis of PNALD. Recent studies found that phytosterols present as contaminants in soy-based lipid emulsions, are also an important factor in the pathogenesis. Moreover, the treatment of PNALD is discussed. CONCLUSIONS: The use of lipid emulsions, phytosterols in particular, is associated with PNALD. Management strategies for the prevention and treatment of PNALD include consideration of early enteral feeding, the use of specialized lipid emulsions such as fish oil emulsions, and isolated small bowel or combined liver and small bowel transplantation. A greater understanding of the pathogenesis of PNALD has led to promising interventions to prevent and treat this condition. Future work should aim to better understand the mechanisms of PNALD and the long-term outcomes of its treatment.
文摘Intestinal failure is characterized by loss of enteral function to absorb necessary nutrients and water to sustain life.Parenteral nutrition(PN)is a lifesaving therapeutic modality for patients with intestinal failure.Lifelong PN is also needed for patients who have short bowel syndrome due to extensive resection or a dysmotility disorder with malabsorption.However,prolonged PN is associated with short-term and long-term complications.Parenteral nutrition-associated liver disease(PNALD)is one of the long-termcomplications associated with the use of an intravenous lipid emulsion to prevent essential fatty acid deficiency in these patients.PNALD affects 30–60%of the adult population on long-term PN.Further,PNALD is one of the indications for isolated liver or combined liver and intestinal transplantation.There is no consensus on how to manage PNALD,but fish oil-based lipid emulsion(FOBLE)has been suggested to play an important role both in its prevention and reversal.There is significant improvement in liver function in those who received FOBLE as lipid supplement compared with those who received soy-based lipid emulsion.Studies have also demonstrated that FOBLE reverses hepatic steatosis and reduces markers of inflammation in patients on long-term PN.Future prospective studies with larger sample sizes are needed to further strengthen the positive role of FOBLE in PNALD.