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Query: UMLS:C0015695 (fatty liver)
 13,941 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient was treated by home parenteral nutrition because of alimentary failure due to active Crohn's disease, enterocutaneous fistulae, and the short bowel syndrome following massive intestinal resection. This therapy has now been continued for twelve months during which her symptoms, weight, and quality of life have all improved. Complications included an episode of Staph. Albus bacteraemia, and displacement of the replacement catheter. Hepatic steatosis due to excessive dextrose administration because of apparent allergy to Intralipid constitutes an unresolved problem.
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PMID:Home parenteral nutrition: the first twelve months. 680 99

Intestinal failure-associated liver disease develops in 40% to 60% of infants who require long-term total parenteral nutrition (TPN) for intestinal failure and 15% to 40% of adults on home parenteral nutrition. The clinical spectrum includes hepatic steatosis, cholestasis, cholelithiasis, and hepatic fibrosis. Progression to biliary cirrhosis and the development of portal hypertension and liver failure occurs in a minority but is more common in infants and neonates than in adults. The pathogenesis is multifactorial. In infants it is related to prematurity, low birth weight, duration of PN, short bowel syndrome requiring multiple laparotomies, and recurrent sepsis. Other important mechanisms include lack of enteral feeding, which leads to reduced gut hormone secretion; reduction of bile flow and biliary stasis, which leads to the development of cholestasis; and biliary sludge and gallstones, which exacerbate hepatic dysfunction. In adults, IFALD is less common and related to age, length of time on PN, total caloric intake, and lipid or glucose overload. In preterm infants, a deficiency of taurine or cysteine may play a role, whereas in both adults and children, choline deficiency may exacerbate IFALD. Lipid emulsions, choline deficiency, and manganese toxicity are associated with both hepatic steatosis and cholestasis in adults and children. Management strategies for the prevention of intestinal failure-induced liver disease include early enteral feeding, a multidisciplinary approach to the management of parenteral nutrition, and aseptic catheter techniques to reduce sepsis. The addition of choline, taurine, and cysteine to PN solutions may also play a role. Oral administration of ursodeoxycholic acid may improve bile flow and reduce gallbladder stasis. Survival after either isolated small bowel or combined liver and small bowel transplantation is approximately 50% at 5 years, making this an acceptable therapeutic option in adults and children with irreversible liver and intestinal failure.
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PMID:Intestinal failure-associated liver disease: what do we know today? 1647 75

The prevalence of short bowel syndrome appears to be increasing because of more aggressive surgical and medical approaches to the management of neonatal intraabdominal catastrophies. Hence, a large cohort of neonates with intestinal failure occupies neonatal intensive care units, requiring chronic total parenteral nutrition (TPN) in hopes that the residual bowel will adapt, thereby permitting weaning of TPN. Alternatively, when there is no hope for adaptation, these infants are maintained on TPN in hopes that they will grow to a size and state of general health satisfactory for either isolated intestinal transplant when liver function is preserved or combined liver-intestinal transplantation when the liver is irreparably damaged. Thus, it is imperative to provide enough parenteral nutrition to facilitate growth while minimizing TPN constituents predisposing to liver damage. Liver disease associated with intestinal failure (IFALD) seems to occur due to a variety of host factors combined with deleterious components of TPN. Host factors include an immature bile secretory mechanism, bile stasis due to fasting, and repeated septic episodes resulting in endotoxemia. Many constituents of TPN are associated with liver damage. Excessive glucose may result in fatty liver and/or hepatic fibrosis, excessive protein may lead to reduced bile flow, and phytosterols present in intravenous lipid may produce direct oxidant damage to the liver or may impede cholesterol synthesis and subsequent bile acid synthesis. Parenteral strategies employed to minimize TPN damage include reducing glucose infusion rates, reducing parenteral protein load, and reducing parenteral lipid load. Furthermore, preliminary studies suggest that fish oil-based lipid solutions may have a salutary effect on IFALD. Ultimately, provision of enteral nutrition is imperative for preventing or reversing IFALD as well as facilitating bowel adaptation. While studies of trophic hormones are ongoing, the most reliable current method to facilitate adaptation is to provide enteral nutrition. Continuous enteral feeding remains the mainstay of enteral nutrition support.
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PMID:Nutritional management of infants with short bowel syndrome. 1746 95

Parenteral nutrition liver disease (PNLD) develops in 40-60% of infants who require long-term PN for intestinal failure. The clinical spectrum includes hepatic steatosis, cholestasis, cholelithiasis, and hepatic fibrosis. Progression to biliary cirrhosis and the development of portal hypertension and liver failure occurs in a minority who require combined liver and intestinal transplantation. The pathogenesis is multifactorial and is related to prematurity, low birth weight, duration of PN, short bowel syndrome requiring multiple laparotomies and recurrent sepsis. Other important mechanisms include lack of enteral feeding which leads to reduced gut hormone secretion, reduction of bile flow and biliary stasis which leads to the development of cholestasis, biliary sludge and gallstones, which exacerbate hepatic dysfunction, especially in premature neonates with immature hepatic function. The use of lipid emulsions, particularly soy bean emulsions have been associated with hepatic cholestasis in children, although there are little data now to support toxicity from other PN components. Management strategies for the prevention of parenteral nutrition liver disease include consideration of early enteral feeding, a multidisciplinary approach to the management of parenteral nutrition with a specialized nutritional care team and aseptic catheter techniques to reduce sepsis. The use of specialized lipid emulsions such as fish oil emulsions and or SMOF (Soy bean/Medium Chain Triglyceride/Olive Oil/Fish oil) improves established cholestasis and may prevent the onset. Oral administration of ursodeoxycholic acid may improve bile flow and reduce gall bladder stasis, although there is little data to suggest that prophylactic use prevents the onset of PNLD. Survival following either isolated small bowel or combined liver and small bowel transplantation is approximately 50% at 5 years making this an acceptable therapeutic option in children with irreversible liver and intestinal failure.
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PMID:Preventing parenteral nutrition liver disease. 2092 19

Patients developing the short bowel syndrome (SBS) are at risk for hepatobiliary disease, as are morbidly obese individuals. We hypothesized that morbidly obese SBS individuals would be at increased risk for developing hepatobiliary complications. We reviewed 79 patients with SBS, 53 patients with initial body mass index (BMI) < 35 were controls. Twenty-six patients with initial BMI > 35 were the obese group. Obese patients were more likely to be weaned off parenteral nutrition (PN) (58% vs. 21%). Pre-resection BMI was significantly lower in controls (26 vs. 41). BMI at 1, 2, and 5 years was decreased in controls but persistently increased in obese patients. Obese patients were more likely to undergo cholecystectomy prior to SBS (42% vs. 32%) and after SBS (80% vs. 39%, p < 0.05). Fatty liver was more frequent in the obese group prior to SBS (23% vs. 0%, p < 0.05) but was similar to controls after SBS (23% vs. 15%). Fibrosis (8% vs. 13%) and cirrhosis/portal hypertension (19% vs. 21%) were similar in obese and control groups. Overall, end stage liver disease (ESLD) was similar in obese and control groups (19% vs. 11%) but was significantly higher in obese patients receiving PN (45% vs. 14%, p < 0.05). Obese patients developing SBS are at increased risk of developing hepatobiliary complications. ESLD was similar in the two groups overall but occurs more frequently in obese patients maintained on chronic PN.
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PMID:Preresection obesity increases the risk of hepatobiliary complications in short bowel syndrome. 2320 59

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-term complications 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.
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PMID:Fish oil-based lipid emulsion: current updates on a promising novel therapy for the management of parenteral nutrition-associated liver disease. 2585 84

Short bowel syndrome (SBS) is associated with changes in the intestinal microbiome and marked local and systemic inflammation. There is also a late complication of SBS, intestinal failure associated liver disease (IFALD) in which hepatic steatosis progresses to cirrhosis. Most patients with SBS arrive at massive intestinal resection after a contaminating intraabdominal catastrophe and have a history of exposure to broad-spectrum antibiotics. We therefore investigated whether the administration of broad-spectrum antibiotics in conjunction with SBS in zebrafish (ZF) would replicate these systemic effects observed in humans to identify potentially druggable targets to aid in the management of SBS and resulting IFALD. In zebrafish with SBS, broad-spectrum antibiotics altered the microbiome, decreased inflammation, and reduced the development of hepatic steatosis. After two weeks of broad-spectrum antibiotics, these fish exhibited decreased alpha diversity, with less variation in microbial community composition between SBS and sham fish. Additionally, administration of broad-spectrum antibiotics was associated with decreased expression of intestinal toll-like receptor 4 (tlr4), increased expression of the intestinal gene encoding the Farnesoid X receptor (fxr), decreased expression of downstream hepatic cyp7a1, and decreased development of hepatic steatosis. SBS in zebrafish reproducibly results in increased epithelial surface area as occurs in human patients who demonstrate intestinal adaptation, but antibiotic administration in zebrafish with SBS reduced these gains with increased cell death in the intervillus pocket that contains stem/progenitor cells. These alternate states in SBS zebrafish might direct the development of future human therapies.NEW & NOTEWORTHY In a zebrafish model that replicates a common clinical scenario, systemic effects of the administration of broad-spectrum antibiotics in a zebrafish model of SBS identified two alternate states that led to the establishment of fat accumulation in the liver or its absence. Broad-spectrum antibiotics given to zebrafish with SBS over 2 wk altered the intestinal microbiome, decreased intestinal and hepatic inflammation, and decreased hepatic steatosis.
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PMID:Broad-spectrum antibiotics alter the microbiome, increase intestinal fxr, and decrease hepatic steatosis in zebrafish short bowel syndrome. 3259 9