The association of cyclic parenteral nutrition and decreased incidence of cholestatic liver disease in patients with gastroschisis

Jensen, Aaron R.; Goldin, Adam B.; Koopmeiners, Joseph S.; Stevens, Jennifer A.; Waldhausen, John H.T.; Kim, Stephen S.

doi:10.1016/j.jpedsurg.2008.10.033

Cited by 63 publications

(34 citation statements)

References 33 publications

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“…In small infants without enteral feeds, cycling off may be limited to 6 hours 50 . In a retrospective review of neonates with gastroschisis, cholestasis at 50 days was observed in 9.8% of the cycled group versus 48.8% in the group 51 receiving continuous PN. We routinely provide PN in a cycled fashion as soon as metabolic and fluid status allow.…”

Section: Cyclingmentioning

confidence: 99%

Prevention and Treatment of Intestinal Failure-Associated Liver Disease in Children

Raphael

Duggan

2013

Semin Liver Dis

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Intestinal failure-associated liver disease (IFALD), a serious complication occurring in infants, children and adults exposed to long-term parenteral nutrition (PN), causes a wide-spectrum of disease, ranging from cholestasis and steatosis to fibrosis and eventually cirrhosis. Known host risk factors for IFALD include low birth weight, prematurity, short bowel syndrome and recurrent sepsis. The literature suggests that components of PN may also play a part of the multifactorial pathophysiology. Because some intravenous lipid emulsions (ILE) may contribute to inflammation and interfere with bile excretion, treatment with ILE minimization and/or ILEs composed primarily of omega-3 fatty acids can be helpful but requires careful monitoring for growth failure and essential fatty acid deficiency (EFAD). Data from randomized controlled trials are awaited to support widespread use of these approaches. Other IFALD treatments include cycling PN, ursodeoxycholic acid, sepsis prevention, photoprotection and polyvinylchloride-free tubing. Management and prevention of IFALD remains a clinical challenge.

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Section: Cyclingmentioning

confidence: 99%

Prevention and Treatment of Intestinal Failure-Associated Liver Disease in Children

Raphael

Duggan

2013

Semin Liver Dis

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“…Other treatment options, include reduction of lipid dose to less than 1 gram/kg/day and cyclic PN infusions, have had mixed results. 10–13 Likewise, pharmacologic interventions such as treatment with metronidazole, ursodeoxycholic acid, or choline, have had moderate success. 14 In refractory hepatic failure in the setting of intestinal failure, liver transplantation (with or without small bowel) remains the only option.…”

Section: Introductionmentioning

confidence: 99%

Parenteral Fish Oil Improves Outcomes in Patients With Parenteral Nutrition-Associated Liver Injury

et al. 2009

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OBJECTIVE The objective was to determine the safety and efficacy of a fish oil-based intravenous lipid emulsion (IFE) in the treatment of PNALD. SUMMARY AND BACKGROUND DATA Parenteral nutrition-associated liver disease (PNALD) can be a lethal complication in children with short bowel syndrome (SBS). IFE based on soybean oil administered with parenteral nutrition (PN) may contribute to its etiology. METHODS We performed an open-labeled trial of a fish-oil IFE in 42 infants with SBS who developed cholestasis (serum direct bilirubin > 2 mg/dL) while receiving soybean IFE. Safety and efficacy outcomes were compared with those from a contemporary cohort of 49 infants with SBS and cholestasis whose PN course included soybean IFE only. The primary efficacy end-point was time to reversal of cholestasis (direct bilirubin ≤2 mg/dL). RESULTS Three deaths and 1 liver transplantation occurred in the fish oil cohort, compared to 12 deaths and 6 transplants in the controls (P=0.005). Among survivors not transplanted during PN, cholestasis reversed while receiving PN in 19/38 patients in the fish oil cohort vs. 2/36 patients in the controls. Based on Cox models, subjects receiving fish oil-IFE experienced reversal of cholestasis 6 times faster (95% CI=2.0,37.3) than those receiving soybean IFE. The provision of fish oil IFE was not associated with hypertriglyceridemia, coagulopathy, essential fatty acid deficiency. Moreover, hypertriglyceridemic events and abnormal INR levels were more common among controls. CONCLUSIONS Fish oil IFE is safe, may be effective in treating PNALD, and may reduce mortality and organ transplantation rates in children with SBS.

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“…In neonates, cycling should be limited to 6 hrs off PN due to diminished glycemic reserves (10, 11). In a recent retrospective study evaluating cycling PN in infants with gastroschisis, the cycling PN group was almost 3 times less likely to develop IFALD compared to the continuous group (12). The difference, however, was not statistically significant as the sample size was small.…”

Section: Parenteral Nutritionmentioning

confidence: 99%

Innovative parenteral and enteral nutrition therapy for intestinal failure

Fallon

Meijer

et al. 2010

Seminars in Pediatric Surgery

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Children with intestinal failure suffer from insufficient intestinal length or function, making them dependent on parenteral nutrition (PN) for growth and survival. PN and its components are associated with many complications ranging from simple electrolyte abnormalities to life-threatening PNassociated liver disease, which is also called intestinal failure-associated liver disease (ILALD). From a nutrition perspective, the ultimate goal is to provide adequate caloric requirements and make the transition from PN to full enteral Nutrition (EN) successful. Upon review of the literature, we have summarized the most effective and innovative PN and EN therapies for this patient population. Antibiotic-coated catheters and antibiotic or ethanol locks can be implemented, as they appear effective in reducing catheter-related infection and thus further reduce the risk of IFALD. Lipid emulsions should be given judiciously. The use of an omega-3 fatty acid-based formulation should be considered in patients who develop IFALD. Trophic feeding is important for intestinal adaptation, and EN should be initiated early to help wean patients from PN. Long term management of children with IF continues to be an emerging field. We have entered uncharted territory as more children survive complications of IF, including IFALD. Careful monitoring and individualized management to ensure maintenance of growth with avoidance of complications are the keys to successful patient outcomes.

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The association of cyclic parenteral nutrition and decreased incidence of cholestatic liver disease in patients with gastroschisis

Cited by 63 publications

References 33 publications

Prevention and Treatment of Intestinal Failure-Associated Liver Disease in Children

Prevention and Treatment of Intestinal Failure-Associated Liver Disease in Children

Parenteral Fish Oil Improves Outcomes in Patients With Parenteral Nutrition-Associated Liver Injury

Innovative parenteral and enteral nutrition therapy for intestinal failure

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