Reversal of Parenteral Nutrition-Associated Liver Disease in Two Infants With Short Bowel Syndrome Using Parenteral Fish Oil: Implications for Future Management (original) (raw)
Related papers
2014
The use of fish oil-based lipid emulsions (FOLE) in the treatment of intestinal failure-associated liver disease (IFALD) remains investigational. Additional evidence for safety and efficacy, particularly in the neonatal and pediatric populations, is needed. Retrospective chart review was conducted on 10 infants with short bowel syndrome who received FOLE for IFALD. Direct bilirubin concentrations normalized in surviving subjects within 4.1 to 22.7 weeks of starting treatment. Although earlier initiation of FOLE was not associated with more rapid normalization of direct bilirubin concentrations, it trended toward a significant correlation with reduced length of hospital stay (P = .058). The reduction in direct bilirubin levels and transition from parenteral to enteral feeding were statistically significant within 6 weeks of initiating the FOLE. Subjects did not have impaired growth and did not develop an essential fatty acid deficiency. These infants were discharged from the hospital 7.9 to 42.3 weeks after starting FOLE treatment, and 2 infants had transitioned completely off parenteral nutrition at discharge. In this study, FOLE appeared to be a safe and effective treatment for IFALD in infants with short bowel syndrome. Future studies are necessary to determine whether FOLE can help to prevent or shorten the duration of cholestasis. Keywords: hepatology/liver diseases; intravenous fish oil-based lipid emulsion; infant; early lifespan nutrition; newborn or neonate; gastrointestinal disorders; enteral and parenteral feeding; nutrition support/special diets Background
Journal of Pediatric Surgery, 2011
Although premature infants with short bowel syndrome are at the highest risk of developing intestinal failure-associated liver disease (IFALD), they have great capacity for intestinal growth and adaptation if IFALD can be prevented. Conventional soybean oil-based intravenous lipid emulsions have been associated with IFALD. This study presents data on 5 premature neonates with short bowel syndrome treated with a combination of parenteral fish oil-and olive/soybean-based lipid emulsion for periods ranging between 7 and 17 months. Despite an enteral tolerance of less than 50% in 4 of these patients during their first year of life, direct bilirubin levels normalized while on this combination of ClinOleic (Baxter, Maurepas, France)/Omegaven (Fresenius Kabi, Bad Homburg, Germany) at a 1:1 ratio. None of our patients developed irreversible IFALD even though all of them were premature, had undergone multiple major surgical procedures, and had experienced several episodes of sepsis. Thus far, we have not seen any adverse effects of this mixed lipid emulsion in these preterm infants. All 5 patients are growing and developing well and have normal liver function.
Pharmacotherapy, 2011
Study Objective. To evaluate the use of enteral fish oil for the treatment of parenteral nutrition-associated liver disease (PNALD). Design. Retrospective case series. Setting. Pediatric academic hospital and outpatient clinic. Patients. Six parenteral nutrition-dependent infants with short-bowel syndrome and PNALD. Measurements and Main Results. The six infants received supplementation with enteral fish oil, and treatment was evaluated over a 12-week period. The PNALD, as reflected by elevated total bilirubin levels, completely reversed in four of the six infants within a mean ± SD of 5 ± 2.6 weeks (range 2-8 wks) after initiation of the enteral fish oil supplementation. In addition, improvement in enteral feedings occurred after starting enteral fish oil therapy. Conclusion. Enteral fish oil may be an effective adjunctive treatment option for infants with PNALD, particularly for those infants with PNALD who are tolerating some amount of enteral nutrition as the result of an adequate amount of small bowel.
Canadian Journal of Gastroenterology, 2012
OBJECTIVE: To describe the authors’ experience with the implementation of a multidisciplinary approach and use of fish oil emulsion (FOE) in the management of infants with short bowel syndrome (SBS) and parenteral nutrition-associated liver disease (PNALD).METHODS: Between August 2006 and June 2009, four cases of SBS and severe PNALD were managed by the team using specifically developed protocols. The FOE was initiated if serum direct bilirubin levels were ≥100 μmol/L. To quantify the degree of exposure to high serum direct bilirubin levels over time, the area under the curve (AUC) for each patient was calculated before and after initiation of FOE. Linear regression analyses were performed to evaluate correlations between the AUC, duration of cholestasis and initiation of FOE.RESULTS: All patients survived and no complications were observed during the study period. After the first patient, FOE was initiated progressively earlier, but poor correlation between the AUC before and after...
Reports
Intestinal-failure-associated liver disease (IFALD) is a common complication of prolonged parenteral nutrition (PN). Risk factors for IFALD include clinical features, as well as medical interventions, and its management was initially based on the decrease or interruption of parenteral nutrition while increasing enteral nutrition. However, the tolerance of full enteral nutrition in children with intestinal failure may require prolonged intestinal rehabilitation over a period of years. As a consequence, infants unable to wean from PN are prone to develop end-stage liver disease. We describe the case of an infant receiving long-term PN who was diagnosed with IFALD wherein we were able to reverse IFALD by switching lipid emulsions to fish oil monotherapy. A systemic review of case reports and case series on reversing IFALD using fish oil lipid emulsion follows the case description.
Advances in nutrition (Bethesda, Md.), 2014
We previously reported the beneficial effect of fish oil-based lipid emulsions (FOLEs) as monotherapy in the treatment of parenteral nutrition-associated liver disease (PNALD). In this report, we share our ongoing experience at Texas Children's Hospital, Houston, Texas in the use of FOLE in treatment of PNALD as presented at the 2013 Experimental Biology meeting. We describe the findings of a single center, prospective, observational study of infants <6 mo of age with PNALD who received parenteral FOLE as monotherapy. A total of 97 infants received FOLE under the compassionate-use protocol for the treatment of PNALD. Eighty-three (86%) survived with resolution of cholestasis and 14 (14%) died. The median conjugated bilirubin (CB) concentration at the initiation of FOLE therapy was 4.8 mg/dL (range 2.1-26). The median time to resolution of cholestasis was 40 d (range 3-158). Compared with infants with mild cholestasis (CB of 2.1-5 mg/dL at the initiation of FOLE), nonsurvivors...
Prevention of parenteral nutrition-associated liver disease: role of omega-3 fish oil
Current opinion in organ transplantation, 2010
Parenteral nutrition-associated liver disease (PNALD) is the most severe complication of long-term parenteral nutrition. Its cause remains unclear, although recent studies suggest that the omega-6 polyunsaturated fatty acids in plant oil-based lipid emulsions and the associated phytosterols contribute to the development of hepatotoxicity. In contrast, fish oil-based lipid emulsions are composed mainly of omega-3 polyunsaturated fatty acids and are hypothesized to be hepatoprotective. This review will discuss fish oil-based lipid emulsions in the prevention of PNALD. In several animal models of PNALD, the use of an intravenous fish oil-based lipid emulsion improved parenteral nutrition-associated cholestasis without resultant essential fatty acid deficiency or growth impairment. Following these results and preliminary human data, an open trial for compassionate use was initiated, followed by a randomized controlled trial to evaluate the current management of pediatric PNALD. To date,...