Implementation of a Multidisciplinary Team Approach and Fish Oil Emulsion Administration in the Management of Infants with Short Bowel Syndrome and Parenteral Nutrition-Associated Liver Disease

Sant’Anna, Ana; Altamimi, Eyad; Saab, Joanne; Mileski, Heather; Cameron, Brian H.; Fitzgerald, Peter; Sant’Anna, Guilherme M.

doi:10.1155/2012/571829

Cited by 15 publications

(11 citation statements)

References 20 publications

(20 reference statements)

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“…Historically, children who progressed to cirrhosis from PNALD and were unable to wean to enteral nutrition frequently required liver transplantation or succumbed to ESLD 6,7 . With the introduction of reduced SO lipid dosing and parenteral FO therapy, mortality from PNALD has decreased for most children, from approximately 25% prior to 2004 to less than 10% in recent years 15,18–20 .…”

Section: Discussionmentioning

confidence: 99%

The Natural History of Cirrhosis From Parenteral Nutrition-Associated Liver Disease After Resolution of Cholestasis With Parenteral Fish Oil Therapy

Nandivada¹,

Chang²,

Potemkin³

et al. 2015

Annals of Surgery

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Objective To determine the natural history of cirrhosis from parenteral nutrition-associated liver disease (PNALD) after resolution of cholestasis with fish oil (FO) therapy Background Historically, cirrhosis from PNALD resulted in end-stage liver disease (ESLD), often requiring transplantation for survival. With FO therapy, most children now experience resolution of cholestasis and rarely progress to ESLD. However, outcomes for cirrhosis after resolution of cholestasis are unknown and patients continue to be considered for liver/multivisceral transplantation. Methods Prospectively collected data was reviewed for children with cirrhosis due to PNALD who had resolution of cholestasis after treatment with FO from 2004 to 2012. Outcomes evaluated included need for liver/multivisceral transplantation, mortality, and the clinical progression of liver disease. Results Fifty-one patients with cirrhosis from PNALD were identified, with 76% demonstrating resolution of cholestasis after FO therapy. The mean direct bilirubin decreased from 6.4 ± 4 mg/dL to 0.2 ± 0.1 mg/dL (p <0.001) 12 months after resolution of cholestasis, with a mean time to resolution of 74 days. None of the patients required transplantation or died from ESLD. Pediatric End-Stage Liver Disease (PELD) scores decreased from 16 ± 4.6 to −1.2 ± 4.6, 12 months after resolution of cholestasis (p <0.001). In children who remained PN-dependent, the PELD score remained normal throughout the follow-up period. Conclusions Cirrhosis from PNALD may be stable rather than progressive once cholestasis resolves with FO therapy. Furthermore, these patients may not require transplantation and show no clinical evidence of liver disease progression, even when persistently PN-dependent.

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Section: Discussionmentioning

confidence: 99%

The Natural History of Cirrhosis From Parenteral Nutrition-Associated Liver Disease After Resolution of Cholestasis With Parenteral Fish Oil Therapy

Nandivada¹,

Chang²,

Potemkin³

et al. 2015

Annals of Surgery

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“…In a different approach, the reduction of soy-bean based lipid has been achieved by adding a fish-oil emulsion [505], or using lipid emulsions consisting of a mixture of either soy-bean based lipid and medium-chained triglyceride MCT-lipid [502,527], or a mixture of a fish-oil emulsion and a soy-bean olive-oil emulsion [528], or soybean based lipid and olive oil and MCT-lipid and fish-oil (SMOF emulsion) [527,529e533]. In randomized controlled trials comparing a SMOF emulsion with a soy-bean based emulsion the fish-oil containing SMOF emulsion proved to be safe and more effective in reducing bilirubin levels or oxidative stress [529,531e533].…”

Section: Commentarymentioning

confidence: 99%

ESPEN guideline on clinical nutrition in liver disease

et al. 2019

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This update of evidence-based guidelines (GL) aims to translate current evidence and expert opinion into recommendations for multidisciplinary teams responsible for the optimal nutritional and metabolic management of adult patients with liver disease. The GL was commissioned and financially supported by ESPEN. Members of the guideline group were selected by ESPEN. We searched for meta-analyses, systematic reviews and single clinical trials based on clinical questions according to the PICO format. The evidence was evaluated and used to develop clinical recommendations implementing the SIGN method. A total of 85 recommendations were made for the nutritional and metabolic management of patients with acute liver failure, severe alcoholic steatohepatitis, non-alcoholic fatty liver disease, liver cirrhosis, liver surgery and transplantation as well as nutrition associated liver injury distinct from fatty liver disease. The recommendations are preceded by statements covering current knowledge of the underlying pathophysiology and pathobiochemistry as well as pertinent methods for the assessment of nutritional status and body composition.

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“…The emulsifier in this lipid emulsion, based on glycerol and egg yolk phospholipid, is similar to that used in soybean IVFE. A number of case reports, case series, and retrospectively controlled trials of IV fish oil have been reported in infants with or at risk for IFALD 98 – 107 . No therapeutic or prophylactic double‐blind randomized controlled trials have been completed to demonstrate the efficacy of this therapy for this indication.…”

Section: Case Examplesmentioning

confidence: 99%

Scientific, Economic, Regulatory, and Ethical Challenges of Bringing Science‐Based Pediatric Nutrition Products to the U.S. Market and Ensuring Their Availability for Patients

Merritt

Goldsmith

2014

J Parenter Enteral Nutr

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Many nutrition products and related drugs are unavailable or not consistently available to clinicians despite a body of clinical data and experience supporting their use. Many of these can be related to drug shortages that have increased since 2009. In addition, there are potentially useful products that are not approved for a specific use or are no longer being manufactured. This review broadly examines the product availability gap from the perspectives of a clinician/former nutrition industry medical director and an economist. The process of pediatric nutrition product and related drug innovation, as well as its drivers and the steps involved in bringing a product to market, is first described. This is followed by an assessment of factors influencing product availability beyond the innovation process, including regulatory issues, manufacturing compliance, purchasing practices, and other factors related to drug and nutrition product pricing and reimbursement. Three pediatric case examples are reviewed and placed in the context of the prior review. Last, recent and future possible steps toward closing the product availability gap are discussed.

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Implementation of a Multidisciplinary Team Approach and Fish Oil Emulsion Administration in the Management of Infants with Short Bowel Syndrome and Parenteral Nutrition-Associated Liver Disease

Cited by 15 publications

References 20 publications

The Natural History of Cirrhosis From Parenteral Nutrition-Associated Liver Disease After Resolution of Cholestasis With Parenteral Fish Oil Therapy

The Natural History of Cirrhosis From Parenteral Nutrition-Associated Liver Disease After Resolution of Cholestasis With Parenteral Fish Oil Therapy

ESPEN guideline on clinical nutrition in liver disease

Scientific, Economic, Regulatory, and Ethical Challenges of Bringing Science‐Based Pediatric Nutrition Products to the U.S. Market and Ensuring Their Availability for Patients

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