Persistence of hepatic fibrosis in pediatric intestinal failure patients treated with intravenous fish oil lipid emulsion.

Abstract:

BACKGROUND:Pediatric intestinal failure (PIF) is a life-altering chronic condition with significant morbidity and mortality. Omegaven® therapy has been used to treat children with advanced intestinal failure associated liver disease. Our objective was to determine the evolution of hepatic fibrosis in PIF patients who received Omegaven® and describe their clinical outcome. METHODS:A retrospective review in PIF patients who received Omegaven® was performed. Patients were included if they had liver biopsies completed before Omegaven® therapy and after resolution of hyperbilirubinemia. Biopsy results were evaluated to determine the degree of fibrosis, inflammation, and cholestasis. Clinical and biochemical data was collected. RESULTS:Six patients were identified. Assessment of fibrosis at last follow-up demonstrated improvement in 2 patients and progression or stable fibrosis in 4/6. All patients demonstrated reduction in cholestasis and inflammation. One patient received a liver/intestine transplant and a second is listed, both of them with progressive fibrosis. One patient achieved full enteral nutrition, while the rest remain partially parenteral nutrition dependent. CONCLUSION:Use of Omegaven® is associated with reduced cholestasis and inflammation, but with persistence or worsening of fibrosis in some patients. A subset of patients with progressive fibrosis may develop portal hypertension and progressive liver disease.

journal_name

J Pediatr Surg

authors

Belza C,Thompson R,Somers GR,de Silva N,Fitzgerald K,Steinberg K,Courtney-Martin G,Wales PW,Avitzur Y

doi

10.1016/j.jpedsurg.2017.01.048

subject

Has Abstract

pub_date

2017-05-01 00:00:00

pages

795-801

issue

5

eissn

0022-3468

issn

1531-5037

pii

S0022-3468(17)30081-7

journal_volume

52

pub_type

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