ABSTRACT
Liver disease is estimated to develop in 40% to 60% of infants on long-term parenteral
nutrition (PN) secondary to intestinal failure. The etiology of intestinal failure-associated
liver disease (IFALD) is multifactorial with primary contributors including prematurity,
sepsis, deficiencies or hepatotoxicities of infusates, and lack of enterally stimulated
bile flow. IFALD treatment strategies have historically emphasized the following:
choleretics such as ursodeoxycholic acid, bowel decontamination for small bowel bacterial
overgrowth, bowel tapering and lengthening procedures, and manipulations of PN prescriptions
(e.g., cycling). This review highlights current and proposed novel treatment and management
strategies for IFALD. These include a discussion of state-of-the art central line
care practices, novel bowel-lengthening procedures such as serial transverse enteroplasty,
isolated liver transplant for IFALD, probiotics and glutamine for bowel decontamination,
hormonal therapies for achieving bowel adaptation, and a discussion of new PN formulations
that may have emerging roles in IFALD.
KEYWORDS
Intestinal failure - total parenteral nutrition - cholestasis - liver
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Beth A CarterM.D.
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Baylor College
of Medicine
6621 Fannin Street, CCC 1010.00, Houston, TX 77030