Intestinal Failure–Associated Liver Disease

Lacaille, Florence; Gupte, Girish; Colomb, V.; D’Antiga, Lorenzo; Hartman, Corina; Hojsak, Iva; Kolaček, Sanja; Puntis, John; Shamir, Raanan

doi:10.1097/mpg.0000000000000586

Cited by 180 publications

(93 citation statements)

References 184 publications

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“…4 Host factors, includingprematurity, small for gestational age, abdominal surgery, and episodes of sepsis, have been reviewed elsewhere. 4, 8, 9 However, none of the listed risk factors has been carefully studied in a prospective, controlled trial. 4, 8, 9 In addition, no single risk factor has been implicated as causative of liver injury in all patients on prolonged PN, thus, a multi-factorial etiology seems likely.…”

Section: Pathogenesis Of Ifaldmentioning

confidence: 99%

“…2, 4, 8, 9 The evidence supporting the recommendation for a reduced dose of SO-ILE or replacing it with other source of ILE, such FO-based, is graded as weak or probably effective. 2, 4, 8, 9 Nevertheless, these practices have been widely adopted in the United States based on clinical experience and absence of alternative effective approaches.…”

Section: Preventive and Therapeutic Approachesmentioning

confidence: 99%

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Intestinal Microbiota, Lipids, and the Pathogenesis of Intestinal Failure–Associated Liver Disease

Lee

Sokol

2015

The Journal of Pediatrics

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Section: Pathogenesis Of Ifaldmentioning

confidence: 99%

Section: Preventive and Therapeutic Approachesmentioning

confidence: 99%

Intestinal Microbiota, Lipids, and the Pathogenesis of Intestinal Failure–Associated Liver Disease

Lee

Sokol

2015

The Journal of Pediatrics

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“…IF-associated liver disease (IFALD) is a serious complication and the leading cause of morbidity and mortality in children IF patients34. However, the mechanisms underlying the development of IFALD are poorly understood.…”

mentioning

confidence: 99%

Altered systemic bile acid homeostasis contributes to liver disease in pediatric patients with intestinal failure

Xiao

Cao

Zhou

et al. 2016

Sci Rep

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Intestinal failure (IF)-associated liver disease (IFALD), as a major complication, contributes to significant morbidity in pediatric IF patients. However, the pathogenesis of IFALD is still uncertain. We here investigate the roles of bile acid (BA) dysmetabolism in the unclear pathogenesis of IFALD. It found that the histological evidence of pediatric IF patients exhibited liver injury, which was characterized by liver bile duct proliferation, inflammatory infiltration, hepatocyte apoptosis and different stages of fibrosis. The BA compositions were altered in serum and liver of pediatric IF patients, as reflected by a primary BA dominant composition. In IF patients, the serum FGF19 levels decreased significantly, and were conversely correlated with ileal inflammation grades (r = −0.50, p < 0.05). In ileum, the inflammation grades were inversely associated with farnesoid X receptor (FXR) expression (r = −0.55, p < 0.05). In liver, the expression of induction of the rate-limiting enzyme in bile salt synthesis, cytochrome P450 7a1 (CYP7A1) increased evidently. In conclusion, ileum inflammation decreases FXR expression corresponding to reduce serum FGF19 concentration, along with increased hepatic bile acid synthesis, leading to liver damages in IF patients.

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“…These have thus been implicated in the pathogenesis of IFALD [68]. On the other hand, omega-3 emulsions such as Omegaven™ have anti-inflammatory effects and appear to offer hepatic protection [56].…”

Section: Discussionmentioning

confidence: 99%

Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of Omegaven™

Khalaf

Karjoo

Danielson

et al. 2017

Pediatr Gastroenterol Hepatol Nutr

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Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to Omegaven™, a fat emulsion comprised of omega-3 fatty acids. Omegaven™ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with Omegaven™ was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and complete resolution of cholestasis with a direct bilirubin of zero within two weeks of initiation of Omegaven™. No current guidelines for the diagnosis and management of hypoganglionosis are available. We recommend a multidisciplinary approach and the use of novel therapies such as fat emulsions composed of omega-3 fatty acids for improved patient outcomes. Appropriate compassionate use protocols should be obtained from the Food and Drug Administration prior to initiation of Omegaven™.

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Intestinal Failure–Associated Liver Disease

Cited by 180 publications

References 184 publications

Intestinal Microbiota, Lipids, and the Pathogenesis of Intestinal Failure–Associated Liver Disease

Intestinal Microbiota, Lipids, and the Pathogenesis of Intestinal Failure–Associated Liver Disease

Altered systemic bile acid homeostasis contributes to liver disease in pediatric patients with intestinal failure

Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of Omegaven™

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