Review article: diagnosis and management of intestinal failure‐associated liver disease in adults

Abstract

Summary Background Hepatic disturbances in the context of intestinal failure and parenteral nutrition (PN) are frequently encountered and carry a significant burden of morbidity and sometimes mortality. The term intestinal failure‐associated liver disease (IFALD) refers to liver injury due to intestinal failure and associated PN, in the absence of another evident cause of liver disease, encompassing a spectrum of conditions from deranged liver enzymes, steatosis/ steatohepatitis, cholestasis as well as progressive fibrosis, cirrhosis and end‐stage liver disease. Aims To present an up to date perspective on the diagnosis/definition, aetiologies and subsequent management of IFALD and to explore future consideration for the condition, including pharmacological therapies Results In adults using long‐term PN for benign chronic intestinal failure, 1%‐4% of all deaths are attributed to IFALD. The aetiology of IFALD is multifactorial and can be broadly divided into nutritional factors (eg lipid emulsion type) and patient‐related factors (eg remaining bowel anatomy). Given its multifaceted aetiology, the management of IFALD requires clinicians to investigate a number of factors simultaneously. Patients with progressive liver disease should be considered for combined liver‐intestine transplantation, although multivisceral grafts have a worse prognosis. However, there is no established non‐invasive method to identify progressive IFALD such that liver biopsy, where appropriate, remains the gold standard. Conclusion A widely accepted definition of IFALD would aid in diagnosis, monitoring and subsequent management. Management can be complex with a number of factors to consider. In the future, dedicated pharmacological interventions may become more prominent in the management of IFALD.

Intravenous supplementation type and volume are associated with 1-year outcome and major complications in patients with chronic intestinal failure

“…The same incidence rate can be accounted for the 31 incident cases of IFALD cholestasis/liver failure. These data are of some relevance as no prospective study has yet been carried out on this HPN/IF complication 17…”

Causes and Prognosis of Intestinal Failure in Crohn’s Disease: An 18-year Experience From a National Centre

“…Intestinal failure-associated liver disease was defined as an increase to 1.5 times the upper limit of normal or more for >6 months of blood concentration levels of at least two of the three following measurements: alkaline phosphatase, gamma‐glutamyl transferase, and bilirubin. 14 The Unit was alerted to all deaths occurring during the period of follow-up at Salford Royal Hospital. Deaths occurring subsequent to discharge from the hospital were not recorded, and hence these data were censored at date of discharge from follow-up.…”

Hepatobiliary Manifestations of Short Bowel Syndrome and Intestinal Failure–Associated Liver Disease

“…10 In contrast with excess lipid and caloric supplementation, deficiencies in essential nutrients that play a key role in fatty acid metabolism, such as choline, carnitine, and taurine, have also been demonstrated to result in hepatic steatosis and chronic cholestasis. 5 Sufficient quantities of these nutrients are unstable in PN solution and would require additional parenteral or oral supplementation, providing a potential avenue for formal studies in the prevention or resolution of IFALD.…”

“…A number of noninvasive markers of liver fibrosis and steatosis to include FibroScan, Fibrosis‐4 index, and magnetic resonance spectroscopy have recently been explored and correlated to traditional risk factors for the development of IFALD in an attempt to better define the prevalence and risk factors for disease development 5 . However, none of the noninvasive markers have been applied broadly enough to be incorporated into the diagnosis of IFALD, and liver biopsy remains the gold standard.…”