Assessing Non-Invasive Liver Function in Patients with Intestinal Failure Receiving Total Parenteral Nutrition—Results from the Prospective PNLiver Trial

Blüthner, Elisabeth; Pape, Ulrich‐Frank; Stockmann, Martin; Karber, Mirjam; Maasberg, Sebastian; Pevny, Sophie; Gerlach-Runge, Undine A.; Pascher, Andreas; Pratschke, Johann; Tacke, Frank; Bednarsch, Jan

doi:10.3390/nu12051217

Cited by 9 publications

(12 citation statements)

References 47 publications

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“…Actually, both factors related to IF and PN play a key role in the development of IFALD. Among IF-related factors, NEC with subsequent SBS, multiple surgeries, the lack of enteral feeding, disturbed enterohepatic bile flow, the presence of inflammation, oxidative stress, immaturity of the liver, bacterial overgrowth, catheter-related blood stream infection, and sepsis are the main determinants (3,5,15,(17)(18)(19). On the other side, imbalance (deficiency/excess) of parenteral nutrients and their composition may contribute to the development of IFALD (5).…”

Section: Prevalence Etiology and Risk Factorsmentioning

confidence: 99%

“…Due to impaired nutritional status and short remnant bowel length, nine patients were dependent on total parenteral nutrition (SPN) whereas 11 patients were able to halve their parenteral caloric intake over time (RPN). Over time, authors detected a progressive reduction of liver maximum capacity test (LiMax) values in SPN group from baseline to 6, 12, and then 24 months of follow-up (18). LiMax test is a non-invasive diagnostic method for determining enzymatic liver function, based on the 13 C-labeled metacetin substrate which is metabolized exclusively by cytochrome P450 1A2 to 13 CO 2 and acetaminophen (41).…”

Section: Diagnosismentioning

confidence: 99%

“…Prematurity and low birth weight represent the main risk factors for IFALD, with the highest reported incidence in infants born at <34 weeks of gestation and who weigh <2 kg ( 13 ). Premature infants who develop NEC are at particular risk for IFALD because, in addition to liver immaturity, they are exposed to multiple risk factors such as higher rate of sepsis, short bowel, disrupted enterohepatic bile acid circulation, enteral feed intolerance, and longer duration of PN ( 3 , 18 ). Actually, both factors related to IF and PN play a key role in the development of IFALD.…”

Section: Prevalence Etiology and Risk Factorsmentioning

confidence: 99%

“…Usually, cholestasis is prominent in infants and children whereas steatosis and steatohepatitis are more common in older children and adults ( 13 ). Not always biochemical enzyme levels correlate with the degree of fibrosis and they can also be normal during disease progression, whereas an association between the severity of the liver injury and the duration of PN is documented ( 18 , 34 ). In 2021, Mutanen et al ( 10 ) analyzed liver histology in 77 children with IF at a median age of 1.7 years and in 48 of them after almost 3 years.…”

Section: Clinical and Histological Manifestationsmentioning

confidence: 99%

“…Transaminases showed mild reduction in RPN group, while were stable in patients with SPN after 12 months and increased after 24 months. LiMax anticipated the deterioration of the other liver function tests appearing more sensitive in detecting early changes in liver function in comparison with other liver function tests, laboratory-based scores, or imaging-based liver function tests ( 18 ). It could be useful to detect early hepatic dysfunction in patients with IF receiving PN.…”

Section: Diagnosismentioning

confidence: 99%

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IFALD in children: What's new? A narrative review

2022

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Intestinal failure-associated liver disease (IFALD) is a progressive liver disease complicating intestinal failure (IF). It is a preventable and reversible condition, but at the same time, a potential cause of liver cirrhosis and an indication to combined or non-combined liver and small bowel transplantation. The diagnostic criteria are not yet standardized, so that its prevalence varies widely in the literature. Pathophysiology seems to be multifactorial, related to different aspects of intestinal failure and not only to the long-term parenteral nutrition treatment. The survival rates of children with IF have increased, so that the main problems today are preventing complications and ensuring a good quality of life. IFALD is one of the most important factors that limit long-term survival of patients with IF. For this reason, more and more interest is developing around it and the number of published articles is increasing rapidly. The purpose of this narrative review was to focus on the main aspects of the etiology, pathophysiology, management, prevention, and treatment of IFALD, based on what has been published mainly in the last 10 years. Controversies and current research gaps will be highlighted with the aim to pave the way for new project and high-quality clinical trials.

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Section: Prevalence Etiology and Risk Factorsmentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

Section: Prevalence Etiology and Risk Factorsmentioning

confidence: 99%

Section: Clinical and Histological Manifestationsmentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

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IFALD in children: What's new? A narrative review

2022

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Intestinal failure–associated liver disease: Current challenges in screening, diagnosis, and parenteral nutrition considerations

Tabone,

Mooney,

Donnellan

2024

Nut in Clin Prac

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Intestinal failure–associated liver disease (IFALD) is a serious life‐limiting complication that can occur throughout the clinical course of intestinal failure and its management by parenteral nutrition (PN). Despite this, there is a lack of a standardized definition for IFALD, which makes this insidious condition increasingly difficult to screen and diagnose in clinical practice. Attenuating the progression of liver disease before the onset of liver failure is key to improving morbidity and mortality in these patients. This requires timely detection and promptly addressing reversible factors. Although there are various noninvasive tools available to the clinician to detect early fibrosis or cirrhosis in various chronic liver disease states, these have not been validated in the patient population with IFALD. Such tools include biochemical composite scoring systems for fibrosis, transient elastography, and dynamic liver function tests. This review article aims to highlight the existing real need for an accurate, reproducible method to detect IFALD in its early stages. In addition, we also explore the role PN plays in the pathogenesis of this complex multifactorial condition. Various aspects of PN administration have been implicated in the etiology of IFALD, including the composition of the lipid component, nutrient excess and deficiency, and infusion timing. We aim to highlight the clinical relevance of these PN‐associated factors in the development of IFALD and how these can be managed to mitigate the progression of IFALD.

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