Parenteral nutrition (PN) has revolutionized the care of patients with intestinal failure by providing nutrition intravenously. Worldwide, PN remains a standard tool of nutrition delivery in neonatal, pediatric, and adult patients. Though the benefits are evident, patients receiving PN can suffer serious cholestasis due to lack of enteral feeding and sometimes have fatal complications from liver injury and gut atrophy, including PN-associated liver disease or intestinal failure-associated liver disease. Recent studies into gut-systemic cross talk via the bile acid-regulated farnesoid X receptor (FXR)-fibroblast growth factor 19 (FGF19) axis, gut microbial control of the TGR5-glucagon-like peptide (GLP) axis, sepsis, and role of prematurity of hepatobiliary receptors are greatly broadening our understanding of PN-associated injury. It has also been shown that the composition of ω-6/ω-3 polyunsaturated fatty acids given parenterally as lipid emulsions can variably drive damage to hepatocytes and cell integrity. This manuscript reviews the mechanisms for the multifactorial pathogenesis of liver disease and gut injury with PN and discusses novel ameliorative strategies.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/ncp.10461

Mechanisms of Parenteral Nutrition–Associated Liver and Gut Injury

php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php). Research and Reports in Neonatology 2018:8 33–44 Research and Reports in Neonatology Dovepress

/pdf/acute-bilirubin-encephalopathy-and-its-progression-to-27vyy4slaq.pdf

Acute Bilirubin Encephalopathy and Its Progression to Kernicterus: Current Perspectives

IVACAFTOR restores FGF19 regulated bile acid homeostasis in cystic fibrosis patients with an S1251N or a G551D gating mutation

Previously in Part I of this two-part review, we discussed the current and recent advances in the understanding of the molecular biology and neuropathology of bilirubin neurotoxicity (BNTx). Here in Part II, we summarize current treatment options available to treat the severely jaundiced infants to prevent significant brain damage and improve clinical outcomes. In addition, we review potential novel therapies that are in various stages of research and development. We will emphasize treatments for both prevention and treatment of both acute bilirubin encephalopathy (ABE) and kernicterus spectrum disorders (KSDs), highlighting the treatment of the most disabling neurological sequelae of children with mild-to-severe KSDs whose "rare disease" status often means they are overlooked by the clinical research community at large. As with other secondary dystonias, treatment of the dystonic motor symptoms in kernicterus is the greatest clinical challenge.

/pdf/review-of-bilirubin-neurotoxicity-ii-preventing-and-treating-40gyxbtzwr.pdf

Review of bilirubin neurotoxicity II: preventing and treating acute bilirubin encephalopathy and kernicterus spectrum disorders

The immunoassays methods need avoiding interferences that can influence result interpretation. Main sources of interference arise from either patient status, preparation and physiology or laboratory process and procedures. The aim of this non-systematic critical review is to highlight the preanalytical interferences on laboratory immunoassays. Blood hormone profile changes according with age and depending on sex: these are important variables, mainly in newborn, during both sexual maturation and childbearing. Gonadotropins FSH and LH show a sharp increase with age in females, whereas in males LH appears rather stable. With age both males and females show progressive decay of the hormone profile. Stress causes variations, as it influences GH, prolactin, Cortisol and the total/free ratio of thyroid hormone. Diurnal variations, day of cycle, influence by estrogens on thyroid hormone are relevant for result variability. Paraproteins and autoantibodies can interfere in some assays particularly drug, vitamin D and thyroid hormone. As regards the variables due to sample matrix, and to evacuated tubes components, some additives and anticoagulants have been reported to influence specific assays, e.g. thyroid hormone. Hemolysis, lipemia and bilirubin cause interferences on specific techniques/tests, e.g. ferritin, TSH, Vitamin B12, progesterone and folic acid. Nicotine and cocaine addictions interfere with some hormones. Thus, laboratory professionals should be aware of preanalytical problems particularly important when dealing with the immunoassays, by taking appropriate actions to avoid any relevant interferences.

Causes of Preanalytical Interferences on Laboratory Immunoassays - A Critical Review.

Effects of Soybean Lipid Infusion on Unbound Free Fatty Acids and Unbound Bilirubin in Preterm Infants.

Introduction: Fibroblast growth factor 19 (FGF19) is a gut-derived hormone that regulates the expression of CYP7A1, the rate-limiting enzyme in bile acid (BA) synthesis pathway. Dysregulati...

Developmental regulation of the gut-liver (FGF19-CYP7A1) axis in neonates.

Heterophile antibody interference affecting multiple Roche immunoassays: A case study

The Case | Hyponatremia in a patient with obstructive jaundice

Background: Hyperbilirubinemia occurs in over 80% of newborns and severe bilirubin toxicity can lead to neurological dysfunction and death, especially in preterm infants. Currently, the risk of bil...

Mary Carayannopoulos

Papers

Effects of Soybean Lipid Infusion on Unbound Free Fatty Acids and Unbound Bilirubin in Preterm Infants.

Developmental regulation of the gut-liver (FGF19-CYP7A1) axis in neonates.

Heterophile antibody interference affecting multiple Roche immunoassays: A case study

The Case | Hyponatremia in a patient with obstructive jaundice

Unbound bilirubin measurements by a novel probe in preterm infants.