title: Pediatric parenteral nutrition-associated liver disease and cholestasis: Novel advances in pathomechanisms-based prevention and treatment.
abstract: Parenteral nutrition constitutes a life-saving therapeutic tool in patients unable to ingest/absorb oral or enteral delivered nutrients. Liver function tests abnormalities are a common therapy-related complication, thus configuring the so-called Parenteral Nutrition Associated Liver Disease (PNALD) or cholestasis (PNAC). Although the damage is frequently mild, and resolves after discontinuation of parenteral nutrition, in some cases it progresses into cirrhotic changes, especially in neonates and infants. We present a literature review focusing on the pathogenetic mechanisms-driven prevention and therapies for the cases where parenteral nutrition cannot be discontinued. Ursodeoxycholic acid has been proposed in patients with cholestatic hepatopathy, but its efficacy needs to be better established. Little evidence is available on efficacy of anti-oxidants, antibiotics, probiotics and anti TNFα. Lipid emulsions based on fish oil with a high content of long-chain polyunsaturated fatty acids ω-3 appear effective both in decreasing intrahepatic inflammation and in improving biliary flow. Most recent promising variations such as soybean/MCT/olive/fish oil emulsion [third generation lipid emulsion (SMOFlipid)] are under investigation. In conclusion, we remark the emergence of a number of novel pathomechanisms underlying the severe liver impairment damage (PNALD and PNAC) in patients treated with parenteral nutrition. Only few traditional and innovative therapeutic strategies have hitherto been shown promising.
authors.affiliation: Pediatrics AORN "Santobono-Pausilipon", Naples, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Orso
authors.affiliation: Pediatrics AORN "Santobono-Pausilipon", Naples, Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Mandato
authors.affiliation: Pediatric Residency Program, Department of Translational Medical Sciences, University of Naples "Federico II", Salerno, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Veropalumbo
authors.affiliation: Pediatrics AORN "Santobono-Pausilipon", Naples, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Cecchi
authors.affiliation: Chairs of Pediatric Surgery, Department of Medicine and Surgery, University of Salerno, Salerno, Italy.
authors.forename: Alfredo
authors.initials: A
authors.lastname: Garzi
authors.affiliation: Pediatrics, Department of Medicine and Surgery, University of Salerno, Salerno, Italy; European Laboratory of Food Induced Disease (ELFID), Naples, Italy. Electronic address: pvajro@unisa.it.
authors.forename: Pietro
authors.initials: P
authors.lastname: Vajro
mesh_terms: D002648:Child; D002675:Child, Preschool; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D002779:Cholestasis / Q000209:etiology* / Q000517:prevention & control / Q000628:therapy; D005217:Fat Emulsions, Intravenous / Q000009:adverse effects / Q000627:therapeutic use; D015525:Fatty Acids, Omega-3 / Q000627:therapeutic use; D005395:Fish Oils / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008107:Liver Diseases / Q000209:etiology* / Q000517:prevention & control / Q000628:therapy; D010288:Parenteral Nutrition / Q000009:adverse effects* / Q000379:methods; D013024:Soybean Oil / Q000627:therapeutic use; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D005217:Fat Emulsions, Intravenous; D015525:Fatty Acids, Omega-3; D005395:Fish Oils; D014580:Ursodeoxycholic Acid; D013024:Soybean Oil
keywords: Children; Cholestasis; Liver disease; PNALD; Parenteral nutrition