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Parenteral nutrition-associated cholestasis

MedGen UID:
475934
•Concept ID:
C3274301
•
Finding
Synonyms: Parenteral Nutrition - Associated Cholestasis; parenteral nutrition-associated cholestasis; PNAC; total Parenteral Nutrition Associated Cholestasis; TPN- Associated Cholestasis
 
Monarch Initiative: MONDO:0035777
Orphanet: ORPHA567983

Definition

A rare hepatic disease characterized by intrahepatic cholestasis and deterioration of liver function in patients receiving parenteral nutrition for extended periods of time (signs may appear as early as within the first two weeks of initiation of parenteral nutrition). The condition commonly occurs in neonates and usually resolves with transition to enteral feeding, although severe cases may progress to liver fibrosis, cirrhosis, and portal hypertension. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVParenteral nutrition-associated cholestasis

Professional guidelines

PubMed

Neonatal Cholestasis: Updates on Diagnostics, Therapeutics, and Prevention.
Feldman AG, Sokol RJ
Neoreviews 2021 Dec 1;22(12):e819-e836. doi: 10.1542/neo.22-12-e819. PMID: 34850148Free PMC Article
Intravenous Lipid Emulsions in the Prevention and Treatment of Liver Disease in Intestinal Failure.
Rochling FA
Nutrients 2021 Mar 10;13(3) doi: 10.3390/nu13030895. PMID: 33801970Free PMC Article
Intestinal failure-associated liver disease (IFALD): insights into pathogenesis and advances in management.
Lee WS, Chew KS, Ng RT, Kasmi KE, Sokol RJ
Hepatol Int 2020 May;14(3):305-316. Epub 2020 Apr 30 doi: 10.1007/s12072-020-10048-8. PMID: 32356227

Recent clinical studies

Etiology

Analysis of risk factors for parenteral nutrition-associated cholestasis in preterm infants: a multicenter observational study.
Wang YS, Shen W, Yang Q, Lin R, Tang LX, Bai RM, Yang D, Zhang J, Zhang YJ, Yu WT, Song SR, Kong J, Song SY, Mao J, Tong XM, Li ZK, Wu F, Lin XZ
BMC Pediatr 2023 May 20;23(1):250. doi: 10.1186/s12887-023-04068-0. PMID: 37210514Free PMC Article
Neonatal Cholestasis: Updates on Diagnostics, Therapeutics, and Prevention.
Feldman AG, Sokol RJ
Neoreviews 2021 Dec 1;22(12):e819-e836. doi: 10.1542/neo.22-12-e819. PMID: 34850148Free PMC Article
Parenteral nutrition-associated cholestasis: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review.
Rangel SJ, Calkins CM, Cowles RA, Barnhart DC, Huang EY, Abdullah F, Arca MJ, Teitelbaum DH; 2011 American Pediatric Surgical Association Outcomes and Clinical Trials Committee
J Pediatr Surg 2012 Jan;47(1):225-40. doi: 10.1016/j.jpedsurg.2011.10.007. PMID: 22244423
Parenteral nutrition-associated cholestasis.
Teitelbaum DH, Tracy T
Semin Pediatr Surg 2001 May;10(2):72-80. doi: 10.1053/spsu.2001.22386. PMID: 11329608
Parenteral nutrition-associated cholestasis.
Teitelbaum DH
Curr Opin Pediatr 1997 Jun;9(3):270-5. doi: 10.1097/00008480-199706000-00016. PMID: 9229168

Diagnosis

Fecal sphingolipids predict parenteral nutrition-associated cholestasis in the neonatal intensive care unit.
Moutinho TJ, Powers DA, Hanson GF, Levy S, Baveja R, Hefner I, Mohamed M, Abdelghani A, Baker RL, Papin JA, Moore SR, Hourigan SK
JPEN J Parenter Enteral Nutr 2022 Nov;46(8):1903-1913. Epub 2022 Apr 7 doi: 10.1002/jpen.2374. PMID: 35285019Free PMC Article
Neonatal Cholestasis: Updates on Diagnostics, Therapeutics, and Prevention.
Feldman AG, Sokol RJ
Neoreviews 2021 Dec 1;22(12):e819-e836. doi: 10.1542/neo.22-12-e819. PMID: 34850148Free PMC Article
Intravenous Lipid Emulsions in Infants: Is Balanced Better?
Rostas SE, McPherson C
Neonatal Netw 2019 Jan;38(1):39-45. doi: 10.1891/0730-0832.38.1.39. PMID: 30679255
Fish oil-based lipid emulsion in the treatment of parenteral nutrition-associated cholestasis.
Costa S, Iannotta R, Maggio L, Barone G, Serrao F, Vento G
Ital J Pediatr 2018 Aug 23;44(1):101. doi: 10.1186/s13052-018-0539-0. PMID: 30139361Free PMC Article
Total parenteral nutrition-associated cholestasis and risk factors in preterm infants.
Alkharfy TM, Ba-Abbad R, Hadi A, Sobaih BH, AlFaleh KM
Saudi J Gastroenterol 2014 Sep-Oct;20(5):293-6. doi: 10.4103/1319-3767.141688. PMID: 25253364Free PMC Article

Therapy

Analysis of risk factors for parenteral nutrition-associated cholestasis in preterm infants: a multicenter observational study.
Wang YS, Shen W, Yang Q, Lin R, Tang LX, Bai RM, Yang D, Zhang J, Zhang YJ, Yu WT, Song SR, Kong J, Song SY, Mao J, Tong XM, Li ZK, Wu F, Lin XZ
BMC Pediatr 2023 May 20;23(1):250. doi: 10.1186/s12887-023-04068-0. PMID: 37210514Free PMC Article
Intestinal failure-associated liver disease (IFALD): insights into pathogenesis and advances in management.
Lee WS, Chew KS, Ng RT, Kasmi KE, Sokol RJ
Hepatol Int 2020 May;14(3):305-316. Epub 2020 Apr 30 doi: 10.1007/s12072-020-10048-8. PMID: 32356227
Parenteral nutrition-associated cholestasis: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review.
Rangel SJ, Calkins CM, Cowles RA, Barnhart DC, Huang EY, Abdullah F, Arca MJ, Teitelbaum DH; 2011 American Pediatric Surgical Association Outcomes and Clinical Trials Committee
J Pediatr Surg 2012 Jan;47(1):225-40. doi: 10.1016/j.jpedsurg.2011.10.007. PMID: 22244423
Parenteral nutrition-associated cholestasis.
Teitelbaum DH, Tracy T
Semin Pediatr Surg 2001 May;10(2):72-80. doi: 10.1053/spsu.2001.22386. PMID: 11329608
Parenteral nutrition-associated cholestasis.
Teitelbaum DH
Curr Opin Pediatr 1997 Jun;9(3):270-5. doi: 10.1097/00008480-199706000-00016. PMID: 9229168

Prognosis

Fecal sphingolipids predict parenteral nutrition-associated cholestasis in the neonatal intensive care unit.
Moutinho TJ, Powers DA, Hanson GF, Levy S, Baveja R, Hefner I, Mohamed M, Abdelghani A, Baker RL, Papin JA, Moore SR, Hourigan SK
JPEN J Parenter Enteral Nutr 2022 Nov;46(8):1903-1913. Epub 2022 Apr 7 doi: 10.1002/jpen.2374. PMID: 35285019Free PMC Article
Gram-negative Microbiota Blooms in Premature Twins Discordant for Parenteral Nutrition-associated Cholestasis.
Hourigan SK, Moutinho TJ Jr, Berenz A, Papin J, Guha P, Bangiolo L, Oliphant S, Provenzano M, Baveja R, Baker R, Vilboux T, Levy S, Deopujari V, Nataro JP, Niederhuber JE, Moore SR
J Pediatr Gastroenterol Nutr 2020 May;70(5):640-644. doi: 10.1097/MPG.0000000000002617. PMID: 31939866Free PMC Article
Total parenteral nutrition-associated cholestasis and risk factors in preterm infants.
Alkharfy TM, Ba-Abbad R, Hadi A, Sobaih BH, AlFaleh KM
Saudi J Gastroenterol 2014 Sep-Oct;20(5):293-6. doi: 10.4103/1319-3767.141688. PMID: 25253364Free PMC Article
Parenteral nutrition-associated cholestasis: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review.
Rangel SJ, Calkins CM, Cowles RA, Barnhart DC, Huang EY, Abdullah F, Arca MJ, Teitelbaum DH; 2011 American Pediatric Surgical Association Outcomes and Clinical Trials Committee
J Pediatr Surg 2012 Jan;47(1):225-40. doi: 10.1016/j.jpedsurg.2011.10.007. PMID: 22244423
Ursodiol in patients with parenteral nutrition-associated cholestasis.
San Luis VA, Btaiche IF
Ann Pharmacother 2007 Nov;41(11):1867-72. Epub 2007 Oct 2 doi: 10.1345/aph.1K229. PMID: 17911205

Clinical prediction guides

Analysis of risk factors for parenteral nutrition-associated cholestasis in preterm infants: a multicenter observational study.
Wang YS, Shen W, Yang Q, Lin R, Tang LX, Bai RM, Yang D, Zhang J, Zhang YJ, Yu WT, Song SR, Kong J, Song SY, Mao J, Tong XM, Li ZK, Wu F, Lin XZ
BMC Pediatr 2023 May 20;23(1):250. doi: 10.1186/s12887-023-04068-0. PMID: 37210514Free PMC Article
Fecal sphingolipids predict parenteral nutrition-associated cholestasis in the neonatal intensive care unit.
Moutinho TJ, Powers DA, Hanson GF, Levy S, Baveja R, Hefner I, Mohamed M, Abdelghani A, Baker RL, Papin JA, Moore SR, Hourigan SK
JPEN J Parenter Enteral Nutr 2022 Nov;46(8):1903-1913. Epub 2022 Apr 7 doi: 10.1002/jpen.2374. PMID: 35285019Free PMC Article
Gram-negative Microbiota Blooms in Premature Twins Discordant for Parenteral Nutrition-associated Cholestasis.
Hourigan SK, Moutinho TJ Jr, Berenz A, Papin J, Guha P, Bangiolo L, Oliphant S, Provenzano M, Baveja R, Baker R, Vilboux T, Levy S, Deopujari V, Nataro JP, Niederhuber JE, Moore SR
J Pediatr Gastroenterol Nutr 2020 May;70(5):640-644. doi: 10.1097/MPG.0000000000002617. PMID: 31939866Free PMC Article
Total parenteral nutrition-associated cholestasis and risk factors in preterm infants.
Alkharfy TM, Ba-Abbad R, Hadi A, Sobaih BH, AlFaleh KM
Saudi J Gastroenterol 2014 Sep-Oct;20(5):293-6. doi: 10.4103/1319-3767.141688. PMID: 25253364Free PMC Article
Ursodiol in patients with parenteral nutrition-associated cholestasis.
San Luis VA, Btaiche IF
Ann Pharmacother 2007 Nov;41(11):1867-72. Epub 2007 Oct 2 doi: 10.1345/aph.1K229. PMID: 17911205

Recent systematic reviews

Ursodeoxycholic acid for preventing parenteral nutrition-associated cholestasis in neonates: A systematic review and meta-analysis.
Anne RP, Kadyada SP, Aradhya AS, Oleti TP
Fundam Clin Pharmacol 2024 Aug;38(4):685-694. Epub 2024 Feb 11 doi: 10.1111/fcp.12993. PMID: 38342497
Efficacy and safety of ursodeoxycholic acid in children with cholestasis: A systematic review and meta-analysis.
Huang L, Li S, Chen J, Zhu Y, Lan K, Zeng L, Jiang X, Zhang L
PLoS One 2023;18(1):e0280691. Epub 2023 Jan 31 doi: 10.1371/journal.pone.0280691. PMID: 36719881Free PMC Article
Parenteral fish oil-containing lipid emulsions may reverse parenteral nutrition-associated cholestasis in neonates: a systematic review and meta-analysis.
Park HW, Lee NM, Kim JH, Kim KS, Kim SN
J Nutr 2015 Feb;145(2):277-83. Epub 2014 Dec 17 doi: 10.3945/jn.114.204974. PMID: 25644348
Incidence, prevention, and treatment of parenteral nutrition-associated cholestasis and intestinal failure-associated liver disease in infants and children: a systematic review.
Lauriti G, Zani A, Aufieri R, Cananzi M, Chiesa PL, Eaton S, Pierro A
JPEN J Parenter Enteral Nutr 2014 Jan;38(1):70-85. Epub 2013 Jul 26 doi: 10.1177/0148607113496280. PMID: 23894170
Parenteral nutrition-associated cholestasis: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review.
Rangel SJ, Calkins CM, Cowles RA, Barnhart DC, Huang EY, Abdullah F, Arca MJ, Teitelbaum DH; 2011 American Pediatric Surgical Association Outcomes and Clinical Trials Committee
J Pediatr Surg 2012 Jan;47(1):225-40. doi: 10.1016/j.jpedsurg.2011.10.007. PMID: 22244423

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