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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

Feldman AG, Sokol RJ
Neoreviews 2021 Dec 1;22(12):e819-e836. doi: 10.1542/neo.22-12-e819. PMID: 34850148Free PMC Article
Rochling FA
Nutrients 2021 Mar 10;13(3) doi: 10.3390/nu13030895. PMID: 33801970Free PMC Article
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

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
Feldman AG, Sokol RJ
Neoreviews 2021 Dec 1;22(12):e819-e836. doi: 10.1542/neo.22-12-e819. PMID: 34850148Free PMC Article
Ghosh S, Devereaux MW, Anderson AL, Gehrke S, Reisz JA, D'Alessandro A, Orlicky DJ, Lovell M, El Kasmi KC, Shearn CT, Sokol RJ
Hepatology 2021 Dec;74(6):3284-3300. Epub 2021 Aug 27 doi: 10.1002/hep.32071. PMID: 34310734Free PMC Article
Teitelbaum DH, Tracy T
Semin Pediatr Surg 2001 May;10(2):72-80. doi: 10.1053/spsu.2001.22386. PMID: 11329608
Teitelbaum DH
Curr Opin Pediatr 1997 Jun;9(3):270-5. doi: 10.1097/00008480-199706000-00016. PMID: 9229168

Diagnosis

Chen YC, Chou CM, Huang SY, Chen HC
Nutrients 2023 Jan 30;15(3) doi: 10.3390/nu15030706. PMID: 36771412Free PMC Article
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
Feldman AG, Sokol RJ
Neoreviews 2021 Dec 1;22(12):e819-e836. doi: 10.1542/neo.22-12-e819. PMID: 34850148Free PMC Article
Lee HH, Jung JM, Nam SH, Lim G, Chung ML
Acta Paediatr 2016 Jul;105(7):e313-9. Epub 2016 May 4 doi: 10.1111/apa.13441. PMID: 27097151
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

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
Ghosh S, Devereaux MW, Anderson AL, Gehrke S, Reisz JA, D'Alessandro A, Orlicky DJ, Lovell M, El Kasmi KC, Shearn CT, Sokol RJ
Hepatology 2021 Dec;74(6):3284-3300. Epub 2021 Aug 27 doi: 10.1002/hep.32071. PMID: 34310734Free PMC Article
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
Teitelbaum DH, Tracy T
Semin Pediatr Surg 2001 May;10(2):72-80. doi: 10.1053/spsu.2001.22386. PMID: 11329608
Teitelbaum DH
Curr Opin Pediatr 1997 Jun;9(3):270-5. doi: 10.1097/00008480-199706000-00016. PMID: 9229168

Prognosis

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
Kasirer Y, Bin-Nun A, Raveh A, Schorrs I, Mimouni FB, Hammerman C
Am J Perinatol 2019 Nov;36(13):1382-1386. Epub 2019 Jan 8 doi: 10.1055/s-0038-1676977. PMID: 30620942
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
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
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

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
Chen YC, Chou CM, Huang SY, Chen HC
Nutrients 2023 Jan 30;15(3) doi: 10.3390/nu15030706. PMID: 36771412Free PMC Article
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
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
Fischer CJ, Bickle Graz M, Muehlethaler V, Palmero D, Tolsa JF
J Paediatr Child Health 2013 Sep;49(9):E413-9. Epub 2013 May 28 doi: 10.1111/jpc.12244. PMID: 23714420

Recent systematic reviews

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
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
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
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
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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