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Acute fatty liver of pregnancy(AFLP)

MedGen UID:
264102
•Concept ID:
C1455728
•
Disease or Syndrome
Synonyms: Acute fatty liver, gestational; AFLP
SNOMED CT: Acute fatty liver of pregnancy (716379000); AFLP - acute fatty liver of pregnancy (716379000); Nonalcoholic fatty liver during pregnancy (716379000)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Non-Mendelian inheritance (Orphanet)
 
Monarch Initiative: MONDO:0016573
Orphanet: ORPHA243367

Definition

Acute fatty liver of pregnancy is a rare but severe complication occurring in the third trimester of pregnancy or in early postpartum period bearing a risk for perinatal and maternal mortality with manifestations of jaundice, rise of hepatic injuries and evolving to acute liver failure and encephalopathy. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute fatty liver of pregnancy
Follow this link to review classifications for Acute fatty liver of pregnancy in Orphanet.

Professional guidelines

PubMed

AGA Clinical Practice Update on Pregnancy-Related Gastrointestinal and Liver Disease: Expert Review.
Kothari S, Afshar Y, Friedman LS, Ahn J
Gastroenterology 2024 Oct;167(5):1033-1045. Epub 2024 Aug 12 doi: 10.1053/j.gastro.2024.06.014. PMID: 39140906
Thrombocytopenia in pregnancy: Diagnosis and approach to management.
Pishko AM, Levine LD, Cines DB
Blood Rev 2020 Mar;40:100638. Epub 2019 Nov 6 doi: 10.1016/j.blre.2019.100638. PMID: 31757523
Acute Fatty Liver Disease of Pregnancy: Updates in Pathogenesis, Diagnosis, and Management.
Liu J, Ghaziani TT, Wolf JL
Am J Gastroenterol 2017 Jun;112(6):838-846. Epub 2017 Mar 14 doi: 10.1038/ajg.2017.54. PMID: 28291236

Recent clinical studies

Etiology

Obstetric Disorders and Critical Illness.
Griffin KM, Oxford-Horrey C, Bourjeily G
Clin Chest Med 2022 Sep;43(3):471-488. doi: 10.1016/j.ccm.2022.04.008. PMID: 36116815Free PMC Article
Pregnancy-Associated Liver Diseases.
Terrault NA, Williamson C
Gastroenterology 2022 Jul;163(1):97-117.e1. Epub 2022 Mar 8 doi: 10.1053/j.gastro.2022.01.060. PMID: 35276220
Thrombocytopenia in pregnancy: Diagnosis and approach to management.
Pishko AM, Levine LD, Cines DB
Blood Rev 2020 Mar;40:100638. Epub 2019 Nov 6 doi: 10.1016/j.blre.2019.100638. PMID: 31757523
Acute Fatty Liver Disease of Pregnancy: Updates in Pathogenesis, Diagnosis, and Management.
Liu J, Ghaziani TT, Wolf JL
Am J Gastroenterol 2017 Jun;112(6):838-846. Epub 2017 Mar 14 doi: 10.1038/ajg.2017.54. PMID: 28291236
Coagulation disorders in liver disease.
Amitrano L, Guardascione MA, Brancaccio V, Balzano A
Semin Liver Dis 2002 Feb;22(1):83-96. doi: 10.1055/s-2002-23205. PMID: 11928081

Diagnosis

Pregnancy-Associated Liver Diseases.
Terrault NA, Williamson C
Gastroenterology 2022 Jul;163(1):97-117.e1. Epub 2022 Mar 8 doi: 10.1053/j.gastro.2022.01.060. PMID: 35276220
Acute Fatty Liver of Pregnancy.
Nelson DB, Byrne JJ, Cunningham FG
Obstet Gynecol 2021 Mar 1;137(3):535-546. doi: 10.1097/AOG.0000000000004289. PMID: 33543900
Acute Fatty Liver of Pregnancy.
Nelson DB, Byrne JJ, Cunningham FG
Clin Obstet Gynecol 2020 Mar;63(1):152-164. doi: 10.1097/GRF.0000000000000494. PMID: 31725416
HELLP Syndrome: Pathophysiology and Current Therapies.
Wallace K, Harris S, Addison A, Bean C
Curr Pharm Biotechnol 2018;19(10):816-826. doi: 10.2174/1389201019666180712115215. PMID: 29998801
Acute Fatty Liver Disease of Pregnancy: Updates in Pathogenesis, Diagnosis, and Management.
Liu J, Ghaziani TT, Wolf JL
Am J Gastroenterol 2017 Jun;112(6):838-846. Epub 2017 Mar 14 doi: 10.1038/ajg.2017.54. PMID: 28291236

Therapy

Acute Liver Failure.
Ledgerwood C, Villgran V, Mardirossian N, Dumont T, DiSilvio B
Crit Care Nurs Q 2022 Jul-Sep 01;45(3):248-257. doi: 10.1097/CNQ.0000000000000409. PMID: 35617091
Pregnancy-associated hemolytic uremic syndrome.
Alobaidi S, AlDabbagh A, Alamoudi A, Almowarey M, Akl A
Saudi J Kidney Dis Transpl 2020 Nov-Dec;31(6):1180-1188. doi: 10.4103/1319-2442.308326. PMID: 33565429
HELLP Syndrome: Pathophysiology and Current Therapies.
Wallace K, Harris S, Addison A, Bean C
Curr Pharm Biotechnol 2018;19(10):816-826. doi: 10.2174/1389201019666180712115215. PMID: 29998801
Liver Failure in Pregnancy.
Bacak SJ, Thornburg LL
Crit Care Clin 2016 Jan;32(1):61-72. Epub 2015 Oct 9 doi: 10.1016/j.ccc.2015.08.005. PMID: 26600444
Imitators of severe preeclampsia.
Sibai BM
Obstet Gynecol 2007 Apr;109(4):956-66. doi: 10.1097/01.AOG.0000258281.22296.de. PMID: 17400860

Prognosis

Acute liver failure in pregnancy.
Alexander V, Benjamin SJ, Subramani K, Sathyendra S, Goel A
Indian J Gastroenterol 2024 Apr;43(2):325-337. Epub 2024 May 1 doi: 10.1007/s12664-024-01571-9. PMID: 38691240
Acute Fatty Liver of Pregnancy.
Nelson DB, Byrne JJ, Cunningham FG
Clin Obstet Gynecol 2020 Mar;63(1):152-164. doi: 10.1097/GRF.0000000000000494. PMID: 31725416
Acute Fatty Liver Disease of Pregnancy: Updates in Pathogenesis, Diagnosis, and Management.
Liu J, Ghaziani TT, Wolf JL
Am J Gastroenterol 2017 Jun;112(6):838-846. Epub 2017 Mar 14 doi: 10.1038/ajg.2017.54. PMID: 28291236
Mechanisms of intrahepatic triglyceride accumulation.
Ress C, Kaser S
World J Gastroenterol 2016 Jan 28;22(4):1664-73. doi: 10.3748/wjg.v22.i4.1664. PMID: 26819531Free PMC Article
Liver Failure in Pregnancy.
Bacak SJ, Thornburg LL
Crit Care Clin 2016 Jan;32(1):61-72. Epub 2015 Oct 9 doi: 10.1016/j.ccc.2015.08.005. PMID: 26600444

Clinical prediction guides

Comparing acute fatty liver of pregnancy from hemolysis, elevated liver enzymes, and low platelets syndrome.
Byrne JJ, Seasely A, Nelson DB, Mcintire DD, Cunningham FG
J Matern Fetal Neonatal Med 2022 Apr;35(7):1352-1362. Epub 2020 Apr 19 doi: 10.1080/14767058.2020.1754790. PMID: 32308076
Acute Fatty Liver of Pregnancy.
Ademiluyi A, Amakye DO, Jackson N, Betty S
Am J Case Rep 2021 Nov 28;22:e933252. doi: 10.12659/AJCR.933252. PMID: 34839346Free PMC Article
Acute Fatty Liver Disease of Pregnancy: Updates in Pathogenesis, Diagnosis, and Management.
Liu J, Ghaziani TT, Wolf JL
Am J Gastroenterol 2017 Jun;112(6):838-846. Epub 2017 Mar 14 doi: 10.1038/ajg.2017.54. PMID: 28291236
Advances in understanding and treating liver diseases during pregnancy: A review.
Kamimura K, Abe H, Kawai H, Kamimura H, Kobayashi Y, Nomoto M, Aoyagi Y, Terai S
World J Gastroenterol 2015 May 7;21(17):5183-90. doi: 10.3748/wjg.v21.i17.5183. PMID: 25954092Free PMC Article
Liver disease in pregnancy.
Lee NM, Brady CW
World J Gastroenterol 2009 Feb 28;15(8):897-906. doi: 10.3748/wjg.15.897. PMID: 19248187Free PMC Article

Recent systematic reviews

Plasma exchange improves survival in acute liver failure - An updated systematic review and meta-analysis focussed on comparing within single etiology.
Sithamparapillai K, Zachariah U, Eapen CE, Goel A
Indian J Gastroenterol 2024 Apr;43(2):397-406. Epub 2024 May 1 doi: 10.1007/s12664-024-01557-7. PMID: 38691239
Leptospirosis in pregnancy: A systematic review.
Selvarajah S, Ran S, Roberts NW, Nair M
PLoS Negl Trop Dis 2021 Sep;15(9):e0009747. Epub 2021 Sep 14 doi: 10.1371/journal.pntd.0009747. PMID: 34520461Free PMC Article
Treating acute fatty liver of pregnancy with artificial liver support therapy: Systematic review.
Wu Z, Huang P, Gong Y, Wan J, Zou W
Medicine (Baltimore) 2018 Sep;97(38):e12473. doi: 10.1097/MD.0000000000012473. PMID: 30235743Free PMC Article
Effect of caesarean section on maternal and foetal outcomes in acute fatty liver of pregnancy: a systematic review and meta-analysis.
Wang HY, Jiang Q, Shi H, Xu YQ, Shi AC, Sun YL, Li J, Ning Q, Shen GX
Sci Rep 2016 Jul 8;6:28826. doi: 10.1038/srep28826. PMID: 27387594Free PMC Article

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