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HELLP syndrome(HELLP)

MedGen UID:
58162
Concept ID:
C0162739
Disease or Syndrome
Synonyms: HELLP; Hemolysis, elevated liver enzymes, and low platelet count; Hemolysis, Elevated Liver Enzymes, Lowered Platelets
SNOMED CT: HELLP - Syndrome of hemolysis, elevated liver enzymes and low platelet (95605009); Hemolysis-elevated liver enzymes-low platelet count syndrome (95605009); HELLP syndrome (95605009)
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.
 
Monarch Initiative: MONDO:0008585
OMIM®: 189800
Orphanet: ORPHA244242

Definition

A rare hemorrhagic disorder due to an acquired platelet anomaly characterized by hemolysis, elevated liver enzymes and thrombocytopenia that affects pregnant or post-partum women, and is frequently associated with severe preeclampsia. Symptoms are variable, typically including right upper quadrant or epigastric abdominal pain, nausea, vomiting, excessive weight gain, generalized edema, hypertension, general malaise, right shoulder pain, backache, and/or headache. Hepatic hemorrhage and rupture, renal failure, and pulmonary edema can result in maternal and/or fetal death. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHELLP syndrome

Professional guidelines

PubMed

Gauer RL, Whitaker DJ
Am Fam Physician 2022 Sep;106(3):288-298. PMID: 36126009
Ditisheim A, Sibai BM
Clin Obstet Gynecol 2017 Mar;60(1):190-197. doi: 10.1097/GRF.0000000000000253. PMID: 28005587
Haram K, Svendsen E, Abildgaard U
BMC Pregnancy Childbirth 2009 Feb 26;9:8. doi: 10.1186/1471-2393-9-8. PMID: 19245695Free PMC Article

Recent clinical studies

Etiology

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
Petca A, Miron BC, Pacu I, Dumitrașcu MC, Mehedințu C, Șandru F, Petca RC, Rotar IC
Medicina (Kaunas) 2022 Feb 21;58(2) doi: 10.3390/medicina58020326. PMID: 35208649Free PMC Article
Zununi Vahed S, Rahbar Saadat Y, Ardalan M
Microvasc Res 2021 Nov;138:104226. Epub 2021 Jul 9 doi: 10.1016/j.mvr.2021.104226. PMID: 34252400
Alese MO, Moodley J, Naicker T
J Matern Fetal Neonatal Med 2021 Jan;34(1):117-123. Epub 2019 Jan 31 doi: 10.1080/14767058.2019.1572737. PMID: 30704316
Haram K, Svendsen E, Abildgaard U
BMC Pregnancy Childbirth 2009 Feb 26;9:8. doi: 10.1186/1471-2393-9-8. PMID: 19245695Free PMC Article

Diagnosis

Adorno M, Maher-Griffiths C, Grush Abadie HR
Crit Care Nurs Clin North Am 2022 Sep;34(3):277-288. Epub 2022 Jul 20 doi: 10.1016/j.cnc.2022.04.009. PMID: 36049847
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
Petca A, Miron BC, Pacu I, Dumitrașcu MC, Mehedințu C, Șandru F, Petca RC, Rotar IC
Medicina (Kaunas) 2022 Feb 21;58(2) doi: 10.3390/medicina58020326. PMID: 35208649Free PMC Article
Alese MO, Moodley J, Naicker T
J Matern Fetal Neonatal Med 2021 Jan;34(1):117-123. Epub 2019 Jan 31 doi: 10.1080/14767058.2019.1572737. PMID: 30704316
Wallace K, Harris S, Addison A, Bean C
Curr Pharm Biotechnol 2018;19(10):816-826. doi: 10.2174/1389201019666180712115215. PMID: 29998801

Therapy

Gauer RL, Whitaker DJ
Am Fam Physician 2022 Sep;106(3):288-298. PMID: 36126009
Katsi V, Skalis G, Vamvakou G, Tousoulis D, Makris T
Curr Hypertens Rep 2020 Aug 6;22(8):58. doi: 10.1007/s11906-020-01058-w. PMID: 32761267
Wallace K, Harris S, Addison A, Bean C
Curr Pharm Biotechnol 2018;19(10):816-826. doi: 10.2174/1389201019666180712115215. PMID: 29998801
Haram K, Svendsen E, Abildgaard U
BMC Pregnancy Childbirth 2009 Feb 26;9:8. doi: 10.1186/1471-2393-9-8. PMID: 19245695Free PMC Article
Sibai BM
Obstet Gynecol 2007 Apr;109(4):956-66. doi: 10.1097/01.AOG.0000258281.22296.de. PMID: 17400860

Prognosis

Petca A, Miron BC, Pacu I, Dumitrașcu MC, Mehedințu C, Șandru F, Petca RC, Rotar IC
Medicina (Kaunas) 2022 Feb 21;58(2) doi: 10.3390/medicina58020326. PMID: 35208649Free PMC Article
Erez O, Romero R, Jung E, Chaemsaithong P, Bosco M, Suksai M, Gallo DM, Gotsch F
Am J Obstet Gynecol 2022 Feb;226(2S):S786-S803. doi: 10.1016/j.ajog.2021.12.001. PMID: 35177220Free PMC Article
Alese MO, Moodley J, Naicker T
J Matern Fetal Neonatal Med 2021 Jan;34(1):117-123. Epub 2019 Jan 31 doi: 10.1080/14767058.2019.1572737. PMID: 30704316
Vest AR, Cho LS
Curr Atheroscler Rep 2014 Mar;16(3):395. doi: 10.1007/s11883-013-0395-8. PMID: 24477794
Haram K, Svendsen E, Abildgaard U
BMC Pregnancy Childbirth 2009 Feb 26;9:8. doi: 10.1186/1471-2393-9-8. PMID: 19245695Free PMC Article

Clinical prediction guides

Erez O, Romero R, Jung E, Chaemsaithong P, Bosco M, Suksai M, Gallo DM, Gotsch F
Am J Obstet Gynecol 2022 Feb;226(2S):S786-S803. doi: 10.1016/j.ajog.2021.12.001. PMID: 35177220Free PMC Article
Zununi Vahed S, Rahbar Saadat Y, Ardalan M
Microvasc Res 2021 Nov;138:104226. Epub 2021 Jul 9 doi: 10.1016/j.mvr.2021.104226. PMID: 34252400
Alese MO, Moodley J, Naicker T
J Matern Fetal Neonatal Med 2021 Jan;34(1):117-123. Epub 2019 Jan 31 doi: 10.1080/14767058.2019.1572737. PMID: 30704316
Elkafrawi D, Sisti G, Araji S, Khoury A, Miller J, Rodriguez Echevarria B
Medicina (Kaunas) 2020 Apr 13;56(4) doi: 10.3390/medicina56040174. PMID: 32295061Free PMC Article
Sandvoß M, Potthast AB, von Versen-Höynck F, Das AM
Reprod Sci 2017 Apr;24(4):568-574. Epub 2016 Sep 27 doi: 10.1177/1933719116667216. PMID: 27651178

Recent systematic reviews

Sun WJ, Hu J, Zhang Q, Shan JM
Hypertens Pregnancy 2023 Dec;42(1):2276726. Epub 2023 Nov 8 doi: 10.1080/10641955.2023.2276726. PMID: 37937841
Mossayebi MH, Iyer NS, McLaren RA Jr, Moussa HN, Sibai BM, Al-Kouatly HB
Am J Obstet Gynecol 2023 Nov;229(5):502-515.e10. Epub 2023 May 5 doi: 10.1016/j.ajog.2023.04.046. PMID: 37150281
Dubey S, Rani J
Taiwan J Obstet Gynecol 2020 Sep;59(5):643-651. doi: 10.1016/j.tjog.2020.07.003. PMID: 32917311
Hofmeyr GJ, Lawrie TA, Atallah ÁN, Torloni MR
Cochrane Database Syst Rev 2018 Oct 1;10(10):CD001059. doi: 10.1002/14651858.CD001059.pub5. PMID: 30277579Free PMC Article
Appenzeller S, Souza FH, Wagner Silva de Souza A, Shoenfeld Y, de Carvalho JF
Semin Arthritis Rheum 2011 Dec;41(3):517-23. Epub 2011 Aug 24 doi: 10.1016/j.semarthrit.2011.05.007. PMID: 21868064

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