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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 a mixture of major and minor genetic determinants possibly together with environmental factors. Diseases inherited in this manner are termed complex diseases.
 
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

Cadoret F, Guerby P, Cavaignac-Vitalis M, Vayssiere C, Parant O, Vidal F
J Matern Fetal Neonatal Med 2021 Dec;34(24):4029-4034. Epub 2020 Mar 9 doi: 10.1080/14767058.2019.1702956. PMID: 32146852
Erez O, Mastrolia SA, Thachil J
Am J Obstet Gynecol 2015 Oct;213(4):452-63. Epub 2015 Mar 31 doi: 10.1016/j.ajog.2015.03.054. PMID: 25840271
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
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
Westbrook RH, Dusheiko G, Williamson C
J Hepatol 2016 Apr;64(4):933-45. Epub 2015 Nov 30 doi: 10.1016/j.jhep.2015.11.030. PMID: 26658682
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

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
Fardelmann KL, Alian AA
Anesthesiol Clin 2020 Mar;38(1):85-105. doi: 10.1016/j.anclin.2019.10.005. PMID: 32008659
Palomo M, Blasco M, Molina P, Lozano M, Praga M, Torramade-Moix S, Martinez-Sanchez J, Cid J, Escolar G, Carreras E, Paules C, Crispi F, Quintana LF, Poch E, Rodas L, Goma E, Morelle J, Espinosa M, Morales E, Avila A, Cabello V, Ariceta G, Chocron S, Manrique J, Barros X, Martin N, Huerta A, Fraga-Rodriguez GM, Cao M, Martin M, Romera AM, Moreso F, Manonelles A, Gratacos E, Pereira A, Campistol JM, Diaz-Ricart M
Clin J Am Soc Nephrol 2019 Dec 6;14(12):1719-1732. Epub 2019 Nov 6 doi: 10.2215/CJN.05830519. PMID: 31694864Free PMC Article
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

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
Chandrasekaran S, Simon R
Clin Obstet Gynecol 2020 Mar;63(1):165-174. doi: 10.1097/GRF.0000000000000501. PMID: 31770122
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
Conde-Agudelo A, Romero R
Am J Obstet Gynecol 2022 Jan;226(1):68-89.e3. Epub 2021 Jul 21 doi: 10.1016/j.ajog.2021.07.009. PMID: 34302772Free 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
Palomo M, Blasco M, Molina P, Lozano M, Praga M, Torramade-Moix S, Martinez-Sanchez J, Cid J, Escolar G, Carreras E, Paules C, Crispi F, Quintana LF, Poch E, Rodas L, Goma E, Morelle J, Espinosa M, Morales E, Avila A, Cabello V, Ariceta G, Chocron S, Manrique J, Barros X, Martin N, Huerta A, Fraga-Rodriguez GM, Cao M, Martin M, Romera AM, Moreso F, Manonelles A, Gratacos E, Pereira A, Campistol JM, Diaz-Ricart M
Clin J Am Soc Nephrol 2019 Dec 6;14(12):1719-1732. Epub 2019 Nov 6 doi: 10.2215/CJN.05830519. PMID: 31694864Free PMC Article

Recent systematic reviews

Conde-Agudelo A, Romero R
Am J Obstet Gynecol 2022 Jan;226(1):68-89.e3. Epub 2021 Jul 21 doi: 10.1016/j.ajog.2021.07.009. PMID: 34302772Free PMC Article
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
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
Browne JL, Schrier VJ, Grobbee DE, Peters SA, Klipstein-Grobusch K
J Acquir Immune Defic Syndr 2015 Sep 1;70(1):91-8. doi: 10.1097/QAI.0000000000000686. PMID: 26322669
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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