MCID: HLL004
MIFTS: 57

Hellp Syndrome malady

Liver diseases, Blood diseases categories

Summaries for Hellp Syndrome

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42NIH Rare Diseases, 63Wikipedia, 32MalaCards
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NIH Rare Diseases:42 Hellp syndrome is a rare but serious illness in pregnancy. this condition can start quickly, most often in the last 3 months of pregnancy (the third trimester). it can also start soon after the baby is delivered. hellp stands for hemolysis, elevated liver enzyme levels and a low platelet count. these problems are often found in women with this syndrome. these problems, along with the high blood pressure commonly found in association with hellp syndrome, can lead to problems for both the mother and the baby. many cases of hellp syndrome are associated with  preeclampsia or eclampsia. the exact cause of hellp syndrome remains unknown, making it difficult to determine which women will be affected. treatment may involve medication and/or delivery of the baby.    last updated: 1/26/2010

MalaCards: Hellp Syndrome, also known as syndrome of haemolysis, elevated liver enzymes and low platelet, is related to eclampsia and hepatitis. An important gene associated with Hellp Syndrome is HADHA (hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit), and among its related pathways are Platelet degranulation and Complement and coagulation cascades. The compounds estrogen and warfarin have been mentioned in the context of this disorder. Affiliated tissues include liver, placenta and endothelial, and related mouse phenotypes are liver/biliary system and cardiovascular system.

Wikipedia:63 HELLP syndrome is a life-threatening obstetric complication usually considered to be a variant or... more...

Aliases & Classifications for Hellp Syndrome

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8Disease Ontology, 42NIH Rare Diseases, 44Novoseek, 60UMLS, 56SNOMED-CT, 39NCIt, 34MeSH, 25ICD10
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Liver diseases, Blood diseases


Aliases & Descriptions:

hellp syndrome 8 42 44 60
syndrome of haemolysis, elevated liver enzymes and low platelet 8
hemolysis, elevated liver enzymes, lowered platelets 42


External Ids:

Disease Ontology8 DOID:13133
NCIt39 C84750
SNOMED-CT56 199010001, 95605009
MeSH34 D017359
ICD1025 O14.2

Related Diseases for Hellp Syndrome

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17GeneCards, 18GeneDecks
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Diseases related to Hellp Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 212)
idRelated DiseaseScoreTop Affiliating Genes
1eclampsia31.2MTHFR, SERPINC1
2hepatitis31.1SLC17A5, GPT
3pre-eclampsia31.1APOH, HADHA, F5, MTHFR, EPHX2, AHSP
4antiphospholipid syndrome30.9APOH, F5, MTHFR, VWF, SERPINC1, ADAMTS13
5hypertension30.8EPHX2, SERPINE1, MTHFR, F5
6severe pre-eclampsia30.7APOH, F5, SERPINE1, SERPINC1, GSTP1
7thrombocytopenia30.6APOH, F5, HP, VWF, GPT, SLC17A5
8lupus erythematosus30.5SERPINC1, F5, APOH
9purpura30.4SERPINC1, ADAMTS13
10pancreatitis30.4SLC17A5, BCHE, GSTP1
11acute pancreatitis30.3SLC17A5, GPT
12hemolytic anemia30.3APOH, HP, VWF, ADAMTS13
13thrombophilia30.3APOH, F5, MTHFR, VWF, SERPINE1, SERPINC1
14hemolytic-uremic syndrome30.2ADAMTS13, VWF
15proteinuria30.2ADAMTS13, HP, APOH
16disseminated intravascular coagulation30.2F5, SERPINE1, SERPINC1, ADAMTS13
17myocardial infarction30.1F5, MTHFR, AHSP, ADAMTS13, SERPINC1, SERPINE1
18malaria29.9ADAMTS13, HP
19sarcoma29.9GSTP1
20central retinal vein occlusion29.9APOH, F5, MTHFR
21portal vein thrombosis29.9F5, MTHFR, SERPINC1
22hepatitis b29.9GPT, SLC17A5
23cystic fibrosis29.9BCHE, OTC, HP, HADHA
24retinal vein occlusion29.9APOH, F5, MTHFR, SERPINC1
25placental abruption29.9F5, MTHFR, SERPINC1
26glucose intolerance29.9GPT, SERPINE1
27hepatic vein thrombosis29.9SERPINC1, SERPINE1
28ischemia29.9ADAMTS13, SLC17A5, HP, AHSP, BNIP3
29acute kidney failure29.9ADAMTS13, GPT, HP
30leukemia29.9MTHFR, HP, OTC, BCHE, GSTP1
31coronary artery disease29.9MTHFR, AHSP, HP, VWF, SERPINE1, BCHE
32hepatitis a10.5
33retinitis10.5
34retinal detachment10.4
35systemic lupus erythematosus10.3
36image syndrome10.3
37cerebritis10.3
38diabetes insipidus10.3
39thrombotic thrombocytopenic purpura10.2
40cortical blindness10.2
41infarct of liver10.2
42pulmonary edema10.1
43cholecystitis10.1
44glomerulonephritis10.1
45hellp syndrome, maternal, of pregnancy10.1
46retinal artery occlusion10.0APOH
47ovarian hyperstimulation syndrome10.0F5
48pneumonia10.0BCHE
49alpha thalassemia10.0AHSP
50dementia10.0BCHE

Graphical network of the top 20 diseases related to Hellp Syndrome:



Diseases related to hellp syndrome

Clinical Features for Hellp Syndrome

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Drugs & Therapeutics for Hellp Syndrome

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Hellp Syndrome

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Anatomical Context for Hellp Syndrome

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32MalaCards
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MalaCards organs/tissues related to Hellp Syndrome:

32
Liver, Placenta, Endothelial, Testes, Kidney, Brain, Bone, Myeloid, Cervix, Bone marrow, Uterus, Skin, Lung, Spinal cord, Pituitary

Animal Models for Hellp Syndrome or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Hellp Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537010.2SERPINC1, SERPINE1, OTC, HP, F5, HADHA
2MP:000538510.0BNIP3, HADHA, EPHX2, HP
3MP:00053769.8ADAMTS13, BNIP3, APOH, HADHA, F5, MTHFR
4MP:00107689.7AHSP, MTHFR, F5, HADHA, APOH, HP

Publications for Hellp Syndrome

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50PubMed
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Articles related to Hellp Syndrome:

(show top 50)    (show all 523)
idTitleAuthorsYear
1
Placental syncytiotrophoblast maintains a specific type of glycocalyx at the fetomaternal border: the glycocalyx at the fetomaternal interface in healthy women and patients with HELLP syndrome. (23585336)
2013
2
Bilateral Exudative Retinal Detachment in HELLP Syndrome. (22615699)
2012
3
HELLP syndrome with postpartum hepatic infarction and subcapsular bleeding. (22515349)
2012
4
A case of video-assisted retroperitoneal debridement in a patient with HELLP syndrome. (22678339)
2012
5
Good outcome in HELLP syndrome with lobar cerebral hematomas. (21161697)
2011
6
Biochemistry of HELLP syndrome. (21404915)
2011
7
Factors affecting maternal and perinatal outcomes in HELLP syndrome: evaluation of 126 cases. (21077527)
2010
8
Cost-effectiveness of recurrence risk guided care versus care as usual in women who suffered from early-onset preeclampsia including HELLP syndrome in their previous pregnancy (the PreCare study). (20932350)
2010
9
Short- and long-term outcome of infants born after maternal (pre)-eclampsia, HELLP syndrome and thrombophilia: a retrospective, cohort study. (20673607)
2010
10
Overrepresentation of BclI polymorphism of the glucocorticoid receptor gene in pregnant women with HELLP syndrome. (19336230)
2009
11
Postpartum spontaneous rupture of a liver hematoma associated with preeclampsia and HELLP syndrome. (18979111)
2009
12
Regional anaesthesia for primary caesarean section in patients with preterm HELLP syndrome: a review of 102 cases. (20101854)
2009
13
HELLP syndrome A-Z: facing an obstetric emergency. (19748446)
2009
14
Postpartum spinal cord injury in a woman with HELLP syndrome. (18795483)
2008
15
Alpha-hemoglobin-stabilizing protein (AHSP) in hemolysis, elevated liver enzyme, and low platelet (HELLP) syndrome, intrauterine growth restriction (IUGR) and fetal death. (18347943)
2008
16
Differential gene expression profiling in HELLP syndrome placentas. (18421023)
2008
17
Purtscher-like retinopathy in a patient with HELLP syndrome. (17452181)
2007
18
Amniotic fluid index as a predictor of adverse perinatal outcome in the HELLP syndrome. (17506369)
2007
19
In situ split liver transplantation using a donor with a recent history of HELLP syndrome. (16495819)
2006
20
Risk factors for post-partum complications occurring after preeclampsia and HELLP syndrome. A study in 453 consecutive pregnancies. (16118033)
2006
21
The transplacental passage of prednisolone in pregnancies complicated by early-onset HELLP syndrome. (16226134)
2005
22
Permanent visual deficits secondary to the HELLP syndrome. (15937436)
2005
23
The HELLP syndrome in the antiphospholipid syndrome: retrospective study of 16 cases in 15 women. (15647435)
2005
24
HELLP syndrome as a cause of unexpected rapid maternal death--a case report and review of the literature. (15744546)
2005
25
Acute renal failure due to HELLP syndrome and acute renal failure in mid gestation. (15935639)
2005
26
Alteration of homocysteine catabolism in pre-eclampsia, HELLP syndrome and placental insufficiency. (15552268)
2004
27
Dexamethasone compared with betamethasone for glucocorticoid treatment of postpartum HELLP syndrome. (12628531)
2003
28
Results of the pregnancies with HELLP syndrome. (12911166)
2003
29
Plasma VEGF levels are increased in women with severe preeclampsia or HELLP syndrome]. (12891468)
2003
30
Acute renal failure complicating HELLP syndrome, SLE and anti-phospholipid syndrome: successful outcome using plasma exchange therapy. (12729047)
2003
31
The impact of parity on the incidence of HELLP syndrome and small for gestational age infants in hypertensive pregnant women. (12196855)
2002
32
Successful use of recombinant factor VIIa for massive bleeding after caesarean section due to HELLP syndrome. (12373090)
2002
33
Spontaneous liver hematoma and a hepatic rupture in HELLP syndrome: report of two cases. (12061700)
2002
34
Clinical and biophysical aspects of HELLP-syndrome. (12530105)
2002
35
Vasospasms are characteristic in cases with eclampsia/preeclampsia and HELLP syndrome: proposal of an angiospastic syndrome of pregnancy. (11372766)
2001
36
Corticosteroid therapy for conservative management in marginally-viable pregnancy complicated by HELLP syndrome. (10584642)
1999
37
Continuous renal replacement therapy in patients with HELLP syndrome. (10394815)
1999
38
Relation between gestational thrombocytopenia and the syndrome of hemolysis, elevated liver enzymes, and low platelet count (HELLP syndrome). (9692341)
1998
39
Obstetrical anaesthesia for a parturient with preeclampsia, HELLP syndrome and acute cortical blindness. (9598260)
1998
40
Plasmapheresis in the management of HELLP syndrome. (15512115)
1998
41
CT demonstration of resolution of hepatic lesions in HELLP syndrome: a case report. (9505565)
1998
42
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) pathophysiology and anesthetic considerations. (9223938)
1997
43
The HELLP syndrome at 16 weeks gestation: possible association with the antiphospholipid syndrome. (9325513)
1997
44
Postpartum recovery after severe pre-eclampsia and HELLP-syndrome. (9120747)
1996
45
Plasma exchange in a patient with postpartum HELLP syndrome. (8870421)
1996
46
HELLP syndrome: Clinical profile of seven patients. (17372423)
1996
47
Coagulation and plasma fibronectin parameters in HELLP syndrome. (8543109)
1995
48
HELLP syndrome: laboratory parameters and clinical course in four patients treated with plasma exchange. (7759467)
1994
49
Plasma exchange for preeclampsia: II. Unsuccessful antepartum utilization for severe preeclampsia with or without HELLP syndrome. (7706195)
1994
50
A case of mild ocular manifestations in pregnancy induced hypertension with HELLP syndrome. (7976276)
1994

Genetic Variations for Hellp Syndrome

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Expression for genes affiliated with Hellp Syndrome

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Hellp Syndrome

Search GEO for disease gene expression data for Hellp Syndrome.

Pathways for genes affiliated with Hellp Syndrome

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53Reactome, 29KEGG, 37NCBI BioSystems Database, 52R&D Systems
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Pathways related to Hellp Syndrome according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
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10.3SERPINE1, VWF, F5
2
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10.3F5, VWF, SERPINE1, SERPINC1
3
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10.3SERPINC1, SERPINE1, VWF, F5

Compounds for genes affiliated with Hellp Syndrome

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44Novoseek, 49PharmGKB, 11DrugBank, 24HMDB, 2BitterDB, 28IUPHAR, 59Tocris Bioscience
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Compounds related to Hellp Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 178)
idCompoundScoreTop Affiliating Genes
1estrogen4410.8APOH, AHSP
2warfarin44 49 11 2413.7SERPINC1, VWF, APOH
3aspartate4410.6GPT, SLC17A5, OTC, BCHE, EPHX2
4acetaminophen44 2 49 11 2414.6GPT, EPHX2, SLC17A5
5heparin44 28 11 2413.6APOH, ADAMTS13, MTHFR, AHSP, HP, BCHE
6desmopressin44 59 28 1113.6ADAMTS13, SERPINC1, F5, VWF, SERPINE1
7ristocetin4410.6F5, SERPINC1, ADAMTS13, VWF
8ticlopidine44 1111.6VWF, APOH, SERPINC1, ADAMTS13
9kininogen4410.6SERPINE1, SERPINC1, F5, VWF, HP, APOH
10malondialdehyde4410.6F5, HP, GPT, SLC17A5, APOH
11valproate4410.6SLC17A5, MTHFR, GPT, OTC, EPHX2
12serine4410.6HADHA, EPHX2, HP, ADAMTS13, BCHE
13vitamin b124410.6SLC17A5, VWF, F5, MTHFR, HP, GPT
14urea44 11 2412.6OTC, EPHX2, HP, SLC17A5, GPT, F5
15uric acid44 2411.6APOH, HP, GPT, SLC17A5, SERPINE1, F5
16metformin44 49 1112.6GPT, SLC17A5, MTHFR, SERPINE1, VWF, F5
17glutamate4410.6APOH, HADHA, EPHX2, AHSP, GPT, OTC
18pge14410.5BCHE, F5, APOH, VWF, SERPINE1, SLC17A5
19endotoxin4410.5ADAMTS13, SERPINE1, HP, AHSP, F5, APOH
20folate4410.5GSTP1, APOH, F5, MTHFR, HP, SLC17A5
21simvastatin44 49 59 28 11 2415.5BCHE, VWF, GPT, SLC17A5, F5, SERPINE1
22aprotinin44 1111.5VWF, SLC17A5, SERPINC1, F5, SERPINE1
23prostacyclin4410.5HADHA, F5, SERPINE1, APOH, SERPINC1, VWF
24homocysteine44 2411.5HP, AHSP, F5, APOH, MTHFR, SERPINC1
25polyethylene glycol4410.5SERPINC1, BCHE, F5, SLC17A5, HP
26nicotine44 49 28 1113.5AHSP, SERPINE1, SERPINC1, GPT, BCHE, VWF
27ascorbic acid44 2411.5GSTP1, VWF, HP, F5, SERPINC1, MTHFR
28cyclophosphamide44 49 1112.5MTHFR, SERPINE1, BCHE, ADAMTS13, APOH, GSTP1
29glucose4410.5BCHE, AHSP, OTC, BNIP3, APOH, HADHA
30valproic acid44 49 11 2413.4EPHX2, GPT, SLC17A5, OTC, MTHFR
31methotrexate44 49 1112.4MTHFR, SLC17A5, GPT, SERPINE1, GSTP1, APOH
32sodium dodecylsulfate4410.4F5, HP, VWF, GSTP1, SERPINE1
33testosterone44 59 11 2413.4SERPINE1, SLC17A5, GSTP1, F5, VWF, HP
34arachidonic acid44 28 11 2413.4HADHA, HP, VWF, AHSP, EPHX2, LGALS13
35fibrinogen4410.4MTHFR, APOH, F5, ADAMTS13, SERPINC1, SERPINE1
36alanine4410.4HP, VWF, GPT, F5, GSTP1, SLC17A5
37cholesterol44 28 11 2413.4AHSP, SERPINE1, F5, APOH, SLC17A5, MTHFR
38lactate4410.4BCHE, BNIP3, APOH, HADHA, F5, HP
39aspirin44 49 28 2413.4SERPINC1, BCHE, GSTP1, SERPINE1, VWF, HADHA
40glutamine4410.4OTC, EPHX2, SLC17A5, GPT, VWF, HADHA
41hepaplastin4410.3GPT, SLC17A5, SERPINC1
42arginine4410.3BCHE, SERPINC1, OTC, GPT, GSTP1, F5
43ancrod4410.3SERPINC1, SERPINE1, F5
44atorvastatin44 49 28 11 2414.3SERPINE1, F5, VWF, GPT, SLC17A5
45nitric oxide44 11 2412.3BNIP3, SERPINE1, APOH, VWF, OTC, SLC17A5
46p0024410.3HP, APOH, F5, GPT, MTHFR
47creatinine4410.2SLC17A5, APOH, HADHA, F5, MTHFR, HP
48vegf4410.2SERPINE1, HP, SERPINC1, F5, GSTP1, APOH
49hirudin4410.2F5, SERPINC1, SERPINE1, VWF
50ximelagatran44 1111.0SERPINC1, GPT, F5

GO Terms for genes affiliated with Hellp Syndrome

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16Gene Ontology
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Cellular components related to Hellp Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet alpha granule lumenGO:03109310.3SERPINE1, VWF, F5
2extracellular spaceGO:0056159.8ADAMTS13, APOH, F5, HP, SERPINE1, SERPINC1

Biological processes related to Hellp Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1negative regulation of fibrinolysisGO:05191810.4SERPINE1, APOH
2negative regulation of blood coagulationGO:03019510.4SERPINE1, APOH
3platelet activationGO:03016810.3ADAMTS13, SERPINE1, VWF, F5
4platelet degranulationGO:00257610.2F5, VWF, SERPINE1
5positive regulation of blood coagulationGO:03019410.1SERPINE1, APOH

Molecular functions related to Hellp Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protease bindingGO:00202010.3SERPINC1, SERPINE1, VWF
2hemoglobin bindingGO:03049210.1HP, AHSP

Products for genes affiliated with Hellp Syndrome

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Sources for Hellp Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet