MCID: HLL004
MIFTS: 54

Hellp Syndrome malady

Rare diseases, Blood diseases, Cardiovascular diseases, Liver diseases categories
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Summaries for Hellp Syndrome

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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 based summary: Hellp Syndrome, also known as syndrome of haemolysis, elevated liver enzymes and low platelet, is related to eclampsia and purpura. 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 Complement and coagulation cascades and Formation of Fibrin Clot (Clotting Cascade). The compounds inogatran and hirugen have been mentioned in the context of this disorder. Affiliated tissues include liver, placenta and testes, and related mouse phenotypes are liver/biliary system and homeostasis/metabolism.

Wikipedia:65 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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Hellp Syndrome, Aliases & Descriptions:

Name: Hellp Syndrome 8 42 44 62
Syndrome of Haemolysis, Elevated Liver Enzymes and Low Platelet 8
 
Hemolysis, Elevated Liver Enzymes, Lowered Platelets 42


Classifications:



External Ids:

Disease Ontology8 DOID:13133
MeSH34 D017359
SNOMED-CT57 95605009, 199010001
NCIt39 C84750
ICD1025 O14.2

Related Diseases for Hellp Syndrome

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

(show top 50)    (show all 137)
idRelated DiseaseScoreTop Affiliating Genes
1eclampsia32.0SERPINC1
2purpura30.8SERPINC1, ADAMTS13
3pancreatitis30.6SLC17A5, BCHE
4severe pre-eclampsia30.6APOH, SERPINC1, F5
5lupus erythematosus30.4F5, SERPINC1, APOH
6placental abruption30.3SERPINC1, F5
7antiphospholipid syndrome30.3APOH, SERPINC1, ADAMTS13, F5
8cholestasis30.3HADHA, SLC17A5
9acute kidney failure30.3HP, ADAMTS13
10thrombophilia30.2APOH, SERPINC1, F5
11disseminated intravascular coagulation30.2SERPINC1, ADAMTS13, F5
12hemolytic anemia30.2APOH, ADAMTS13, HP
13central retinal vein occlusion30.2APOH, F5
14cystic fibrosis30.1HADHA, HP, BCHE
15proteinuria30.0HP, ADAMTS13, APOH
16lupus nephritis29.9APOH, ADAMTS13
17ischemia29.9SLC17A5, HP, ADAMTS13
18myocardial infarction29.9SERPINC1, ADAMTS13, F5
19retinal vein occlusion29.9APOH, SERPINC1, F5
20pre-eclampsia29.6SLC17A5, F5, BCHE, HP, ADAMTS13, SERPINC1
21thrombocytopenia29.3SLC17A5, F5, BCHE, HP, ADAMTS13, SERPINC1
22hepatitis10.8
23hypertension10.6
24retinitis10.6
25retinal detachment10.5
26cholangitis10.4SLC17A5
27coronary thrombosis10.4SERPINC1
28branch retinal artery occlusion10.4F5
29patent foramen ovale10.4F5
30evans' syndrome10.4ADAMTS13
31systemic lupus erythematosus10.4
32diabetes insipidus10.4
33cerebritis10.4
34blindness10.4
35retinal artery occlusion10.3APOH
36acute fatty liver of pregnancy10.3
37endotheliitis10.3
38thrombophlebitis10.3APOH
39congenital disorder of glycosylation type 1a10.3HP, SERPINC1
40splenomegaly10.3SLC17A5, HP
41cortical blindness10.3
42thrombotic thrombocytopenic purpura10.3
43post-thrombotic syndrome10.2F5, SERPINC1
44legg-calve-perthes disease10.2SERPINC1, F5
45sagittal sinus thrombosis10.2SERPINC1, F5
46thrombocytosis10.2HP, SERPINC1
47thrombotic thrombocytopenic purpura, acquired10.2ADAMTS13, HP
48blood protein disease10.2SERPINC1, F5
49portal vein thrombosis10.2F5, SERPINC1
50dysfibrinogenemia10.2SERPINC1, F5

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



Diseases related to hellp syndrome

Symptoms for Hellp Syndrome

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Drugs & Therapeutics for Hellp Syndrome

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Drug clinical trials:

Search ClinicalTrials for Hellp Syndrome

Search NIH Clinical Center for Hellp Syndrome

Genetic Tests for Hellp Syndrome

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Anatomical Context for Hellp Syndrome

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MalaCards organs/tissues related to Hellp Syndrome:

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

Animal Models for Hellp Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Hellp Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.8F5, HP, SERPINC1, HADHA
2MP:00053766.3SLC17A5, HADHA, APOH, SERPINC1, ADAMTS13, HP
3MP:00107686.1HADHA, APOH, SERPINC1, ADAMTS13, HP, BCHE

Publications for Hellp Syndrome

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Articles related to Hellp Syndrome:

(show top 50)    (show all 527)
idTitleAuthorsYear
1
Postpartum HELLP syndrome and subcapsular liver haematoma. (24789156)
2014
2
Plasma From Patients With HELLP Syndrome Increases Blood-Brain Barrier Permeability. (25194151)
2014
3
CMV infection, TLR-2 and -4 expression, and cytokine profiles in early-onset preeclampsia with HELLP syndrome. (24612145)
2014
4
Changes of placental syndecan-1 expression in preeclampsia and HELLP syndrome. (23807541)
2013
5
COHELLP: collaborative randomized controlled trial on corticosteroids in HELLP syndrome. (23697398)
2013
6
HELLP syndrome and its relation with the antiphospholipid syndrome. (24333078)
2013
7
Use of recombinant human soluble thrombomodulin in the management of HELLP syndrome complicated by DIC. (20627281)
2010
8
The HELLP syndrome. (20491358)
2010
9
Leptin receptor (LEPR) SNP polymorphisms in HELLP syndrome patients determined by quantitative real-time PCR and melting curve analysis. (20149225)
2010
10
Gestational diabetes insipidus, HELLP syndrome and eclampsia in a twin pregnancy: a case report. (20118941)
2010
11
Maternal deaths due to eclampsia and HELLP syndrome. (19027902)
2009
12
ABO incompatible liver transplantation as a bridge to treat HELLP syndrome. (19794830)
2009
13
Prevalence rate of acute renal failure in HELLP syndrome: a summary from a single country's data. (19839862)
2009
14
Bilateral serous retinal detachment in a woman with HELLP syndrome HELLP syndrome and retinal detachment. (19358039)
2009
15
The HELLP syndrome, antiphospholipid antibodies, and syndromes. (17912576)
2008
16
Mannose-binding lectin (MBL) codon 54 gene polymorphism protects against development of pre-eclampsia, HELLP syndrome and pre-eclampsia-associated intrauterine growth restriction. (17314117)
2007
17
Dexamethasone, HELLP syndrome, and study design. (16677590)
2006
18
Characteristics and treatment of hepatic rupture caused by HELLP syndrome. (16579935)
2006
19
Argon beam coagulator for treatment of hepatic rupture with hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. (16449174)
2006
20
Addition of platelet transfusions to corticosteroids does not increase the recovery of severe HELLP syndrome. (16388885)
2006
21
Neonatal outcome after preterm delivery in HELLP syndrome. (16807990)
2006
22
Lack of association between the CYP11B2 gene polymorphism and preeclampsia, eclampsia, and the HELLP syndrome in Turkish women. (16303227)
2006
23
Understanding and managing HELLP syndrome: the integral role of aggressive glucocorticoids for mother and child. (16631593)
2006
24
The transplacental passage of prednisolone in pregnancies complicated by early-onset HELLP syndrome. (16226134)
2005
25
Dexamethasone treatment does not improve the outcome of women with HELLP syndrome: a double-blind, placebo-controlled, randomized clinical trial. (16260197)
2005
26
Pseudocholinesterase deficiency associated with HELLP syndrome. (15311366)
2004
27
Case of HELLP syndrome with an unusual presentation. (15242509)
2004
28
Pregnancy complicated by HELLP syndrome]. (15884197)
2004
29
The effect of glyceryl trinitrate on hypertension in women with severe preeclampsia, HELLP syndrome, and eclampsia. (15117599)
2004
30
Dexamethasone compared with betamethasone for glucocorticoid treatment of postpartum HELLP syndrome. (12628531)
2003
31
A case of postpartum cerebellar infarction with hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. (12186992)
2002
32
The impact of parity on the incidence of HELLP syndrome and small for gestational age infants in hypertensive pregnant women. (12196855)
2002
33
HELLP syndrome and factor V Leiden. (11267721)
2001
34
Time course of recovery and complications of HELLP syndrome with two different treatments: heparin or dexamethasone. (11323019)
2001
35
Doppler sonographic findings for hypertension in pregnancy and HELLP syndrome. (10642960)
1999
36
Altered lipid metabolism in preeclampsia and HELLP syndrome: links to enhanced platelet reactivity and fetal growth. (10625202)
1999
37
HELLP syndrome: recognition and perinatal management. (10498110)
1999
38
A review of HELLP syndrome. (10642976)
1999
39
Relation between gestational thrombocytopenia and the syndrome of hemolysis, elevated liver enzymes, and low platelet count (HELLP syndrome). (9692341)
1998
40
Liver function following pregnancy complicated by the HELLP syndrome. (9853772)
1998
41
HELLP syndrome: undiagnosed in a case of impending rupture of uterus. A case report. (9859107)
1998
42
Obstetrical anaesthesia for a parturient with preeclampsia, HELLP syndrome and acute cortical blindness. (9598260)
1998
43
Thrombocytopenia in preeclampsia: an earlier detector of HELLP syndrome. (9361486)
1997
44
Ethnic variation in the incidence of HELLP syndrome in a hypertensive pregnant population. (9494922)
1997
45
HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) pathophysiology and anesthetic considerations. (9223938)
1997
46
Lactate dehydrogenase isoenzymes in patients with HELLP syndrome]. (8963883)
1996
47
Plasma exchange for preeclampsia: III. Immediate peripartal utilization for selected patients with HELLP syndrome. (7706196)
1994
48
An atypical case of hemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome. (7907061)
1994
49
HELLP syndrome: a variant of severe preeclampsia. (8196820)
1994
50
The Hellp syndrome. Clinical and laboratory test results]. (1631474)
1992

Variations for Hellp Syndrome

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Expression for genes affiliated with Hellp Syndrome

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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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Pathways related to Hellp Syndrome according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
Complement Activation, Classical Pathway37
Complement and Coagulation Cascades37
9.5F5, SERPINC1
2
Show member pathways
9.5F5, SERPINC1

Compounds for genes affiliated with Hellp Syndrome

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Compounds related to Hellp Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 127)
idCompoundScoreTop Affiliating Genes
1inogatran449.8SERPINC1, F5
2hirugen449.8SERPINC1, F5
3ecarin449.8SERPINC1, F5
4hepaplastin449.8SLC17A5, SERPINC1
5spectrozyme449.7F5, SERPINC1
6nafamostat mesilate449.6F5, SERPINC1
7danaparoid449.6SERPINC1, F5
8ximelagatran44 1110.5SERPINC1, F5
9levonorgestrel44 61 28 1112.5SLC17A5, F5, SERPINC1
10vitamin-e449.4SLC17A5, HP, F5
11aprotinin44 1110.3F5, SLC17A5, SERPINC1
12ristocetin449.3F5, SERPINC1, ADAMTS13
13desmopressin44 61 28 1112.2ADAMTS13, SERPINC1, F5
14ancrod449.2SERPINC1, F5
15citrate449.2HP, HADHA, SERPINC1, F5
16tween 20449.1BCHE, APOH
17ascorbic acid44 2410.1HP, SLC17A5, F5, SERPINC1
18kaolin449.0APOH, F5
19dextran sulfate449.0F5, SERPINC1, APOH
20warfarin44 50 24 1112.0F5, APOH, SERPINC1
21ticlopidine44 1110.0SERPINC1, ADAMTS13, APOH
22rituximab44 50 1111.0HP, ADAMTS13, APOH
23urea44 24 1111.0ADAMTS13, HP, F5, SLC17A5
24nacl448.9ADAMTS13, F5, HP, SLC17A5
25vitamin b12448.8HP, APOH, F5, SLC17A5
26malondialdehyde448.8F5, SLC17A5, HP, APOH
27uric acid44 249.8HP, SLC17A5, F5, APOH
28kininogen448.7APOH, F5, HP, SERPINC1
29homocysteine44 249.7HP, F5, APOH, SERPINC1
30polyacrylamide448.7HP, SLC17A5, F5, APOH
31prostacyclin448.7SERPINC1, F5, HADHA, APOH
32ethanol44 50 24 1111.7BCHE, SLC17A5, HP, APOH
33phospholipid448.7SLC17A5, F5, APOH, HP
34polyethylene glycol448.6SERPINC1, BCHE, F5, SLC17A5, HP
35pge1448.6APOH, F5, SLC17A5, BCHE
36cocaine44 119.6F5, BCHE, APOH, SLC17A5
37fatty acid448.6BCHE, SLC17A5, APOH, HADHA
38cyclophosphamide44 50 1110.4F5, BCHE, ADAMTS13, APOH
39folate448.3F5, SLC17A5, HP, SERPINC1, APOH
40glucose448.3HP, HADHA, APOH, SLC17A5, BCHE
41alanine448.3F5, SLC17A5, BCHE, SERPINC1, HADHA, HP
42endotoxin448.2SLC17A5, HP, ADAMTS13, APOH, F5
43serine448.0SERPINC1, HP, BCHE, F5, HADHA, ADAMTS13
44lactate447.9HP, HADHA, BCHE, APOH, F5, SLC17A5
45glutamate447.9HADHA, F5, SLC17A5, APOH, BCHE, HP
46aspirin44 50 28 2410.9HP, BCHE, F5, SERPINC1, APOH, HADHA
47fibrinogen447.7SERPINC1, SLC17A5, F5, HP, APOH, ADAMTS13
48calcium44 50 24 1110.4HADHA, APOH, HP, BCHE, ADAMTS13, F5
49heparin44 28 24 1110.2SLC17A5, SERPINC1, APOH, ADAMTS13, HP, BCHE
50creatinine446.9BCHE, HP, ADAMTS13, SERPINC1, F5, HADHA

GO Terms for genes affiliated with Hellp Syndrome

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Cellular components related to Hellp Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1blood microparticleGO:0725628.8SERPINC1, HP, BCHE
2extracellular regionGO:0055768.6F5, BCHE, HP, SERPINC1
3extracellular spaceGO:0056157.1F5, BCHE, HP, ADAMTS13, SERPINC1, APOH

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

idNameGO IDScoreTop Affiliating Genes
1catalytic activityGO:0038249.6BCHE, HP

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
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36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
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51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
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