MCID: IVM001
MIFTS: 34

Ivemark Syndrome malady

Rare diseases, Cardiovascular diseases categories

Summaries for Ivemark Syndrome

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44NIH Rare Diseases, 66Wikipedia, 34MalaCards
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NIH Rare Diseases:44 Ivemark syndrome is a rare condition that affects multiple organ systems of the body. it is characterized by the absence (asplenia) or underdevelopment (hypoplasia) of the spleen,Ā heart malformations, and the abnormal arrangement of the internal organs of the chest and abdomen (situs inversus). symptoms vary greatly depending on the specific abnormalities present. the exact cause of ivemark syndrome is not known. last updated: 9/20/2011

MalaCards: Ivemark Syndrome, also known as bilateral right-sidedness sequence, is related to pancreatitis and right atrial isomerism. An important gene associated with Ivemark Syndrome is LEFTY2 (left-right determination factor 2), and among its related pathways are Signaling by NODAL and L1CAM interactions. The compounds ribonucleic acid and estrogen have been mentioned in the context of this disorder. Affiliated tissues include heart, spleen and kidney, and related mouse phenotype liver/biliary system.

Wikipedia:66 Asplenia with cardiovascular anomalies, also known as Ivemark syndrome and right atrial isomerism, is an... more...

Aliases & Classifications for Ivemark Syndrome

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44NIH Rare Diseases, 23GTR
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Cardiovascular diseases


Aliases & Descriptions:

ivemark syndrome 44
bilateral right-sidedness sequence 44 23
asplenia with cardiovascular anomalies 44
splenic agenesis syndrome 44
asplenia syndrome 44


Related Diseases for Ivemark Syndrome

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18GeneCards, 19GeneDecks
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Diseases related to Ivemark Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show all 23)
idRelated DiseaseScoreTop Affiliating Genes
1pancreatitis10.2
2right atrial isomerism10.2
3hiatus hernia10.2
4hepatitis c10.1
5polydactyly10.1
6situs inversus10.1
7vitiligo10.1
8arthritis10.1
9basal cell carcinoma10.1
10congenital heart disease10.1
11hepatitis10.1
12meningitis10.1
13pulmonary embolism10.1
14heterotaxy10.1
15asplenia, isolated congenital10.1
16atrioventricular septal defect 410.1
17atrioventricular septal defect 510.1
18renal dysplasia10.1
19thrombocytosis10.0
20placental abruption10.0
21lymphangiectasis10.0
22thromboembolism10.0
23congenital microgastria10.0

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



Diseases related to ivemark syndrome

Symptoms for Ivemark Syndrome

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Drugs & Therapeutics for Ivemark Syndrome

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Ivemark Syndrome

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23GTR
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Genetic tests related to Ivemark Syndrome:

id Genetic test Affiliating Genes
1 Bilateral Right-Sidedness Sequence23

Anatomical Context for Ivemark Syndrome

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34MalaCards
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MalaCards organs/tissues related to Ivemark Syndrome:

34
Heart, Spleen, Kidney, Lung, Appendix

Animal Models for Ivemark Syndrome or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Ivemark Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.5MUC1, LEFTY1, LEFTY2

Publications for Ivemark Syndrome

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53PubMed
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Articles related to Ivemark Syndrome:

(show all 25)
idTitleAuthorsYear
1
Hepatitis C-related mixed type vitiligo in a patient with Ivemark syndrome. (24433294)
2014
2
Acute pulmonary embolism revealing Ivemark syndrome in an adult. (23375428)
2013
3
Intestinal microvascular malformations and congenital asplenia in an adolescent possibly expanding the phenotype of Ivemark syndrome. (21946075)
2011
4
Early-onset basal cell carcinoma in a case of Ivemark syndrome. (20959271)
2010
5
Pregnancy and delivery of a patient with Ivemark syndrome (with polysplenia). (17555862)
2008
6
Pancreatic aplasia in a fetus with asplenia-cardiovascular defect-heterotaxy (Ivemark syndrome). (18496831)
2008
7
Ivemark syndrome in association with congenital septum transversum defect and pancreatic divisum. (16763342)
2006
8
Ivemark syndrome with agenesis of the corpus callosum: a case report with a review of the literature. (12424766)
2002
9
Streptococcus bovis meningitis in a neonate with Ivemark syndrome. (11874168)
2002
10
Spontaneous biliary perforation in a child with features of Ivemark syndrome. (10663854)
2000
11
Does Ser364Pro mutation of connexin 43 exist in Taiwanese patients with Ivemark syndrome? (11037645)
2000
12
Mutation analysis of human LEFTY A and LEFTY B genes in children with Ivemark syndrome. (11100524)
2000
13
Ivemark syndrome: asplenia with kidney collecting duct cysts and polysplenia with cerebellar cyst. (11105625)
2000
14
Ontogeny of renal dysplasia in Ivemark syndrome: light and immunohistochemical characterization. (10074963)
1999
15
Successful heart transplantation in a patient with Ivemark syndrome combined with situs inversus, single atrium and ventricle after total cavo-pulmonary connection. (9879878)
1998
16
Ivemark syndrome with asplenia in siblings. (9152295)
1997
17
Ivemark syndrome. A case with successful surgical intervention. (9264168)
1997
18
Ivemark syndrome: a case report. (9257479)
1997
19
Gouty arthritis in a patient with Ivemark syndrome. (8701391)
1996
20
The Ivemark syndrome: prenatal diagnosis of an uncommon cystic renal lesion with heterogeneous associations. (8580017)
1995
21
Ivemark syndrome in siblings. (7172476)
1982
22
Fetal cardiac abnormality and real-time ultrasound study:a case of Ivemark syndrome. (446014)
1979
23
Ivemark syndrome with aortic atresia. (4579213)
1973
24
CONGENITAL HEART DISEASE WITH THE IVEMARK SYNDROME AND ABSENCE OF THE INFERIOR VENA CAVA. (14275331)
1965
25
Internal hernias due to defects in the meso-appendix and mesentery of small bowel, and probable Ivemark syndrome: report of two cases. (13974693)
1963

Variations for Ivemark Syndrome

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Expression for genes affiliated with Ivemark Syndrome

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Ivemark Syndrome

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Pathways for genes affiliated with Ivemark Syndrome

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51PathCards, 56Reactome, 61Thomson Reuters, 58SinoBiological, 31KEGG, 39NCBI BioSystems Database
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Compounds for genes affiliated with Ivemark Syndrome

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46Novoseek, 25HMDB, 12DrugBank, 30IUPHAR, 62Tocris Bioscience
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Compounds related to Ivemark Syndrome according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1ribonucleic acid469.0MUC1, LEFTY2
2estrogen469.0MUC1, LEFTY2
3estradiol46 25 1210.9MUC1, LEFTY2
4progesterone46 30 62 25 1212.7MUC1, LEFTY2

GO Terms for genes affiliated with Ivemark Syndrome

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17Gene Ontology
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Biological processes related to Ivemark Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1transforming growth factor beta receptor signaling pathwayGO:0071799.3LEFTY1, LEFTY2
2cell growthGO:0160499.0LEFTY1, LEFTY2

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

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:0051259.3LEFTY1, LEFTY2
2transforming growth factor beta receptor bindingGO:0051609.2LEFTY1, LEFTY2
3growth factor activityGO:0080839.0LEFTY1, LEFTY2

Products for genes affiliated with Ivemark Syndrome

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Sources for Ivemark Syndrome

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet