MCID: IVM001
MIFTS: 34

Ivemark Syndrome malady

Rare diseases, Cardiovascular diseases categories
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Summaries for Ivemark Syndrome

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NIH Rare Diseases:42 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, 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 based summary: 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 L1CAM interactions and Ca-dependent events. 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:65 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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Ivemark Syndrome, Aliases & Descriptions:

Name: Ivemark Syndrome 42
Bilateral Right-Sidedness Sequence 42 22
Asplenia with Cardiovascular Anomalies 42
 
Splenic Agenesis Syndrome 42
Asplenia Syndrome 42


Classifications:

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


Related Diseases for Ivemark Syndrome

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

(show all 24)
idRelated DiseaseScoreTop Affiliating Genes
1pancreatitis10.2
2right atrial isomerism10.2
3hiatus hernia10.2
4arthritis10.1
5hepatitis10.1
6vitiligo10.1
7hepatitis c10.1
8polydactyly10.1
9situs inversus10.1
10basal cell carcinoma10.1
11congenital heart disease10.1
12meningitis10.1
13pulmonary embolism10.1
14agenesis of the corpus callosum10.1
15heterotaxy10.1
16asplenia, isolated congenital10.1
17atrioventricular septal defect 410.1
18atrioventricular septal defect 510.1
19renal dysplasia10.1
20thrombocytosis10.0
21placental abruption10.0
22lymphangiectasis10.0
23thromboembolism10.0
24congenital 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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Drug clinical trials:

Search ClinicalTrials for Ivemark Syndrome

Search NIH Clinical Center for Ivemark Syndrome

Genetic Tests for Ivemark Syndrome

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Genetic tests related to Ivemark Syndrome:

id Genetic test Affiliating Genes
1 Bilateral Right-Sidedness Sequence22

Anatomical Context for Ivemark Syndrome

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MalaCards organs/tissues related to Ivemark Syndrome:

32
Heart, Spleen, Kidney, Lung, Appendix

Animal Models for Ivemark Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Ivemark Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.5MUC1, LEFTY1, LEFTY2

Publications for Ivemark Syndrome

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Articles related to Ivemark Syndrome:

(show all 26)
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
Familial occurrence of Ivemark syndrome with splenic hypoplasia and asplenia in sibs. (4774542)
1973
24
Ivemark syndrome with aortic atresia. (4579213)
1973
25
CONGENITAL HEART DISEASE WITH THE IVEMARK SYNDROME AND ABSENCE OF THE INFERIOR VENA CAVA. (14275331)
1965
26
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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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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Compounds for genes affiliated with Ivemark Syndrome

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Sources:
44Novoseek, 24HMDB, 11DrugBank, 28IUPHAR, 61Tocris Bioscience
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Compounds related to Ivemark Syndrome according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1ribonucleic acid449.0MUC1, LEFTY2
2estrogen449.0MUC1, LEFTY2
3estradiol44 24 1110.9MUC1, LEFTY2
4progesterone44 28 61 24 1112.7MUC1, LEFTY2

GO Terms for genes affiliated with Ivemark Syndrome

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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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  • Antibodies
  • Proteins
  • Lysates

Sources for Ivemark 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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet