SIV
MCID: STS002
MIFTS: 56

Situs Inversus (SIV) malady

Summaries for Situs Inversus

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42NIH Rare Diseases, 63Wikipedia, 46OMIM, 32MalaCards
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NIH Rare Diseases:42 Situs inversus is a condition in which the internal organs of the abdomen (stomach) and thorax (chest) lie in mirror image of their normal body position. it can occur alone, without any other anomalies, or it can be  a part of a syndrome with various other defects. situs inversus has been inherited in different ways in different families. autosomal dominant, autosomal recessive, and x-linked inheritance have been reported. treatment, when needed, is symptomatic and supportive. last updated: 7/19/2010

MalaCards: Situs Inversus, also known as situs inversus viscerum, is related to primary ciliary dyskinesia and double outlet right ventricle. An important gene associated with Situs Inversus is DNAH11 (dynein, axonemal, heavy chain 11), and among its related pathways are Signaling by NODAL and Transcriptional Regulatory Network in Embryonic Stem Cell. Affiliated tissues include liver, heart and lung, and related mouse phenotypes are digestive/alimentary and respiratory system.

Wikipedia:63 Situs inversus (also called situs transversus or oppositus) is a congenital condition in which the major... more...

Description from OMIM:46 270100

Aliases & Classifications for Situs Inversus

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8Disease Ontology, 42NIH Rare Diseases, 22GTR, 10DISEASES, 44Novoseek, 39NCIt, 56SNOMED-CT, 46OMIM, 27ICD9CM, 34MeSH, 60UMLS
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Aliases & Descriptions:

situs inversus 8 42 22 10 44
situs inversus viscerum 8 42
complete transposition 8
laterality sequence 8
siv 42


External Ids:

Disease Ontology8 DOID:758
NCIt39 C87121
OMIM46 270100
ICD9CM27 759.3
MeSH34 D012857

Related Diseases for Situs Inversus

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

(show top 50)    (show all 242)
idRelated DiseaseScoreTop Affiliating Genes
1primary ciliary dyskinesia30.7TCTE3, DNAH5, DNAH11, DNAI2, DNAI1, FOXJ1
2double outlet right ventricle30.4GDF1, CFC1
3ventricular septal defect30.4CFC1, GDF1
4bronchiectasis30.3DNAI1
5tetralogy of fallot30.3GDF1
6hydrocephalus30.3RFX4
7polycystic kidney disease30.3INVS, NPHP3
8kartagener syndrome30.2DNAI1, DNAH5
9cystic kidney30.2NPHP3, INVS
10congenital heart defect30.0ZIC3
11male infertility30.0DNAH5
12dextrocardia11.1
13duodenitis10.6
14dextrocardia with situs inversus10.6
15pancreatitis10.6
16myocardial infarction10.5
17duodenal obstruction10.5
18in situ carcinoma10.5
19biliary atresia10.4
20polydactyly10.4
21appendicitis10.4
22klippel-feil syndrome10.4
23esophagitis10.4
24hepatitis10.4
25obesity10.4
26swine influenza10.4
27hepatocellular carcinoma10.4
28chondrodysplasia situs inversus imperforate anus polydactyly10.4
29bardet-biedl syndrome10.3
30adenocarcinoma10.3
31colon cancer10.3
32kidney disease10.3
33isolated levocardia10.3
34jeune syndrome situs inversus10.3
35ciliary dyskinesia, primary, 3, with or without situs inversus10.3
36ciliary dyskinesia, primary, 7, with or without situs inversus10.3
37ciliary dyskinesia, primary, 1, with or without situs inversus10.3
38ciliary dyskinesia, primary, 9, with or without situs inversus10.3
39influenza10.3
40vaccinia10.3
41coronary artery disease10.3
42cerebritis10.3
43esophageal atresia10.3
44infertility10.3
45adult syndrome10.3
46adenocarcinoma in situ10.3
47cholelithiasis10.3
48hepatitis a10.3
49acute myocardial infarction10.3
50esophageal cancer10.3

Graphical network of the top 20 diseases related to Situs Inversus:



Diseases related to situs inversus

Clinical Features for Situs Inversus

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46OMIM
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Clinical features from OMIM:

270100

Drugs & Therapeutics for Situs Inversus

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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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Search CenterWatch for Situs Inversus

Genetic Tests for Situs Inversus

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22GTR
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Genetic tests related to Situs Inversus:

id Genetic test Affiliating Genes
1 Situs Inversus22

Anatomical Context for Situs Inversus

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32MalaCards
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MalaCards organs/tissues related to Situs Inversus:

32
Liver, Heart, Lung, Kidney, Pancreas, Colon, Spleen, Eye, Lymph node, Thyroid, Pituitary, Brain, Tongue, Salivary gland, Atrioventricular node

Animal Models for Situs Inversus or affiliated genes

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36MGI
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Publications for Situs Inversus

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50PubMed
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Articles related to Situs Inversus:

(show top 50)    (show all 619)
idTitleAuthorsYear
1
A patient with situs inversus totalis and pancreatic head cancer. (23816700)
2013
2
Monocular elevation deficiency with associated dextrocardia and situs inversus. (24227973)
2013
3
Laparoscopic cholecystectomy in situs inversus totalis: a case report. (24382230)
2013
4
Successful radiofrequency catheter ablation of atrioventricular nodal reentrant tachycardia in a patient with dextrocardia and situs inversus. (22955686)
2013
5
Dextrocardia with situs inversus associated with non-compaction cardiomyopathy. (24030083)
2013
6
Partial sternotomy coronary surgery with triple-vessel disease in dextrocardia and situs inversus totalis. (23559364)
2013
7
Laparoscopic cholecystectomy in patients with situs inversus totalis: literature review of two patients. (23482252)
2012
8
Common bile duct adenocarcinoma in a patient with situs inversus totalis: report of a rare case. (23234596)
2012
9
Hepatic resection using a liver-hanging maneuver and Glissonean pedicle transection for hepatocellular carcinoma in a patient with situs inversus totalis: report of a case. (22391979)
2012
10
A rare case of situs inversus with dextrocardia, lutembacher syndrome, and pericardial effusion. (22567197)
2011
11
Situs inversus totalis. (22128729)
2011
12
Situs inversus totalis associated with subaortic stenosis, restrictive ventricular septal defect, and tricuspid dysplasia in an adult dog. (22547847)
2011
13
Renal transplantation in a patient with Bardet-Biedl syndrome, situs inversus totalis and bifid epiglottis: Anesthetic management. (24391427)
2011
14
Anatomical tricuspid valve replacement in a patient with corrected transposition of the great arteries and situs inversus with 90A^ clockwise rotation of the heart through right thoracotomy. (20796091)
2010
15
Classical orthotopic liver transplantation from a donor with situs inversus totalis using slight rotation of the liver graft. (20529596)
2010
16
ERCP in complete situs inversus viscerum using a "mirror image" technique. (21113889)
2010
17
Laparoscopic cholecystectomy in situs inversus-our experience of 6 cases. (21966139)
2010
18
Congenital cyanotic heart disease with situs inversus totalis: an autopsy case report. (19465810)
2009
19
Successful radiofrequency ablation of a right posteroseptal accessory pathway through an anomalous inferior vena cava and azygos continuation in a patient with incomplete situs inversus. (19387965)
2009
20
Selective lung separation with Fogarty catheter guided by an adult fiberoptic bronchoscope in a paediatric patient with situs inversus. (19136763)
2009
21
Delayed small bowel perforation due to blunt abdominal trauma and periappendicitis in a patient with situs inversus totalis: a report of a case. (19499688)
2009
22
Anatomically left-sided septal slow pathway ablation in dextrocardia and situs inversus totalis. (18556684)
2008
23
Paralysis in the left phrenic nerve after living-donor liver transplantation for biliary atresia with situs inversus. (18975275)
2008
24
Loss of nephrocystin-3 function can cause embryonic lethality, Meckel-Gruber-like syndrome, situs inversus, and renal-hepatic- pancreatic dysplasia. (18371931)
2008
25
Catheter ablation of atrioventricular nodal reentrant tachycardia in a patient with complete situs inversus, atrial septal defect and "inverse" persistent left superior vena cava. (17045667)
2007
26
The relation between organ anatomy and pre-existent vertebral rotation in the normal spine: magnetic resonance imaging study in humans with situs inversus totalis. (17471096)
2007
27
Successful superselective embolization of posttraumatic pseudoaneurysm of replaced hepatic artery in a child with situs inversus totalis. (17825182)
2007
28
Successful ablation of atrioventricular node reentry tachycardia in a patient with crisscross heart and situs inversus levocardia. (17347869)
2006
29
Renal hypoplasia and situs inversus totalis. (15877680)
2005
30
Adult complex spinal dysraphism with situs inversus totalis: a rare association and review. (16227901)
2005
31
Laparoscopic appendectomy in a female patient with situs inversus: case report and literature review. (15119665)
2004
32
Accessory mitral valve tissue associated with situs inversus in an elderly patient. (12829096)
2003
33
Myocardial revascularization for situs inversus totalis and dextrocardia. (17670102)
2003
34
A case of complete situs inversus. (12712813)
2003
35
Coronary artery bypass grafting in dextrocardia with situs inversus totalis. (14735049)
2003
36
Transcatheter closure of perimembranous ventricular septal defect in a patient with situs inversus and dextrocardia. (14560936)
2003
37
Mustard procedure in atrial situs inversus: three-dimensional ablation of intra-atrial reentry tachycardia on a surgically created inversus cavo-tricuspid isthmus. (12116804)
2002
38
TIPS creation in a patient with situs inversus totalis. (12132029)
2002
39
Situs inversus and coronary artery disease. (12079972)
2002
40
Anomalies in left-right axis cause situs inversus and infertility in men. (12709277)
2002
41
Heart block in dextrocardia and situs inversus: a case report. (11686917)
2001
42
Laparoscopic cholecystectomy in situs inversus totalis: is it safe? (11569513)
2001
43
Dextrocardia with and without situs viscerum inversus in two sibs. (3425603)
1987
44
Armchair clue to diagnosis of mirror image dextrocardia with situs inversus in males. (6511751)
1984
45
Dextrocardia with total situs inversus. Radionuclide imaging and ultrasonography of liver and spleen. (6928549)
1980
46
Pericarditis complicating congenital dextrocardia with situs inversus. (5494000)
1970
47
A CASE OF INTERVENTRICULAR SEPTAL DEFECT WITH DEXTROCARDIA AND SITUS INVERSUS TREATED BY SURGERY. (14045014)
1963
48
Partial situs inversus with levocardia. An unusual combination of anomalies. (14483095)
1962
49
Dextrocardia, situs inversus, and twinning. (13260637)
1955
50
Congenital dextrocardia with situs inversus complicated by hypertensive heart disease; reversal of hypertensive changes following thoracolumbar sympathectomy. (18119902)
1949

Genetic Variations for Situs Inversus

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Expression for genes affiliated with Situs Inversus

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

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Pathways for genes affiliated with Situs Inversus

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53Reactome, 51QIAGEN, 12EMD Millipore
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Pathways related to Situs Inversus according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.4CFC1, GDF1
210.3ZIC3, HAND1, RFX4
3
Hide members
10.3TCTE3, DNAH5, DNAH11, DNAI2, DNAI1

Compounds for genes affiliated with Situs Inversus

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GO Terms for genes affiliated with Situs Inversus

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16Gene Ontology
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Cellular components related to Situs Inversus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1dynein complexGO:03028610.4TCTE3, DNAI1
2cilium axonemeGO:03508510.3DNAH5, DNAH11, DNAI2, DNAI1, CCDC103
3axonemal dynein complexGO:00585810.3DNAI2, DNAH11, DNAH5
4microtubuleGO:00587410.2DNAI1, DNAI2, DNAH11, DNAH5, INVS, TCTE3
5ciliumGO:00592910.0INVS, DNAI1, NPHP3

Biological processes related to Situs Inversus according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1determination of pancreatic left/right asymmetryGO:03546910.5ZIC3, NPHP3
2determination of digestive tract left/right asymmetryGO:07190710.4CCDC103, ZIC3
3determination of left/right symmetryGO:00736810.4ZIC3, NPHP3, DNAH11, CFC1
4cell projection organizationGO:03003010.4DNAI1, CCDC103
5determination of liver left/right asymmetryGO:07191010.4NPHP3, ZIC3
6heart loopingGO:00194710.4HAND1, FOXJ1, NPHP3, CCDC103, ZIC3
7epithelial cilium movement involved in determination of left/right asymmetryGO:06028710.3CCDC103, NPHP3
8ciliary or bacterial-type flagellar motilityGO:00153910.1DNAH5, DNAH11

Molecular functions related to Situs Inversus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1microtubule motor activityGO:00377710.4DNAH5, DNAH11, DNAI2

Products for genes affiliated with Situs Inversus

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Sources for Situs Inversus

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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