Preferred Name |
Situs ambiguus |
|
Synonyms |
Incomplete situs inversus |
|
Definitions |
A rare, genetic, developmental defect during embryogenesis characterized by a partial mirror-image transposition of intra-thoracic and/or intra-abdominal organs across the left-right axis of the body. Intra-organ variations and other malformations, such as ciliary motricity anomalies (e.g. Kartagener syndrome), biliary atresia and cardiac defects, are frequently associated. Left (polysplenia syndrome) or right (asplenia syndrome) isomerism are usually observed. |
|
ID |
http://www.orpha.net/ORDO/Orphanet_157769 |
|
alternative_term |
Incomplete situs inversus Situs ambiguous Partial situs inversus |
|
definition |
A rare, genetic, developmental defect during embryogenesis characterized by a partial mirror-image transposition of intra-thoracic and/or intra-abdominal organs across the left-right axis of the body. Intra-organ variations and other malformations, such as ciliary motricity anomalies (e.g. Kartagener syndrome), biliary atresia and cardiac defects, are frequently associated. Left (polysplenia syndrome) or right (asplenia syndrome) isomerism are usually observed. |
|
definition_citation |
Orphanet |
|
expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=157769 |
|
hasDbXref |
ICD-10:Q89.3 MedDRA:10059119 UMLS:C0266642 |
|
label |
Situs ambiguus |
|
notation |
ORPHA:157769 |
|
part_of |
http://www.orpha.net/ORDO/Orphanet_271853 |
|
prefixIRI |
ORDO:Orphanet_157769 |
|
prefLabel |
Situs ambiguus |
|
treeView |
http://www.orpha.net/ORDO/Orphanet_271853 |
|
subClassOf |