Preferred Name | Amniotic band syndrome | |
Synonyms |
Amniotic band sequence Congenital constriction ring syndrome Constriction band syndrome Streeter dysplasia Congenital ring constrictions ABS |
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Definitions |
Constriction rings syndrome is a congenital limb malformation disorder with an extremely variable clinical presentation characterized by the presence of partial to complete, congenital, fibrous, circumferential, constriction bands/rings on any part of the body, although a particular predilection for the upper or lower extremities is seen. Phenotypes range from only a mild skin indentation to complete amputation of parts of the fetus (e.g. digits, distal limb). Compression from the rings may lead to edema, skeletal anomalies (e.g. fractures, foot deformities) and, infrequently, neural compromise. |
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ID |
http://www.orpha.net/ORDO/Orphanet_295000 |
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alternative_term |
Amniotic band sequence Congenital constriction ring syndrome Constriction band syndrome Streeter dysplasia Congenital ring constrictions ABS |
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definition |
Constriction rings syndrome is a congenital limb malformation disorder with an extremely variable clinical presentation characterized by the presence of partial to complete, congenital, fibrous, circumferential, constriction bands/rings on any part of the body, although a particular predilection for the upper or lower extremities is seen. Phenotypes range from only a mild skin indentation to complete amputation of parts of the fetus (e.g. digits, distal limb). Compression from the rings may lead to edema, skeletal anomalies (e.g. fractures, foot deformities) and, infrequently, neural compromise. |
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definition_citation |
Orphanet |
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expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=295000 |
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hasDbXref |
MeSH:D000652 ICD-10:Q79.8 UMLS:C0002636 ICD-11:LD26.5 MedDRA:10064100 OMIM:217100 |
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label |
Amniotic band syndrome |
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notation |
ORPHA:295000 |
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part_of | ||
prefixIRI |
ORDO:Orphanet_295000 |
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prefLabel |
Amniotic band syndrome |
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treeView | ||
subClassOf |