Preferred Name |
Primary lymphedema |
|
Synonyms |
|
|
Definitions |
Primary lymphedema is a lymphatic system malformation characterized by swelling of an extremity that can be associated with other lymphatic effusions, due to an underlying developmental anomaly of the lymphatic system (abnormal lymphoangiogenesis). It can be hereditary or not and be congenital or late onset. |
|
ID |
http://www.orpha.net/ORDO/Orphanet_77240 |
|
definition |
Primary lymphedema is a lymphatic system malformation characterized by swelling of an extremity that can be associated with other lymphatic effusions, due to an underlying developmental anomaly of the lymphatic system (abnormal lymphoangiogenesis). It can be hereditary or not and be congenital or late onset. |
|
definition_citation |
Orphanet |
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expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=77240 |
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has_age_of_onset |
All ages |
|
has_inheritance |
Autosomal recessive Autosomal dominant |
|
hasDbXref |
ICD-11:BD93.0 |
|
label |
Primary lymphedema |
|
notation |
ORPHA:77240 Category |
|
prefixIRI |
ORDO:Orphanet_77240 |
|
prefLabel |
Primary lymphedema |
|
present_in |
Europe AND has_point_prevalence_average_value : 16.7 AND has_point_prevalence_range : 1-5 / 10 000 |
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subClassOf |
http://www.orpha.net/ORDO/Orphanet_68346 http://www.orpha.net/ORDO/Orphanet_89826 http://www.orpha.net/ORDO/Orphanet_211255 |