Ascending aortic aneurysm and histopathology in Alport syndrome: a case report.
BMC Nephrol
; 24(1): 300, 2023 10 12.
Article
in En
| MEDLINE
| ID: mdl-37828432
ABSTRACT
BACKGROUND:
Alport syndrome (AS) is caused by mutations in type IV collagen genes that typically target and compromise the integrity of basement membranes in kidney, ocular, and sensorineural cochlear tissues. Type IV and V collagens are also integral components of arterial walls, and whereas collagenopathies including AS are implicated in aortic disease, the incidence of aortic aneurysm in AS is unknown probably because of underreporting. Consequently, AS is not presently considered an independent risk factor for aortic aneurysm and more detailed case studies including histological evidence of basement membrane abnormalities are needed to determine such a possible linkage. CASE PRESENTATION Here, we present unique histopathological findings of an ascending aortic aneurysm collected at the time of surgery from an AS patient wherein hypertension was the only other known risk factor.CONCLUSIONS:
The studies reveal classical histological features of aortic aneurysm, including atheroma, lymphocytic infiltration, elastin disruption, and myxoid degeneration with probable AS association.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Aortic Aneurysm
/
Aneurysm, Ascending Aorta
/
Nephritis, Hereditary
Limits:
Humans
Language:
En
Journal:
BMC Nephrol
Journal subject:
NEFROLOGIA
Year:
2023
Type:
Article
Affiliation country:
United States