Cardiovascular pathologies in mucopolysaccharidosis type VII (Sly Syndrome).
Cardiovasc Pathol
; 24(5): 322-6, 2015.
Article
en En
| MEDLINE
| ID: mdl-26141114
ABSTRACT
We present the cardiac findings from the autopsy of a 28-year-old male with mucopolysaccharidosis VII (MPS VII), also known as Sly Syndrome, whose diagnosis was confirmed by biochemical testing. The patient died a sudden cardiac death. Autopsy showed thickened and stenotic aortic valve leaflets as well as marked concentric intimal thickening of the aorta and muscular arteries. There was left ventricular hypertrophy as well as mild papillary muscle thickening and fusion. Increased colloid iron staining was seen in the small- and medium-sized arteries of the heart and at the intercalated discs. We discuss the patient's premortem echocardiographic and electrocardiographic studies. In addition, we discuss the pathogenesis of MPS VII and review previous literature on its anatomic and pathologic features.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Aorta
/
Mucopolisacaridosis VII
/
Válvulas Cardíacas
/
Miocardio
Tipo de estudio:
Etiology_studies
Límite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
Cardiovasc Pathol
Asunto de la revista:
ANGIOLOGIA
/
CARDIOLOGIA
/
PATOLOGIA
Año:
2015
Tipo del documento:
Article
País de afiliación:
Estados Unidos