Increased vascular occlusion in patients with pseudoxanthoma elasticum.
Vasa
; 46(1): 47-52, 2017 01.
Article
en En
| MEDLINE
| ID: mdl-27927085
ABSTRACT
BACKGROUND:
Pseudoxanthoma elasticum (PXE) is an autosomal recessive inherited multisystem disorder of the connective tissue caused by a loss-of-function mutation of the ABCC6 gene. It can affect the cardiovascular system, presumably leading to a high prevalence of atherosclerosis. PATIENTS ANDMETHODS:
46 PXE patients and 18 controls underwent an angiological examination consisting of measurement of ankle-brachial index (ABI), strain-gauge arterial reserve (SGAR), arterial resting perfusion, pulse wave index (PWI), central pulse wave velocity, and ultrasound examination.RESULTS:
With an average age of 51.4 ± 12.4 years, 35/46 (76.1 %) of the PXE patients had atherosclerotic lesions, and 10 of them (28.6 %) had a chronic vascular occlusion of one or more peripheral vessels. 34/46 (73.9 %) had a pathologic ABI < 0.9, 15/42 (35.7 %) had a pathological SGAR < 10 mL/100 mL tissue/min, and 23/38 (60.5 %) had a pathological PWI > 180. The differences between the groups were statistically significant for ABI, arterial reserve, and PWI.CONCLUSIONS:
In PXE patients atherosclerosis was found with a much higher prevalence than expected. Moreover, they were at very high risk for total vessel occlusions.â©.Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Seudoxantoma Elástico
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Aterosclerosis
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Enfermedad Arterial Periférica
Tipo de estudio:
Diagnostic_studies
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Etiology_studies
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Observational_studies
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Prevalence_studies
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Risk_factors_studies
Límite:
Adult
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Female
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Humans
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Male
/
Middle aged
País/Región como asunto:
Europa
Idioma:
En
Año:
2017
Tipo del documento:
Article