Síndrome de Marfan con disección aórtica asociada a tromboembolismo venoso e hiperhomocisteinemia / Marfan syndrome associated with aortic dissection, venous thromboembolism and hyperhomocysteinemia
Medicina (B.Aires)
; 72(6): 478-480, dic. 2012. ilus
Article
in Es
| LILACS
| ID: lil-662156
Responsible library:
AR1.2
RESUMEN
El síndrome de Marfan es un desorden genético infrecuente, cuyas manifestaciones clínicas afectan a los sistemas cardiovascular, ocular y músculo esquelético, la gravedad de las manifestaciones cardiovasculares son generalmente responsables de la mortalidad de estos pacientes. La enfermedad tromboembólica venosa está íntimamente relacionada con diversos factores de riesgo, congénitos, adquiridos, mixtos o desconocidos. La hiperhomocisteinemia es un factor de riesgo moderado para tromboembolismo venoso. Presentamos el caso de una mujer de 47 años de edad con síndrome de Marfan asociado a disección aórtica tipo A indolora, que simultáneamente padeció trombosis venosa de miembros inferior y superior izquierdos con embolia de pulmón. Al realizar el cribado de trombofilia se constató hiperhomocisteinemia. Con la terapéutica instituida evolucionó favorablemente.
ABSTRACT
Marfan syndrome is an infrequent genetic disorder of connective tissue whose clinical manifestations mainly affect the cardiovascular, ocular and musculoskeletal systems. Serious cardiovascular manifestations are generally the cause of mortality of Marfan patients. Thromboembolic venous disease is intimately related to different risk factors inherited, acquired, mixed or unknown; hyperhomocysteinemia is a moderate risk factor for venous thromboembolism. We present the case of a 47-year-old woman with Marfan syndrome associated to a painless type A aortic dissection, who simultaneously suffered venous thromboembolism of left upper and lower limbs with pulmonary embolism. Hyperhomocysteinemia was found through thrombofilia screening. The patient's condition has evolved favorably.
Key words
Full text:
1
Collection:
01-internacional
Database:
LILACS
Main subject:
Aortic Aneurysm, Thoracic
/
Hyperhomocysteinemia
/
Venous Thromboembolism
/
Aortic Dissection
/
Marfan Syndrome
Type of study:
Etiology_studies
Limits:
Female
/
Humans
Language:
Es
Journal:
Medicina (B.Aires)
Journal subject:
MEDICINA
Year:
2012
Type:
Article
Affiliation country:
Argentina