Síndrome de Bland-White-Garland / Bland-White-Garland syndrome
Rev. cuba. pediatr
;
88(2): 205-213, abr.-jun. 2016.
Article
in Spanish
| LILACS, CUMED
| ID: lil-783773
RESUMEN
El síndrome de Bland-White-Garland constituye un defecto congénito poco frecuente, pero grave; el 90 % de los pacientes mueren en el primer año de vida si no reciben tratamiento. Desde el período previo a la ecocardiografía hasta la actualidad se describe en los textos una prevalencia de 1 por 300 000 nacidos vivos. Su forma de presentación clínica es variada, y a pesar de ser una anomalía de origen congénito, no es exclusiva de las edades pediátricas. Existen niños que padecen la variedad adulta del síndrome, sujetos enmarcados bajo la fachada clínica de otras entidades nosológicas, o, incluso, que cursan de manera silente, por lo que constituye un grave problema de salud. Su tratamiento es quirúrgico, con supervivencia y pronóstico apropiados si se diagnostica en etapa precoz.
ABSTRACT
Bland-White-Garland syndrome is a rare but serious congenital defect since 90% of patients may die in the first year of life if they are not duly treated. From the period prior to the emergence of echocardiography up to the present day, the prevalence described in literature is 1 per 300 000 live births. Its clinical presentation is varied, and in spite of the fact that it is an anomaly of congenital origin, it does not only occur in pediatric ages. There are children suffering the adult variety of the syndrome; others who are masked under the clinical umbrella of other diseases or even children who are asymptomatic, so this syndrome can represent a serious health problem. Surgical treatment is the choice, with adequate prognosis and survival if diagnosis is made at early phase.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Early Diagnosis
/
Bland White Garland Syndrome
Type of study:
Diagnostic study
/
Prognostic study
/
Risk factors
/
Screening study
Limits:
Humans
Language:
Spanish
Journal:
Rev. cuba. pediatr
Journal subject:
Pediatrics
Year:
2016
Type:
Article
Affiliation country:
Cuba
Institution/Affiliation country:
Cardiocentro Pediátrico William Soler/CU
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