Transient left bundle branch block and left ventricular dysfunction in a patient with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome.
Cardiol Young
; 29(3): 435-438, 2019 Mar.
Article
en En
| MEDLINE
| ID: mdl-30681047
ABSTRACT
The NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome is a rare novel autoinflammatory disorder. Cardiac involvement has not been previously reported. We present a 12-year-old girl with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome who was diagnosed with severely impaired left ventricular function and complete left bundle branch block during an exacerbation of the disease. Cardiac dysfunction proved to be rapidly reversible after initiation of high-dose methylprednisolone.
Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Artritis Juvenil
/
Bloqueo de Rama
/
Disfunción Ventricular Izquierda
/
Disqueratosis Congénita
/
Proteínas Adaptadoras Transductoras de Señales
/
Proteínas Reguladoras de la Apoptosis
/
Enfermedades Autoinflamatorias Hereditarias
/
Mutación
Tipo de estudio:
Diagnostic_studies
/
Risk_factors_studies
Límite:
Child
/
Female
/
Humans
Idioma:
En
Revista:
Cardiol Young
Asunto de la revista:
ANGIOLOGIA
/
CARDIOLOGIA
/
PEDIATRIA
Año:
2019
Tipo del documento:
Article
País de afiliación:
Países Bajos