RESUMEN
AIMS: To evaluate diagnostic accuracy of contrast echocardiography (CE) as compared with CT, for the screening of pulmonary arteriovenous malformations (PAVMs) in hereditary haemorrhagic telangiectasia (HHT); to evaluate the clinical significance of semi-quantitative analysis of a shunt on CE. METHODS AND RESULTS: A blinded prospective study was conducted in 190 consecutive subjects at risk of HHT who underwent screening for PAVMs, including clinical evaluation, pulse oximetry, standard and CE, and chest multirow CT without contrast medium. A semi-quantitative analysis of the shunt size was performed according to the contrast echo opacification of the left-sided chambers: Grade 0, no bubbles; 1, occasional filling with <20 bubbles; 2, moderate filling; 3, complete opacification. The first 100 patients were compared with 100 controls. A total of 119 (63%) patients had positive CE (32.2% Grade 1, 13.1% Grade 2, 11% Grade 3, 6.3% with patent foramen ovale). The overall diagnostic performance of CE was sensitivity 1.00, specificity 0.49, positive predictive value (PPV) 0.32, negative predictive value (NPV) 1.00. The PPV for the different grades was 0.00 for Grade 1, 0.56 for Grade 2, 1.00 for Grade 3; the NPV of Grade 0 was 1.00. A significant correlation was found between the CE grading and the number of PAVM, and complications (P < 0.0001). CONCLUSION: CE is an extremely sensitive procedure for the detection of PAVMs with substantial clinical impact.
Asunto(s)
Malformaciones Arteriovenosas/diagnóstico por imagen , Ecocardiografía/métodos , Arteria Pulmonar/anomalías , Venas Pulmonares/anomalías , Telangiectasia Hemorrágica Hereditaria/complicaciones , Tomografía Computarizada por Rayos X/métodos , Adolescente , Adulto , Anciano , Malformaciones Arteriovenosas/diagnóstico , Malformaciones Arteriovenosas/etiología , Estudios de Casos y Controles , Distribución de Chi-Cuadrado , Niño , Preescolar , Embolización Terapéutica , Femenino , Predisposición Genética a la Enfermedad , Humanos , Masculino , Microburbujas , Persona de Mediana Edad , Oximetría , Estudios Prospectivos , Arteria Pulmonar/diagnóstico por imagen , Venas Pulmonares/diagnóstico por imagen , Sensibilidad y Especificidad , Método Simple Ciego , Adulto JovenRESUMEN
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Asunto(s)
Humanos , Masculino , Adulto , Factor VIIa/administración & dosificación , Heridas Penetrantes/cirugía , Hemorragia/tratamiento farmacológico , Lesión Renal Aguda/complicacionesRESUMEN
Follicular dendritic cell tumour/sarcoma is a rare tumour involving lymph-node or extra-lymph-node sites; review of the literature reveals very few cases of follicular dendritic cell tumour, probably since, in the past, the disease has often been mistaken for other neoplasms: low differentiated carcinomas and fusate cell carcinomas, sarcomas, melanomas, thymic neoplasms, Castle carcinoma and other dendritic cell tumours (especially interdigital cell tumour/sarcoma). In the case described here, attention is focused on the diagnostic difficulties and on the therapeutic profile, comparing data with those reported in the international literature.