RESUMO
Niemann-Pick disease is a rare inherited autosomal recessive disorder, currently classified into six subtypes and characterized by the intracellular accumulation of sphingomyelin in the liver, spleen, lungs, bone marrow or brain. The main pulmonary abnormalities described in high-resolution computed tomography (HRCT) of the chest consist of thickening of the interlobular septa and ground-glass opacities. This case report describes a patient with subtype B Niemann-Pick disease characterized by cysts and ground-glass opacities that were detected on HRCT of the chest.
Assuntos
Cistos/etiologia , Pneumopatias/etiologia , Doença de Niemann-Pick Tipo B/complicações , Adulto , Cistos/diagnóstico por imagem , Cistos/patologia , Humanos , Pneumopatias/diagnóstico por imagem , Pneumopatias/patologia , Masculino , Doença de Niemann-Pick Tipo B/diagnóstico por imagem , Doença de Niemann-Pick Tipo B/patologia , Tomografia Computadorizada por Raios XRESUMO
The aurora sign, a sonographic sign found on the sagittal and transverse view, refers to multiple bands of ring-down artifacts posterior to the right hemidiaphragm. Parenchymal lung disease should be suspected when this is present. We report a case of type B Niemann-Pick disease with pulmonary involvement and the aurora sign on abdominal sonography. High-resolution CT of the chest showed corresponding thickened interlobular septa.