Three-year Follow-up of Niemann-Pick Disease with Pulmonary Involvement: A Case Report
Journal of the Korean Radiological Society
; : 37-40, 2005.
Artigo
em Coreano
| WPRIM (Pacífico Ocidental)
| ID: wpr-27870
Biblioteca responsável:
WPRO
ABSTRACT
Niemann-Pick disease is a rare inherited metabolic storage disease that causes excessive intracellular storage of sphingomyelin in various organs. We present the pulmonary imaging findings with particular emphasis on the CT findings in a case of Niemann-Pick disease type B with pulmonary involvement. The chest radiograph showed fine reticulonodular opacities in both basal lung fields, and the high-resolution chest CT showed centrilobular nodular opacities and smooth thickening of the interlobar fissure and interlobular septum with a basal lung predominance. Coronal reformatted CT revealed a prominent interlobular septal thickening around the diaphragm. The follow-up high-resolution chest CT showed no significant interval changes over a 3-years period.
Texto completo:
Disponível
Base de dados:
WPRIM (Pacífico Ocidental)
Assunto principal:
Diafragma
/
Radiografia Torácica
/
Tomografia Computadorizada por Raios X
/
Doenças de Niemann-Pick
/
Seguimentos
/
Pulmão
Tipo de estudo:
Estudo observacional
/
Estudo prognóstico
Idioma:
Coreano
Revista:
Journal of the Korean Radiological Society
Ano de publicação:
2005
Tipo de documento:
Artigo