Three-year Follow-up of Niemann-Pick Disease with Pulmonary Involvement: A Case Report
Journal of the Korean Radiological Society
; : 37-40, 2005.
Article
in Korean
| WPRIM (Western Pacific)
| ID: wpr-27870
Responsible library:
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.
Full text:
Available
Database:
WPRIM (Western Pacific)
Main subject:
Diaphragm
/
Radiography, Thoracic
/
Tomography, X-Ray Computed
/
Niemann-Pick Diseases
/
Follow-Up Studies
/
Lung
Type of study:
Observational study
/
Prognostic study
Language:
Korean
Journal:
Journal of the Korean Radiological Society
Year:
2005
Document type:
Article