Three-year Follow-up of Niemann-Pick Disease with Pulmonary Involvement: A Case Report.
10.3348/jkrs.2005.52.1.37
- Author:
Choong Wook LEE
1
;
Hyun Woo GOO
Author Information
1. Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Korea. hwgoo@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Children, respiratory system;
Computed tomography (CT), high-resolution;
Lung, CT;
Lung, interstitial disease;
Lung, radiography
- MeSH:
Diaphragm;
Follow-Up Studies*;
Lung;
Niemann-Pick Diseases*;
Radiography, Thoracic;
Tomography, X-Ray Computed
- From:Journal of the Korean Radiological Society
2005;52(1):37-40
- CountryRepublic of Korea
- Language:Korean
-
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.
