J Korean Radiol Soc.  2005 Jan;52(1):37-40. 10.3348/jkrs.2005.52.1.37.

Three-year Follow-up of Niemann-Pick Disease with Pulmonary Involvement: A Case Report

• Lee CW
• Goo HW

• KMID: 1834217
• DOI: http://doi.org/10.3348/jkrs.2005.52.1.37

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.

Keyword

Children, respiratory system; Computed tomography (CT), high-resolution; Lung, CT; Lung, interstitial disease; Lung, radiography

MeSH Terms

Diaphragm
Follow-Up Studies*
Lung
Niemann-Pick Diseases*
Radiography, Thoracic
Tomography, X-Ray Computed