Case report. Pulmonary involvement in Niemann-Pick disease subtype B: CT findings

J Comput Assist Tomogr. 1996 Nov-Dec;20(6):990-2. doi: 10.1097/00004728-199611000-00023.

Abstract

We present a case of Niemann-Pick disease subtype B in which the excessive storage of sphingomyelin within lung interstitium and alveoli produced an infiltrative lung disease demonstrated on high-resolution CT (HRCT). HRCT findings were correlated to pathological features.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Biopsy
  • Female
  • Humans
  • Lung / diagnostic imaging
  • Lung / pathology
  • Lung Diseases / diagnostic imaging*
  • Lung Diseases / etiology
  • Lung Diseases / pathology
  • Niemann-Pick Diseases / complications
  • Niemann-Pick Diseases / diagnostic imaging*
  • Niemann-Pick Diseases / pathology
  • Tomography, X-Ray Computed* / methods