Pulmonary function testing and pulmonary Langerhans cell histiocytosis

Pediatr Blood Cancer. 2007 Sep;49(3):323-8. doi: 10.1002/pbc.20707.


In a long-term single-center follow-up (median 16-years), we studied high-resolution computed tomography (HRCT) and pulmonary function testing (PFT) in pulmonary LCH. Diffusing capacity corrected for alveolar volume (K(CO)) and total lung capacity (TLC) were significantly decreased (P=0.016 and P=0.030, respectively) in patients with extensive HRCT abnormalities. Patients with late stage disease on HRCT had increased forced expiratory volume (FEV1.0)(P=0.037) and vital capacity (VC)(P=0.036). Disease monitoring is important in pulmonary LCH, and since PFT with diffusing capacity provides a measurement of the current lung function, it may be a valuable tool in monitoring pulmonary LCH, and a good complement to imaging.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Child
  • Child, Preschool
  • Cysts / diagnostic imaging
  • Cysts / etiology
  • Cysts / physiopathology
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Histiocytosis, Langerhans-Cell / complications
  • Histiocytosis, Langerhans-Cell / diagnostic imaging*
  • Histiocytosis, Langerhans-Cell / physiopathology*
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Pulmonary Emphysema / diagnostic imaging
  • Pulmonary Emphysema / etiology
  • Pulmonary Emphysema / physiopathology
  • Respiratory Function Tests*
  • Risk Factors
  • Smoking / adverse effects
  • Statistics, Nonparametric
  • Tomography, X-Ray Computed*