title: Pulmonary function testing and pulmonary Langerhans cell histiocytosis.
abstract: 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.
authors.affiliation: Childhood Cancer Research Unit, Department of Woman and Child Health, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Cecilia
authors.initials: C
authors.lastname: Bernstrand
authors.forename: Kerstin
authors.initials: K
authors.lastname: Cederlund
authors.forename: Jan-Inge
authors.initials: JI
authors.lastname: Henter
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D003560:Cysts / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011656:Pulmonary Emphysema / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology; D012129:Respiratory Function Tests; D012307:Risk Factors; D012907:Smoking / Q000009:adverse effects; D018709:Statistics, Nonparametric; D014057:Tomography, X-Ray Computed