Authors
Grazia Crescimanno, Oreste Marrone, Marta Lazzeri, Paolo Innocente Banfi, Agata Lax, Elena Compalati, Rosario Di Marco, Sabrina Planiscig, Paola Confalonieri, Fabrizio Seidita, Filippo Brighina, Marco Confalonieri
Published in
Orphanet journal of rare diseases. Volume 21. Issue 1. Aug 07, 2026. Epub Aug 07, 2026.
Abstract
In late-onset Pompe disease (LOPD), muscle weakness causes restrictive lung impairment. In murine models, glycogen deposition in lung parenchyma suggests additional causes for restriction that remain poorly understood. We investigated whether oscillometry detects respiratory abnormalities not identified by spirometry and evaluated changes after air-stacking manoeuvres.
We prospectively evaluated 16 adults with LOPD from three Italian centres. Alongside spirometry, oscillometry (at 5, 11, and 19 Hz) was performed to assess resistance (R) and reactance (X) across the breathing cycle and by respiratory phase. Measurements were repeated 5 min after an air-stacking manoeuvre.
Participants (7 men, 9 women; 51.6 ± 12.5 years) had moderate-to-severe restriction (FVC 53.3 ± 19.6% predicted) with preserved FEV1/FVC. Two oscillometric patterns emerged. The first one (seven patients) was characterised by an abnormal increase in expiratory resistance within breaths ([R5exp - R5insp] > 0.70 cmH2O·s/L), associated or not with abnormal whole-breath R and intrabreath X changes. The second pattern (nine patients) consisted of normal R and X changes in the breathing cycle. Air-stacking increased FVC (53.3 ± 19.6 to 71.3 ± 19.2% predicted, p < 0.001) without normalising reactance.
In LOPD, oscillometry detects distinct mechanical patterns not identified by spirometry. Air-stacking increases lung volumes without improving respiratory mechanics.
PMID:
42568093
Bibliographic data and abstract were imported from PubMed on 08 Aug 2026.
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