Observational studyOrphanet journal of rare diseases2026
Expiratory phase lung mechanics in late-onset Pompe disease: a multicenter study using oscillometry to identify specific breathing abnormalities.
Observational study in Orphanet journal of rare diseases, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
0 citing papers in PubMed.
No citing paper in PubMed yet.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
12 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionIn 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.
methodsWe 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.
resultsParticipants (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 cmH
conclusionIn LOPD, oscillometry detects distinct mechanical patterns not identified by spirometry. Air-stacking increases lung volumes without improving respiratory mechanics.
Indexed as
Identifiers
What Socratic holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.