Evidence map›Paper›PMID 35147730›Full record

ArticleJournal of neurology2022

Long-term follow-up of respiratory function in facioscapulohumeral muscular dystrophy.

Sjan Teeselink, Sanne C C Vincenten, Nicol C Voermans, Jan T Groothuis, Jonne Doorduin, Peter J Wijkstra, Corinne G C Horlings, Baziel G M van Engelen, Karlien Mul

Open access · hybridAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Journal of neurology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.

0numbers the graph read from it
0cells of the map it votes in
6citing papers in PubMed
1.0field-weighted citation impact, top 28% of its field
1 · What the graph read from it

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.

2 · The registry

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.

3 · Its place in the literature

Who cites it

6 citing papers in PubMed, 13 citations in OpenAlex.

  1. Article
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  3. Observational
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  6. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors at 3 institutions in 2 countries.

Sjan TeeselinkDepartment of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Reinier Postlaan 4 (910), PO Box 9101, 6500 HB, Nijmegen, The Netherlands. sjan.teeselink@radboudumc.nl.ORCID http://orcid.org/0000-0003-1023-2182
Sanne C C VincentenDepartment of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Reinier Postlaan 4 (910), PO Box 9101, 6500 HB, Nijmegen, The Netherlands.ORCID http://orcid.org/0000-0002-0412-0670
Nicol C VoermansDepartment of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Reinier Postlaan 4 (910), PO Box 9101, 6500 HB, Nijmegen, The Netherlands.ORCID http://orcid.org/0000-0002-5837-7295
Jan T GroothuisDepartment of Rehabilitation, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands.ORCID http://orcid.org/0000-0003-3436-2475
Jonne DoorduinDepartment of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Reinier Postlaan 4 (910), PO Box 9101, 6500 HB, Nijmegen, The Netherlands.ORCID http://orcid.org/0000-0002-4157-1649
Peter J WijkstraDepartment of Pulmonary Diseases/Home Mechanical Ventilation, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.ORCID http://orcid.org/0000-0002-3809-5725
Corinne G C HorlingsDepartment of Neurology, Medical University Innsbruck, Innsbruck, Austria.ORCID http://orcid.org/0000-0002-2440-9180
Baziel G M van EngelenDepartment of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Reinier Postlaan 4 (910), PO Box 9101, 6500 HB, Nijmegen, The Netherlands.ORCID http://orcid.org/0000-0001-9867-9047
Karlien MulDepartment of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Reinier Postlaan 4 (910), PO Box 9101, 6500 HB, Nijmegen, The Netherlands.ORCID http://orcid.org/0000-0001-8487-9478
Radboud University Nijmegen · NLInnsbruck Medical University · ATUniversity Medical Center Groningen · NL

Funding

Prinses Beatrix Spierfonds W.OR12-22Prinses Beatrix Spierfonds W.OR18-07
6 · The paper itself

Abstract

objectiveTo evaluate the 5-year change in respiratory function in patients with facioscapulohumeral muscular dystrophy (FSHD).

methodsGenetically confirmed patients with FSHD aged ≥ 18 years were examined twice over five years. Forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) were measured using hand-held spirometry with a face mask. Several clinical outcome measures were correlated to respiratory function.

resultsNinety-two patients were included (57% male, age 18-75 years). At baseline, the spirometry outcomes of 41 patients showed a restrictive ventilatory pattern (FVC < 80% and FEV1/FVC ≥ 70% of predicted) and of 48 patients at follow-up. The mean FVC decreased from baseline to follow-up from 79.0 to 76.7% predicted (p = 0.021). This decrease was driven by a subgroup of 15 patients who had a deterioration of FVC of > 10% predicted. The subgroup of 15 patients was more severely affected at baseline (p = 0.002 for FSHD clinical score and 0.007 for Ricci score). They developed more frequently spinal and thorax deformities (p < 0.001 for kyphoscoliosis and 0.012 for pectus excavatum) and had a larger decline in axial muscle function (p = 0.020). Only weak correlations were found between the change in FVC% predicted and the change in clinical scores between baseline and follow-up.

interpretationRespiratory function remained stable in most patients with FSHD, but a subgroup of patients showed a pronounced deterioration. They showed more severe muscle weakness including the leg muscles at baseline (Ricci score ≥ 6), had spinal and thorax deformities and a relatively fast decline in axial muscle function at follow-up.

Indexed as

Muscular DiseasesMuscular Dystrophy, FacioscapulohumeralAdolescentAdultFemaleFollow-Up StudiesHumansMaleMiddle AgedRespiratory Function TestsSpirometryVital CapacityFacioscapulohumeral muscular dystrophyLong-term follow-upRespiratory functionSpirometry

Identifiers

PMID35147730
PMCPMC8831680
OpenAlexW4210989114

What OpenQuestion holds

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LicenceCC BY
Read underepoch 390

Registered trials

None linked

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 OpenQuestion graph.