Respiratory muscle function in patients with nemaline myopathy.

Child Cross-Sectional Studies Diaphragm Female Humans Male Muscle Weakness Myopathies, Nemaline Respiratory Insufficiency Respiratory Muscles
Diaphragm Nemaline myopathy Respiratory muscles Spirometry Ultrasound

Journal

Neuromuscular disorders : NMD
ISSN: 1873-2364
Titre abrégé: Neuromuscul Disord
Pays: England
ID NLM: 9111470

Informations de publication

Date de publication:
08 2022
Historique:
received: 20 01 2022
revised: 16 06 2022
accepted: 20 06 2022
pubmed: 9 7 2022
medline: 19 8 2022
entrez: 8 7 2022
Statut: ppublish

Résumé

In this cross-sectional study, we comprehensively assessed respiratory muscle function in various clinical forms of nemaline myopathy (NM) including non-volitional tests for diaphragm function. Forty-two patients with NM were included (10 males (25-74 y/o); 32 females (11-76 y/o)). The NM forms were typical (n=11), mild (n=7), or childhood-onset with slowness of movements (n=24). Forced vital capacity (FVC) and maximal inspiratory pressure were decreased in typical NM in comparison with childhood-onset NM with slowness (32.0 [29.0-58.5] vs 81.0 [75.0-87.0]%, p<0.01, and 35.0 [24.0-55.0] vs 81.0 [65.0-102.5] cmH

Identifiants

DOI: 10.1016/j.nmd.2022.06.009 PMID: 35803773
pubmed: 35803773
pii: S0960-8966(22)00194-8
doi: 10.1016/j.nmd.2022.06.009
pii:
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

654-663

Informations de copyright

Copyright © 2022 The Authors. Published by Elsevier B.V. All rights reserved.

Déclaration de conflit d'intérêts

Declarations of interest None.

Auteurs

Esmee S B van Kleef (ESB)

Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands.

Jeroen L M van Doorn (JLM)

Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands.

Michael A Gaytant (MA)

Department of Pulmonary Diseases/Home Mechanical Ventilation, University Medical Center Utrecht, the Netherlands.

Willemien de Weerd (W)

Department of Pediatrics, Beatrix Children's Hospital, University Medical Center Groningen, the Netherlands.

Bettine A H Vosse (BAH)

Department of Pulmonary Diseases, Maastricht University Medical Center, the Netherlands.

Carina Wallgren-Pettersson (C)

Folkhälsan Institute of Genetics, Folkhälsan Research Center, Helsinki, Finland; Department of Medical and Clinical Genetics, Medicum, University of Helsinki, Helsinki, Finland.

Baziel G M van Engelen (BGM)

Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands.

Coen A C Ottenheijm (CAC)

Department of Physiology, Amsterdam UMC (location Vumc), the Netherlands.

Nicol C Voermans (NC)

Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands.

Jonne Doorduin (J)

Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands. Electronic address: Jonne.Doorduin@radboudumc.nl.

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Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Enfant › Respiratory muscle function in patients with...
questionsmedicales.fr › Environnement et santé publique › Santé publique › Méthodes épidémiologiques › Caractéristiques des études épidémiologiques › Études épidémiologiques › Études transversales › Respiratory muscle function in patients with...
questionsmedicales.fr › Appareil locomoteur › Muscles respiratoires › Muscle diaphragme › Respiratory muscle function in patients with...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Respiratory muscle function in patients with...
questionsmedicales.fr › États, signes et symptômes pathologiques › Signes et symptômes › Manifestations neurologiques › Manifestations neuromusculaires › Faiblesse musculaire › Respiratory muscle function in patients with...
questionsmedicales.fr › Maladies du système nerveux › Maladies neuromusculaires › Maladies musculaires › Myopathies congénitales structurales › Myopathies némaline › Respiratory muscle function in patients with...
questionsmedicales.fr › Maladies de l'appareil respiratoire › Troubles respiratoires › Insuffisance respiratoire › Respiratory muscle function in patients with...
questionsmedicales.fr › Appareil locomoteur › Muscles respiratoires › Respiratory muscle function in patients with...