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© 2024 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.

Abstract

Background/Objectives: Duchenne muscular dystrophy (DMD) is the most prevalent progressive muscular dystrophy, and the guidelines recommend the regular assessment of respiratory muscle function. This study aimed to assess the relationship between maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP) and sniff nasal inspiratory pressure (SNIP) measurements and upright-supine spirometry parameters in children with DMD, the predictability of upright–supine spirometry in terms of diaphragm involvement, and the impact of nutrition on muscle strength. Methods: This prospective cross-sectional study examined patients with DMD by comparing upright and supine FVC, MIP, MEP, and SNIP measurements. The effects of the ambulatory status, kyphoscoliosis, chest deformity, and low BMI on respiratory parameters were investigated. Results: Forty-four patients were included in the study. The mean patient age was 10.8 ± 2.9 years. Twenty-five patients were ambulatory. A significant decrease in FVC, FEV1, and FEF25–75 values was detected in the supine position in both ambulatory and non-ambulatory patients (p < 0.05). All patients had low MIP, MEP, and SNIP measurements (less than 60 cm H2O). MIP, MEP, and SNIP values were significantly lower in patients with a low BMI than in those without (p < 0.05). Conclusions: To accurately assess respiratory muscle strength, supine FVC should be combined with upright FVC, MIP, MEP, and SNIP measurements. It is crucial to regularly screen patients for nutrition, as this can significantly affect respiratory muscle function during pulmonology follow-up.

Details

Title
Unveiling the Respiratory Muscle Strength in Duchenne Muscular Dystrophy: The Impact of Nutrition and Thoracic Deformities, Beyond Spirometry
Author
Mine Yuksel Kalyoncu 1 ; Gokdemir, Yasemin 2 ; Cansu Yilmaz Yegit 3 ; Yanaz, Muruvvet 4 ; Gulieva, Aynur 2 ; Merve Selcuk 2 ; Karabulut, Şeyda 2 ; Çakar, Neval Metin 2 ; Ergenekon, Pinar 2 ; Ela Erdem Eralp 2   VIAFID ORCID Logo  ; Öztürk, Gülten 5 ; Unver, Olcay 5 ; Turkdogan, Dilsad 5 ; Sahbat, Yavuz 6 ; Ahmet Hamdi Akgülle 6 ; Karakoç, Fazilet 2 ; Karadag, Bulent 2   VIAFID ORCID Logo 

 Department of Pediatric Pulmonology, Dr. Lutfi Kirdar City Hospital, Istanbul 34865, Turkey 
 Department of Pediatric Pulmonology, School of Medicine, Marmara University, Istanbul 34899, Turkey; [email protected] (Y.G.); [email protected] (A.G.); [email protected] (M.S.); [email protected] (Ş.K.); [email protected] (N.M.Ç.); [email protected] (P.E.); [email protected] (E.E.E.); [email protected] (F.K.); [email protected] (B.K.) 
 Department of Pediatric Pulmonology, Çam and Sakura City Hospital, Istanbul 34480, Turkey; [email protected] 
 Department of Pediatric Pulmonology, Diyarbakir Child Hospital, Diyarbakir 21100, Turkey; [email protected] 
 Department of Pediatric Neurology, School of Medicine, Marmara University, Istanbul 34899, Turkey; [email protected] (G.Ö.); [email protected] (O.U.); [email protected] (D.T.) 
 Department of Orthopaedic Surgery and Traumatology, School of Medicine, Marmara University, Istanbul 34899, Turkey; [email protected] (Y.S.); [email protected] (A.H.A.) 
First page
994
Publication year
2024
Publication date
2024
Publisher
MDPI AG
e-ISSN
22279067
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
3097885644
Copyright
© 2024 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.