scholarly journals Respiratory muscle weakness in facioscapulohumeral muscular dystrophy

2019 ◽  
Vol 60 (6) ◽  
pp. 679-686 ◽  
Author(s):  
Carolin Henke ◽  
Jens Spiesshoefer ◽  
Hans‐Joachim Kabitz ◽  
Simon Herkenrath ◽  
Winfried Randerath ◽  
...  
2012 ◽  
Vol 228 (1) ◽  
pp. 77-87 ◽  
Author(s):  
Justin M Percival ◽  
Nicholas P Whitehead ◽  
Marvin E Adams ◽  
Candace M Adamo ◽  
Joseph A Beavo ◽  
...  

Gerontology ◽  
2021 ◽  
pp. 1-10
Author(s):  
Tatsuma Okazaki ◽  
Yoshimi Suzukamo ◽  
Midori Miyatake ◽  
Riyo Komatsu ◽  
Masahiro Yaekashiwa ◽  
...  

Introduction: The respiratory muscle strength regulates the effectiveness of coughing, which clears the airways and protects people from pneumonia. Sarcopenia is an aging-related loss of muscle mass and function, the worsening of which is associated with malnutrition. The loss of respiratory and swallowing muscle strength occurs with aging, but its effect on pneumonia is unclear. This study aimed to determine the risks of respiratory muscle weakness on the onset and relapse of pneumonia in older people in conjunction with other muscle-related factors such as malnutrition. Methods: We conducted a longitudinal study with 47 pneumonia inpatients and 35 non-pneumonia controls aged 70 years and older. We evaluated the strength of respiratory and swallowing muscles, muscle mass, and malnutrition (assessed by serum albumin levels and somatic fat) during admission and confirmed pneumonia relapse within 6 months. The maximal inspiratory and expiratory pressures determined the respiratory muscle strength. Swallowing muscle strength was evaluated by tongue pressure. Bioelectrical impedance analysis was used to evaluate the muscle and fat mass. Results: The respiratory muscle strength, body trunk muscle mass, serum albumin level, somatic fat mass, and tongue pressure were significantly lower in pneumonia patients than in controls. Risk factors for the onset of pneumonia were low inspiratory respiratory muscle strength (odds ratio [OR], 6.85; 95% confidence interval [CI], 1.56–30.11), low body trunk muscle mass divided by height2 (OR, 6.86; 95% CI, 1.49–31.65), and low serum albumin level (OR, 5.46; 95% CI, 1.51–19.79). For the relapse of pneumonia, low somatic fat mass divided by height2 was a risk factor (OR, 20.10; 95% CI, 2.10–192.42). Discussion/Conclusions: Respiratory muscle weakness, lower body trunk muscle mass, and malnutrition were risk factors for the onset of pneumonia in older people. For the relapse of pneumonia, malnutrition was a risk factor.


CHEST Journal ◽  
2013 ◽  
Vol 144 (4) ◽  
pp. 326A
Author(s):  
Urooj Fatima ◽  
Tasneem Shah ◽  
Paras Dedhia ◽  
Muhammad Ebrahim Khan ◽  
Sean Devine

2014 ◽  
Vol 20 (10) ◽  
pp. S136
Author(s):  
Yoshiharu Kinugasa ◽  
Kensaku Yamada ◽  
Takeshi Sota ◽  
Mari Miyaki ◽  
Shinobu Sugihara ◽  
...  

CHEST Journal ◽  
1986 ◽  
Vol 90 (4) ◽  
pp. 546-552 ◽  
Author(s):  
F. Dennis McCool ◽  
Raymond F. Mayewski ◽  
David S. Shayne ◽  
Charles J. Gibson ◽  
Robert C. Griggs ◽  
...  

Neurology ◽  
2018 ◽  
Vol 92 (4) ◽  
pp. e378-e385 ◽  
Author(s):  
Rianne J.M. Goselink ◽  
Karlien Mul ◽  
Caroline R. van Kernebeek ◽  
Richard J.L.F. Lemmers ◽  
Silvère M. van der Maarel ◽  
...  

ObjectiveTo assess the relation between age at onset and disease severity in facioscapulohumeral muscular dystrophy (FSHD).MethodsIn this prospective cross-sectional study, we matched adult patients with FSHD with an early disease onset with 2 sex-matched FSHD control groups with a classic onset; the first group was age matched, and the second group was disease duration matched. Genetic characteristics, muscle performance, respiratory functioning, hearing loss, vision loss, epilepsy, educational level, and work status were compared with the 2 control groups.ResultsTwenty-eight patients with early-onset FSHD were age (n = 28) or duration (n = 27) matched with classic-onset patients. Patients with early-onset FSHD had more severe muscle weakness (mean FSHD clinical score 11 vs 5 in the age-matched and 9 in the duration-matched group, p < 0.05) and a higher frequency of wheelchair dependency (57%, 0%, and 30%, respectively, p < 0.05). In addition, systemic features were more frequent in early-onset FSHD, most important, hearing loss, decreased respiratory function and spinal deformities. There was no difference in work status. Genetically, the shortest D4Z4 repeat arrays (2–3 units) were found exclusively in the early-onset group, and the largest repeat arrays (8–9 units) were found only in the classic-onset groups. De novo mutations were more frequent in early-onset patients (46% vs 4%).ConclusionsPatients with early-onset FSHD more often have severe muscle weakness and systemic features. The disease severity is greater than in patients with classic-onset FSHD who are matched for disease duration, suggesting that the progression is faster in early-onset patients.


Amyloid ◽  
2008 ◽  
Vol 15 (2) ◽  
pp. 129-136
Author(s):  
Arnt V. Kristen ◽  
Thomas J. Dengler ◽  
J. H. Kristen ◽  
Stefan O. Schonland ◽  
Ute Hegenbart ◽  
...  

Introduction 238 Assessment 239 Treatment 240 A variety of neuromuscular disorders may affect the ventilatory pump at different sites (Table 37.1). Most of these disorders result in respiratory muscle weakness, which results in alveolar hypoventilation and impaired cough. Patients with known neuromuscular disease may present acutely with a presentation related to their underlying neuromuscular disease (such as infection) or occasionally in end-stage ventilatory failure....


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