Can forced vital capacity (FVC) or maximal inspiratory pressure (MIP) be used to predict changes in mobility, swallowing and/or cough peak flow in patients with type 1 myotonic dystrophy?

2018 ◽  
Vol 28 ◽  
pp. S8
Author(s):  
C. Massey ◽  
J. Allen ◽  
N. Nikolenko ◽  
L. Speigel ◽  
A.C. Jimenez-Moreno ◽  
...  
2020 ◽  
Vol 35 (9) ◽  
pp. 600-606
Author(s):  
Hans D. Katzberg ◽  
Jiri Vajsar ◽  
Kevin Vezina ◽  
Heba Qashqari ◽  
Sarah Selvadurai ◽  
...  

Objectives: The purpose of this study was to prospectively evaluate sleep patterns and the presence of sleep-disordered breathing in children with myasthenia gravis. We further aimed to examine the relationship between sleep and daytime respiratory function using spirometry tests including upright and supine forced vital capacity, sniff nasal inspiratory pressure, and maximal inspiratory pressure. Methods: Eleven children between 3 and 18 years old with confirmed myasthenia gravis were recruited from The Hospital for Sick Children Neuromuscular Clinic in this prospective observational study. After informed consent was obtained, patients underwent a comprehensive clinical assessment with collection of anthropometric data. Following this, all subjects performed pulmonary function tests, overnight polysomnography and completed the Epworth Sleepiness Scale questionnaire. Results: Two of eleven children who reported no symptoms of sleep disordered breathing were diagnosed with mild to moderate obstructive sleep apnea. Pulmonary function tests showed abnormal maximal inspiratory pressure in 6 of 11 patients, whereas seated forced vital capacity as well as seated to supine forced vital capacity ratios were normal in the entire group. Conclusions: In our small group of pediatric myasthenia gravis subjects, there was an unexpected finding of obstructive sleep apnea in 2 of the 11 patients studied. Maximal inspiratory pressure appears to be a more sensitive method of detecting abnormalities compared to upright or seated forced vital capacity. A larger multicenter study is needed to validate our findings and to determine the impact of obstructive sleep apnea in the pediatric myasthenia gravis population as well as risk factors associated with sleep disordered breathing.


2011 ◽  
Vol 17 (2) ◽  
pp. 236-239 ◽  
Author(s):  
Hélène Prigent ◽  
David Orlikowski ◽  
Nadège Letilly ◽  
Line Falaize ◽  
Djilali Annane ◽  
...  

1993 ◽  
Vol 18 (3) ◽  
pp. 317-324 ◽  
Author(s):  
W. Donald F. Smith ◽  
David A. Cunningham ◽  
Donald H. Paterson ◽  
Peter A. Rechnitzer

The volume measurement module turbine (VMM) was evaluated in 51 subjects for spirometry in applied physiology against the Stead-Wells spirometer (SW) and Wright peak flow meter (WM). The volume and flow ranges (VMM) were, FEV1 1.32 to 3.94 L (mean 2.62, confidence interval [CI] 2.46 to 2.78); forced vital capacity (FVC) 1.97 to 5.06 L (mean 3.50, CI 3.29 to 3.71); and peak expiratory flow rate (PEFR) 290 to 624 L∙min−1 (mean 434, CI 407 to 461). The mean difference for FEV1 was 0.09 L (CI 0.05 to 0.14), FVC 0.04 L (CI −0.02 to 0.10), and PEFR 18.0 L min−1 (CI 8.7 to 27.3) less than SW or WM. Bias with FEV1 and FVC was not significant, though PEFR demonstrated a significant proportional error. The repeatability coefficients for FEV1 and FVC were 0.18 and 0.20, comparable to the SW; but for PEFR they were greater, 58.4 versus 33.8 L∙min−1 by WM. The VMM turbine is accurate and reliable for the measurement of FEV1 and FVC over the ranges studied; however, care should be taken when interpreting PEFR. Key words: lung volumes, FEV1 FVC


1979 ◽  
Vol 51 (5) ◽  
pp. 456-459 ◽  
Author(s):  
YASUHIRO SHIMADA ◽  
IKUTO YOSHIYA ◽  
KAZUHIKO TANAKA ◽  
TOJT YAMAZAKI ◽  
KEIJI KUMON

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Gro Solbakken ◽  
Sissel Løseth ◽  
Anne Froholdt ◽  
Torunn D. Eikeland ◽  
Terje Nærland ◽  
...  

Abstract Background Pain is prevalent in myotonic dystrophy 1 (DM1). This study investigated whether CTG repeat size, disease duration, BMI and motor and psychological function were related to pain in adult patients with DM1, and if there were gender differences regarding intensity and location of pain. Method Cross-sectional design. Pain was investigated in 50 genetically confirmed DM1 patients by combining clinical assessment and self-reports of pain intensity and locations. Pain scoring results were related to CTG size, disease duration, muscle strength, walking capacity measured by 6-min walk test, activity of daily life by Katz ADL Index, respiratory function by Forced Vital Capacity and BMI. In addition, the degree of reported pain was related to Quality of life measured by WHOQOL-BREF; fatigue was measured by Fatigue severity scale; psychological functions were measured by Beck Depression Inventory, Beck Anxiety Inventory, IQ and Autism spectrum Quotient. Results Pain was reported in 84% of the patients and was significantly correlated with CTG size (r = 0.28 p = 0.050), disease duration (r = 0.38 p = 0.007), quality of life (r = − 0.37 p = 0.009), fatigue (r = 0.33 p = 0.02) and forced vital capacity (r = − 0.51, p = 0.005). Significant gender differences, with higher scores for females, were documented. In male subjects the number of pain locations was significantly correlated with quality of life and the autism quotient. In females, pain intensity was significantly correlated with activity, respiratory function and BMI. Conclusions Pain in DM1 was prevalent, with a strong association to lung function and other aspects of the disease. Significant gender differences were present for pain intensity and number of pain locations. How pain was related to other symptoms differed between male and female subjects. Our findings highlight the importance of assessments of pain in DM1 patients.


2018 ◽  
pp. 53-59
Author(s):  
Yu. V. Kyrychenko

In the modern world, there are many causes (reducing the elasticity of the lungs, reducing bronchial tubes, reducing the strength of the respiratory muscles) that affect the parameters of external respiration. Knowledge of age, sexual and regional features of spirometric indicators helps to differentiate and detect the degree of disturbance of respiratory biomechanics and to choose effective treatments that are most appropriate for established violations. The purpose of the work is to establish the age-old peculiarities of the spirographic indexes of virtually healthy young men and women within the juvenile period of ontogenesis. We conducted a survey of 141 young women (from 16 to 20 years of age) and 154 young men (from 17 to 21 years) of adolescence. The spirographic study was conducted according to the generally accepted methodology of the American Association of Pulmonologists adopted in 1994 on the device Medgraphics Pulmonary Function System 1070 series. The analysis of the obtained results was carried out with the help of the license program "Statistica 5.5" using nonparametric methods of estimating indicators. The progressive age dynamics of the vital capacity, forced vital capacity, forced capacity of the lungs on the inhalation, volume exhalation velocity was detected in young women in 25% and 50% respectively of the forced vital capacity, the average expiration flow, the residual volume of exhalation and the forced inspiratory flow, respectively is 50% of exhalation from the forced vital capacity. In the last year (20 years) of the youthful period of ontogenesis in young women, the maximum arbitrary ventilation of the lungs is increased, the volume exhalation rate is 75%, and from 75% to 85% of exhalation from the forced vital capacity, the one-second volume of forced exhalation and maximal peak flow of exhalation. Within the juvenile period of ontogenesis in young men, the vital capacity of the lungs increases, the maximum arbitrary ventilation of the lungs and the maximum peak flow of exhalation. Only in the last year of the youthful period of ontogeny (21 years) in young men there is an increase in forced vital capacity, volume exhalation velocity, respectively, in 25% of the forced vital capacity, the residual volume of exhalation, one-second volume of forced exhalation was observed at the end.


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