scholarly journals Chest MRI to diagnose early diaphragmatic weakness in Pompe disease

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Laurike Harlaar ◽  
Pierluigi Ciet ◽  
Gijs van Tulder ◽  
Alice Pittaro ◽  
Harmke A. van Kooten ◽  
...  

Abstract Background In Pompe disease, an inherited metabolic muscle disorder, severe diaphragmatic weakness often occurs. Enzyme replacement treatment is relatively ineffective for respiratory function, possibly because of irreversible damage to the diaphragm early in the disease course. Mildly impaired diaphragmatic function may not be recognized by spirometry, which is commonly used to study respiratory function. In this cross-sectional study, we aimed to identify early signs of diaphragmatic weakness in Pompe patients using chest MRI. Methods Pompe patients covering the spectrum of disease severity, and sex and age matched healthy controls were prospectively included and studied using spirometry-controlled sagittal MR images of both mid-hemidiaphragms during forced inspiration. The motions of the diaphragm and thoracic wall were evaluated by measuring thoracic cranial-caudal and anterior–posterior distance ratios between inspiration and expiration. The diaphragm shape was evaluated by measuring the height of the diaphragm curvature. We used multiple linear regression analysis to compare different groups. Results We included 22 Pompe patients with decreased spirometry results (forced vital capacity in supine position < 80% predicted); 13 Pompe patients with normal spirometry results (forced vital capacity in supine position ≥ 80% predicted) and 18 healthy controls. The mean cranial-caudal ratio was only 1.32 in patients with decreased spirometry results, 1.60 in patients with normal spirometry results and 1.72 in healthy controls (p < 0.001). Anterior–posterior ratios showed no significant differences. The mean height ratios of the diaphragm curvature were 1.41 in patients with decreased spirometry results, 1.08 in patients with normal spirometry results and 0.82 in healthy controls (p = 0.001), indicating an increased curvature of the diaphragm during inspiration in Pompe patients. Conclusions Even in early-stage Pompe disease, when spirometry results are still within normal range, the motion of the diaphragm is already reduced and the shape is more curved during inspiration. MRI can be used to detect early signs of diaphragmatic weakness in patients with Pompe disease, which might help to select patients for early intervention to prevent possible irreversible damage to the diaphragm.

2004 ◽  
Vol 96 (1) ◽  
pp. 65-75 ◽  
Author(s):  
Hye-Won Shin ◽  
Christine M. Rose-Gottron ◽  
Dan M. Cooper ◽  
Robert L. Newcomb ◽  
Steven C. George

Exhaled nitric oxide (NO) concentration is a noninvasive index for monitoring lung inflammation in diseases such as asthma. The plateau concentration at constant flow is highly dependent on the exhalation flow rate and the use of corticosteroids and cannot distinguish airway and alveolar sources. In subjects with steroid-naive asthma ( n = 8) or steroid-treated asthma ( n = 12) and in healthy controls ( n = 24), we measured flow-independent NO exchange parameters that partition exhaled NO into airway and alveolar regions and correlated these with symptoms and lung function. The mean (±SD) maximum airway flux (pl/s) and airway tissue concentration [parts/billion (ppb)] of NO were lower in steroid-treated asthmatic subjects compared with steroid-naive asthmatic subjects (1,195 ± 836 pl/s and 143 ± 66 ppb compared with 2,693 ± 1,687 pl/s and 438 ± 312 ppb, respectively). In contrast, the airway diffusing capacity for NO (pl·s-1·ppb-1) was elevated in both asthmatic groups compared with healthy controls, independent of steroid therapy (11.8 ± 11.7, 8.71 ± 5.74, and 3.13 ± 1.57 pl·s-1·ppb-1 for steroid treated, steroid naive, and healthy controls, respectively). In addition, the airway diffusing capacity was inversely correlated with both forced expired volume in 1 s and forced vital capacity (%predicted), whereas the airway tissue concentration was positively correlated with forced vital capacity. Consistent with previously reported results from Silkoff et al. (Silkoff PE, Sylvester JT, Zamel N, and Permutt S, Am J Respir Crit Med 161: 1218-1228, 2000) that used an alternate technique, we conclude that the airway diffusing capacity for NO is elevated in asthma independent of steroid therapy and may reflect clinically relevant changes in airways.


1995 ◽  
Vol 78 (3) ◽  
pp. 1132-1139 ◽  
Author(s):  
M. Orozco-Levi ◽  
J. Gea ◽  
J. Sauleda ◽  
J. M. Corominas ◽  
J. Minguella ◽  
...  

The aim of this study was to evaluate whether respiratory function influences the structure of the latissimus dorsi muscle (LD). Twelve patients (58 +/- 10 yr) undergoing thoracotomy were studied. Lung and respiratory muscle function were evaluated before surgery. Patients showed a forced expired volume in 1 s (FEV1) of 67 +/- 16% of the reference value, an FEV1-forced vital capacity ratio of 69 +/- 9%, a maximal inspiratory pressure of 101 +/- 21% of the reference value, and a tension-time index of the diaphragm (TTdi) of 0.04 +/- 0.02. When patients were exposed to 8% CO2 breathing, TTdi increased to 0.06 +/- 0.03 (P < 0.05). The structural analysis of LD showed that 51 +/- 5% of the fibers were type I. The diameter was 56 +/- 9 microns for type I fibers and 61 +/- 9 microns for type II fibers, whereas the hypertrophy factor was 87 +/- 94 and 172 +/- 208 for type I and II fibers, respectively. Interestingly, the histogram distribution of the LD fibers was unimodal in two of the three individuals with normal lung function and bimodal (additional mode of hypertrophic fibers) in seven of the nine patients with chronic obstructive pulmonary disease. An inverse relationship was found between the %FEV1-forced vital capacity ratio and both the diameter of the fibers (type I: r = -0.773, P < 0.005; type II: r = -0.590, P < 0.05) and the hypertrophy factors (type I: r = -0.647, P < 0.05; type II: r = -0.575, P = 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)


2015 ◽  
Vol 13 (2) ◽  
pp. 249-254 ◽  
Author(s):  
Fabiana Vieira Breijão Zani ◽  
José Eduardo Aguilar-Nascimento ◽  
Diana Borges Dock Nascimento ◽  
Ageo Mário Cândido da Silva ◽  
Fernanda Stephan Caporossi ◽  
...  

ABSTRACT Objective: To evaluate the change in respiratory function and functional capacity according to the type of preoperative fasting. Methods: Randomized prospective clinical trial, with 92 female patients undergoing cholecystectomy by laparotomy with conventional or 2 hours shortened fasting. The variables measured were the peak expiratory flow, forced expiratory volume in the first second, forced vital capacity, dominant handgrip strength, and non-dominant handgrip strength. Evaluations were performed 2 hours before induction of anesthesia and 24 hours after the operation. Results: The two groups were similar in preoperative evaluations regarding demographic and clinical characteristics, as well as for all variables. However, postoperatively the group with shortened fasting had higher values than the group with conventional fasting for lung function tests peak expiratory flow (128.7±62.5 versus 115.7±59.9; p=0.040), forced expiratory volume in the first second (1.5±0.6 versus 1.2±0.5; p=0.040), forced vital capacity (2.3±1.1 versus 1.8±0.9; p=0.021), and for muscle function tests dominant handgrip strength (24.9±6.8 versus 18.4±7.7; p=0.001) and non-dominant handgrip strength (22.9±6.3 versus 17.0±7.8; p=0.0002). In the intragroup evaluation, there was a decrease in preoperative compared with postoperative values, except for dominant handgrip strength (25.2±6.7 versus 24.9±6.8; p=0.692), in the shortened fasting group. Conclusion: Abbreviation of preoperative fasting time with ingestion of maltodextrin solution is beneficial to pulmonary function and preserves dominant handgrip strength.


2013 ◽  
Vol 1 (1) ◽  
Author(s):  
Ester Florencia Sagay ◽  
Hedison Polii ◽  
Herlina I. S. Wungouw

Abstract: Changes to respiratory function due to regular aerobic exercise will affect the value of pulmonary function, especially in Forced Vital Capacity (FVC). This research aimed to determine the effect of aerobic exercise on FVC overweight male students of Unsrat Medical Faculty. This research is analytic with design experimental, one group pre and post test design. The sampling technique used is non-purposive sampling technique. The research sample was taken from the students of the Faculty of Medicine 2009, Univercity of Sam Ratulangi who fulfill the inclusion criteria. Some 32 students were selected as research subjects. After giving informed consent, FVC measurement was done with the spirometer. After it was measured, they were given treatmen in the form aerobic exercise using a stationary bike for three weeks with frequency of exercise three times a week and exercise intensity for 30 minutes. We measured again FVC values after the exercise three times program. Normality test data showed significance for FVC value before treatment by 0.752, and after treatment by 0.912. Comparison of the average value before and after exercise were tested by using a paired test. Significant value for FVC is P = 0.084, means there is no significant difference between FVC values before and after exercise (P> 0.05). The mean FVC was 3.88 before treatment and after treatment the mean value was 4.00, an increase in the average value of 0.11. Conclusion:Aerobic Exercise on a regular basis using a stationary bike on the overweight male student can improve lung function in particular the mean FVC but there was no significant difference from the mean value. Keywords: FVC, Aerobic Exercise, Overweight.   Abstrak: Perubahan fungsi pernapasan karena latihan aerobik secara teratur akan mempengaruhi nilai fungsi paru khususnya Forced Vital Capacity (FVC). Penelitian ini untuk mengetahui pengaruh latihan aerobik terhadap FVC mahasiswa pria Fakultas Kedokteran Unsrat dengan berat badan lebih.Penelitian ini bersifat analitik dengan rancangan eksperimental one grup pre and post test design. Pengambilan sampel dilakukan dengan teknik non purposive sampling. Sampel penelitian diambil dari Mahasiswa Fakultas Kedokteran Universitas Sam Ratulangi Angkatan 2009.Sejumlah 32 orang mahasiswa terpilih sebagai subjek penelitian dan dilakukan pengukuran FVC dengan Spirometer.Setelah itu diberikan perlakuan berupa latihan aerobik menggunakan sepeda statis selama tiga minggu dengan frekuensi latihan tiga kali seminggu dan intensitas latihan selama 30 menit.Selanjutnya dilakukan pengukuran kembali nilai FVC sesudah program latihan.Uji normalitas data menunjukkan nilai signifikansi untuk FVC sebelum perlakuan sebesar 0.752, dan sesudah perlakuan sebesar 0.912. Perbandingan nilai rata  rata  sebelum dan sesudah latihan diuji dengan menggunakan uji t berpasangan.Nilai signifikan untuk FVC adalah P = 0.084, berarti tidak terdapat perbedaan yang bermakna antara nilai FVC sebelum dan sesudah latihan (P > 0.05) .Nilai rerata FVC sebelum pelakuan adalah 3,88  dan nilai rerata sesudah perlakuan adalah  4,00,  terjadi penigkatan nilai rerata sebesar 0,11.Simpulan:Latihan Aerobik menggunakan sepeda statis secara teratur dapat meningkatkan nilai rerata fungsi paru khususnya FVC tetapi tidak terdapat perbedaan yang bermakna dari nilai rerata tersebut. Kata Kunci: FVC, Latihan Aerobik, Berat Badan Lebih (Overweight).


2003 ◽  
Vol 98 (6) ◽  
pp. 1333-1337 ◽  
Author(s):  
Matthias Eikermann ◽  
Harald Groeben ◽  
Johannes Hüsing ◽  
Jürgen Peters

Background Residual paralysis increases the risk of pulmonary complications but is difficult to detect. To test the hypothesis that accelerometry predicts effects of residual paralysis on pulmonary and upper airway function, the authors related tests of pulmonary and pharyngeal function to accelerometry of adductor pollicis muscle in 12 partially paralyzed volunteers. Methods Rocuronium (0.01 mg/kg + 2-10 microg x kg-1 x min-1) was administered to maintain train-of-four (TOF) ratios (assessed every 15 s) of approximately 0.5 and 0.8 over a period of more than 5 min. The authors evaluated pharyngeal and facial muscle functions during steady state relaxation and performed spirometric measurements every 5 min until recovery. Upper airway obstruction was defined as a mean ratio of expiratory and inspiratory flow at 50% of vital capacity of greater than 1. The TOF ratio associated with "acceptable" pulmonary recovery (forced vital capacity and forced inspiratory volume in 1 s of &gt; or =90% of baseline) was calculated using a linear regression model. Results At peak blockade (TOF ratio 0.5 +/- 0.16), forced inspiratory flow was impaired (53 +/- 19%) to a greater degree than forced expiratory flow (75 +/- 20%) with a mean ratio of expiratory and inspiratory flow at 50% of vital capacity of 1.18 +/- 0.6. Upper airway obstruction, observed in 8 of 12 volunteers, paralleled an impaired ability to swallow reported by 10 of 12 volunteers. In contrast, all volunteers except one could sustain a head lift for more than 5 s. The authors calculated that a mean TOF ratio of 0.56 (95% confidence interval, 0.22-0.71) predicts "acceptable" recovery of forced vital capacity, whereas forced inspiratory volume in 1 s was impaired until a TOF ratio of 0.95 (0.82-1.18) was reached. A 100% recovery of TOF ratio predicts an acceptable recovery of forced vital capacity, forced inspiratory volume in 1 s, and mean ratio of expiratory and inspiratory flow at 50% of vital capacity in 93%, 73%, and 88% of measurements (calculated negative predictive values), respectively. Conclusion Impaired inspiratory flow and upper airway obstruction frequently occur during minimal neuromuscular blockade (TOF ratio 0.8), and extubation may put the patient at risk. Although a TOF ratio of unity predicts a high probability of adequate recovery from neuromuscular blockade, respiratory function can still be impaired.


Neurosurgery ◽  
1987 ◽  
Vol 21 (2) ◽  
pp. 193-196 ◽  
Author(s):  
David H. Reines ◽  
Robert C. Harris

Abstract The records of 123 consecutive patients admitted with spinal cord injury were examined for the presence of pulmonary complications. Forty-nine had tetraplegia and 23 had paraplegia; the remainder suffered a variety of neurological deficits. Multiple injuries were encountered in 36 patients. Fifty-three pulmonary complications were noted in 44 (35.7%) patients. The most common problems were atelectasis and pneumonia. There were 22 (18%) deaths. Fourteen deaths were related to pulmonary complications. The mean age of patients who died was 52 ± 13 (SE) compared to 28 ± 12 for survivors. A mean forced vital capacity (FVC) of 1127 ± 410 cc in patients suffering respiratory difficulties compared to a FVC of 1865 ± 85 cc in patients without complications (P &lt; 0.001). Oxygenation (PaO2 90 ± 19 torr) was normal in patients without respiratory problems and was abnormal in patients developing problems (PaO2 76 ± 30 torr; P &lt; 0.05). Twenty patients were treated with a rotating bed. The complication rate of patients on the bed was only 10%. In conclusion, respiratory problems remain a significant cause of morbidity and mortality in spinal cord injury. The forced vital capacity, blood oxygen tension, and age are predictors of pulmonary complications. The use of a multidisciplinary approach and a rotating bed may minimize these problems.


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


1972 ◽  
Vol 42 (3) ◽  
pp. 371-381 ◽  
Author(s):  
J. S. Milne ◽  
J. Williamson

1. Forced expiratory volume in ml (FEV1·0) and forced vital capacity (FVC) were measured in a random sample of older people (215 men, 272 women) aged 62 years and upwards. 2. Multiple regression equations were calculated to predict these variables by using age and height. In contrast with younger groups most of the variance was not explained by these equations. 3. The equations developed predict lower values for FEV1·0 and FVC than other published series most of which contain relatively few older people.


2017 ◽  
Vol 120 (1-2) ◽  
pp. S62
Author(s):  
Alaa Hamed ◽  
Steve Kanters ◽  
Andrew Stewart ◽  
Milki Tilimo ◽  
Anna Bolzani ◽  
...  

2012 ◽  
Vol 2 (1) ◽  
pp. 29-32 ◽  
Author(s):  
Dipok Kumar Sunyal ◽  
Md Ruhul Amin ◽  
Ayesha Yasmin ◽  
Golam Morshed Molla ◽  
Md Liakat Ali ◽  
...  

Background: A few data are available on the effects of pregnancy on pulmonary function in different countries. But no such established data are available in our country. So we designed this study in our population. Objectives: To observe the forced expiratory volume in first second (FEV1) and ratio of forced expiratory volume in first second and forced vital capacity (FEV1/FVC%) in different trimesters of normal pregnant women and to compare them with those of healthy non-pregnant women. Materials and Methods: This observational and analytical study was carried out in the department of Physiology, Dhaka Medical College during July 2004 to June 2005. Total 100 women aged from 25 to 35 years without any recent history of respiratory tract diseases were selected as study population. Among them, 75 normal pregnant women were taken as experimental and 25 healthy non-pregnant women were taken as control groups. The experimental group included 25 pregnant women in first trimester, 25 in second trimester and 25 in third trimester. Forced expiratory volume in first second (FEV1), ratio of percentage of forced expiratory volume in first second and forced vital capacity (FEV1/FVC%) were measured in pregnant and non-pregnant control women. The FEV1 and FEV1/FVC% were measured by using an ‘automatic spirometer’. Statistical analyses were done by unpaired Student’s ‘t’ test between the study groups and p value <0.05 was taken as significant.Results: The mean ± SD of measured values of FEV1 were 2.41 ± 0.87, 2.28 ± 0.59, 2.15 ± 0.74 and 1.89 ± 0.76 liters in non-pregnant women and in pregnant women during first trimester, second trimester and third trimester. The mean ± SD of measured values of FEV1/FVC% were 75.22 ± 16.77, 74.86 ± 11.06, 74.42 ± 17.43 and 71.81 ± 15.87% in nonpregnant women and in pregnant women during first trimester, second trimester and third trimester. Conclusion: The FEV1 and FEV1/FVC% were significantly lower in third trimester pregnant women than that of non-pregnant and first trimester of pregnant women and FEV1/FVC% gradually decreased from first to third trimester of pregnant women. DOI: http://dx.doi.org/10.3329/jemc.v2i1.11926 J Enam Med Col 2012; 2(1): 29-32


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