The purpose of this study is to investigate how respiratory muscle strength correlates cough capacity in patients with weakness.Forty-five amyotrophic lateral sclerosis (ALS), 43 cervical spinal cord injury (SCI), and 42 Duchenne muscular dystrophy (DMD) were recruited. Pulmonary function tests including forced vital (FVC) (maximal expiratory pressure, MEP; maximal inspiratory MIP) performed. correlation between was analyzed.In the SCI group, FVC a supine position (2,597 +/- 648 mL)...

Abstract Purpose. To compare the accuracy rates between ultrasound (US)‐guided and blind knee intra‐articular injection via suprapatellar bursa. Methods. Ninety‐nine patients with radiographically confirmed osteoarthritis (Kellgren/Lawrence grade 2 or 3) without effusion were included. Fifty assigned to US‐guided group 49 group. After a of hyaluronic acid (HA) contrast dye through bursa into joint, radiographic image was obtained in each case ascertain whether all injected material had...

Purpose: To assess the ability of a mechanical in-exsufflator (MI-E), either alone or in combination with manual thrust, to augment cough patients neuromuscular disease (NMD) and respiratory muscle dysfunction.Materials Methods: For this randomized crossover single-center controlled trial, noninvasive ventilator-dependent NMD were recruited.The primary outcome was peak flow (PCF), which measured each patient after that unassisted, manually assisted following maximum insufflation capacity...

Background With advancements in cardiorespiratory care, the lifespan of patients with Duchenne Muscular Dystrophy (DMD) has significantly increased, shifting focus from merely extending life to enhancing quality and managing complications effectively. However, there is a lack interest management adult DMD, highlighting gap holistic care approaches for this aging population. Objective This study aims assess current state DMD South Korea, focusing on multidisciplinary identifying key areas...

Background/Objectives: Airstacking is a technique to improve lung compliance and maximum insufflation capacity (MIC) in patients with neuromuscular disorders by sequentially inflating the lungs using manual resuscitation bag. Traditional methods lack standardization rely on subjective feedback. A pilot study established optimal pressure ranges digital manometer, suggesting its potential standardize airstacking. This evaluates longitudinal effects of airstacking without feedback pulmonary...

Objective This study compared transcutaneous carbon dioxide partial pressure (PtcCO2) and end-tidal (PetCO2) monitoring during sleep for patients with neuromuscular disease. Design is a retrospective of whose PtcCO2 PetCO2 were monitored before they began using noninvasive mechanical ventilation. The outcomes divided into four groupings: group 1, both are greater than or equal to 49 mm Hg; 2, Hg but less 3, 4, Hg. Results A total 39 subjects (mean [SD] age, 27.7 [19.3] yrs) enrolled. values...

To investigate the relationship between serum creatine kinase (CK) level and pulmonary function in Duchenne muscular dystrophy (DMD).A total of 202 patients with DMD admitted to Department Rehabilitation Medicine, Gangnam Severance Hospital were enrolled from January 1, 1999 March 31, 2015. Seventeen excluded. Data collected 185 included age, height, weight, body mass index, tests including forced vital capacity (FVC), peak cough flow, maximal expiratory pressure (MEP), inspiratory (MIP),...

Atopic dermatitis (AD) is a chronic inflammatory skin disease characterized by highly pruritic, erythematous, and eczematous plaques. We previously reported that phospholipase A2 (PLA2) derived from bee venom alleviates AD-like lesions induced 2,4-dinitrochlorobenzene (DNCB) house dust mite extract (Dermatophagoides farinae extract, DFE) in murine model. However, the underlying mechanisms of PLA2 action actopic remain unclear. In this study, we showed treatment inhibited epidermal thickness,...

Objective To evaluate pulmonary functions of patients with amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and myotonic (MMD) at the onset ventilatory insufficiency. Methods This retrospective study included ALS, DMD, MMD regular outpatient clinic follow-up in Department Rehabilitation Medicine Gangnam Severance Hospital before application non-invasive positive pressure ventilation (NIPPV). The were enrolled from August 2001 to March 2014. If experienced...

ABSTRACT Introduction : A change in vital capacity (VC) from standing to supine can be an index of diaphragm paralysis if it exceeds 25%. We aimed verify whether the postural VC difference increases with age and reflects weakness DMD. Methods VCs were measured Postural percentage calculated data. Maximal inspiratory pressure (MIP) MIP as indirect weakness. Results total 220 patients 544 measurements collected. decreased significantly ( P < 0.001 for both). Estimated also 0.001, = 0.006,...

Background: Duchenne muscular dystrophy (DMD) patients can have various issues that affect their quality of life, including eating and digestive conditions. Objective: We sought to identify the relationship between respiratory function digestion related symptoms in with advanced (DMD). Methods: Eating symptoms, loss appetite, nausea, vomiting, diarrhea, constipation, swallowing difficulty, mastication early satiety, aspiration, were evaluated among DMD who nonambulatory required noninvasive...

Abstract Aims Despite advances in contemporary cardiopulmonary therapies, cardiomyopathy remains the leading cause of death patients with Duchenne muscular dystrophy (DMD). Also, long‐term clinical outcomes DMD and is unknown. This study investigated their associated factors late‐stage DMD. Methods results A total 116 (age > 15 years) were enrolled this retrospective study. All followed up at a single tertiary referral hospital. LV systolic dysfunction was dichotomously defined as reduced...

To estimate the efficiency of a cough assistance device, Cough Aid, in patients with weak respiratory muscles bulbar palsy and/or tracheostomy. The Aid is device that has been developed to substitute for glottis function.Before-after trial.A total 74 tracheostomy, as well muscle weakness, were recruited.Forced vital capacity, unassisted peak flow, lung insufflation and assisted flow measured via tracheostomy or oronasal interface. Lung capacity using Aid.In all subjects, capacities...

Abstract Patients with myotonic muscular dystrophy type 1 (DM1) tend to exhibit earlier respiratory insufficiency than patients other neuromuscular diseases at similar or higher forced vital capacity (FVC). This study aimed analyze several pulmonary function parameters determine which factor contributes the most early hypercapnia in DM1. We analyzed ventilation status monitoring, tests (including FVC, maximal voluntary [MVV], and inspiratory expiratory pressure), polysomnography subjects DM1...

Mechanical insufflation-exsufflation has been reported to decrease pneumonia rates by about 90% for patients with Duchenne muscular dystrophy now living into their 40s and 50s without tracheotomy tubes. It greatly reduces respiratory complications hospitalization less than one per 10 patient-years advanced spinal atrophy type 1, through 25-30 years of age. is most successful from the point at which small children become able cooperate it, generally 3 5 However, since 1950s, use extubate...

Purpose: Duchenne muscular dystrophy (DMD) and Becker (BMD) are similar genetic disorders whose patterns of mutation disease phenotypes might be expected to show differences among different countries.We analyzed multiplex ligation-dependent probe amplification (MLPA) data in a large number Korean patients with DMD/BMD.Materials Methods: We obtained 130 positive MLPA results (86 DMD, 27 BMD, 17 female carriers) from 272 candidates (237 clinically suspected 35 possible who took part this...

Objective To reveal the significance of continuous transcutaneous carbon dioxide (CO2) level monitoring through reviewing cases which showed a discrepancy in CO2 levels between arterial blood gas analysis (ABGA) and monitoring. Method Medical record review was conducted retrospectively patients with neuromuscular diseases who had started home mechanical ventilation June 2008 May 2010. The 89 underwent ABGA at 1st hospital day, changes to their were continuously monitored overnight device....

<h3>INTRODUCTION:</h3> The purpose of this study was to investigate the 5-year outcomes noninvasive ventilation (NIV) application in different neuromuscular disease (NMD) groups. <h3>METHODS:</h3> We categorized 180 subjects who had initiated NIV between March 2001 and August 2009 into 4 groups followed them for &gt; 5 y. maintenance rate average duration, applying time, forced vital capacity (FVC) were investigated at time y after initiation each group. <h3>RESULTS:</h3> In with amyotrophic...

Dystrophinopathy is a group of inherited phenotypes arising from pathogenic variants in DMD . We evaluated the clinical and genetic characteristics Korean patients with genetically confirmed dystrophinopathy. retrospectively reviewed medical records (January 2004-September 2020) myopathy database maintained at study hospital found 227 218 unrelated families Clinical included 120 (53%) Duchenne muscular dystrophy (DMD) cases, 20 (9%) intermediate phenotype (IMD) 65 (29%) Becker (BMD) 18 (8%)...

Freeman-Sheldon syndrome (FSS) is a rare distal arthrogryposis syndrome. There are few reports on the respiratory insufficiency of FSS. Additionally, there no detailed information pulmonary functional evaluation. A 17-year-old male patient with FSS developed failure, leading him to be admitted hospital several times for evaluation and treatment. Of those he was admitted, two were due pneumonia. His functions indicative restrictive lung disease potentially caused by severe scoliosis. After...