In Vivo Reprogramming Using Yamanaka Factors in the CNS: A Scoping Review.

Central nervous system diseases, particularly neurodegenerative disorders, pose significant challenges in medicine. These conditions, characterized by progressive neuronal loss, have remained largely incurable, exacting a heavy toll on individuals and society. In recent years, in vivo reprogramming using Yamanaka factors has emerged as a promising approach for central nervous system regeneration.

Effects of Modified Diaphragmatic Training on Gastroesophageal Reflux Disease Questionnaire Score, Diaphragmatic Excursion, and Maximum Inspiratory Pressure in Adults with Gastroesophageal Reflux Disease After COVID-19: A Single-Blinded Randomized Control.

Background: Although diaphragmatic training has been shown to improve gastroesophageal reflux disease (GERD) symptoms, its effectiveness in adults with GERD after COVID-19 has not been evaluated. This study examined the effectiveness of modified diaphragmatic training (MDT) on GERD questionnaire (GERDQ) score, diaphragmatic excursion, and maximum inspiratory pressure (MIP) in adults with GERD after COVID-19.

Methods: This single-blinded randomized control trial was conducted at Persahabatan Hospital from February to April 2023.

Mechanical Insufflation-Exsufflation: The Rest of the Story.

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 and hospitalization rates to less than one per 10 patient-years for advanced spinal muscular atrophy type 1, through 25-30 years of age. It is most successful from the point at which small children become able to cooperate with it, generally from 3 to 5 years of age.

Mobile Apps to Improve Brace-Wearing Compliance in Patients with Idiopathic Scoliosis: A Quality Analysis, Functionality Review and Future Directions.

This study was performed to review which mHealth apps that improve brace-wearing compliance are currently available, and to carry out their quality assessments by listing their functionalities. We found ten mHealth apps in the literature review and commercial mHealth apps market (Google Play and App store). Then, the quality of these apps was evaluated by their transparency, health content, excellent technical content, security/privacy, issues of usability, and subjective ratings (THESIS) scale, and the functionalities of the included apps were reviewed.

Standardization of Air Stacking as Lung Expansion Therapy for Patients With Restrictive Lung Disease: A Pilot Study.

Objective: Although air stacking is a widely used lung expansion therapy essential for restrictive lung diseases, important details such as peak insufflation pressure (PIP) and number of squeezes have not been investigated. The purpose of this study was to standardize a method of air stacking to minimize problems with its application by identifying the optimal pressure and number of squeezes performed by professional physicians and investigating the current status of routine air stacking implementation in patients.

Methods: This prospective cross-sectional test-retest study involved individuals who had neuromuscular disorders and had performed air stacking exercise for longer than 1 year.

Pulmonary rehabilitation in high cervical spinal cord injury: a series of 133 consecutive cases.

Study Design: This is a retrospective study.

Objectives: To detail respiratory management after a high cervical spinal cord injury (HCSCI).

Setting: A tertiary university hospital's pulmonary rehabilitation center to which most individuals with HCSCI and ventilatory insufficiency throughout Korea are referred.

Clinical Implications of Routine Monitoring of Pulmonary Function and Ventilation in Patients with Duchenne Muscular Dystrophy.

Purpose: To investigate the effect of regular monitoring of pulmonary function and ventilatory status on the initiation of non-invasive ventilation (NIV) between patients who were routinely monitored before receiving NIV and those who were not.

Materials And Methods: This retrospective cohort study included subjects with Duchenne muscular dystrophy (DMD) who first received NIV between 2010 and 2019. The subjects were assigned to either the regular-follow-up (REG) group or the non-REG group, according to their follow-up status, before initiating NIV.

The association between cardiac involvement and long-term clinical outcomes in patients with Duchenne muscular dystrophy.

Aims: Despite advances in contemporary cardiopulmonary therapies, cardiomyopathy remains the leading cause of death in patients with Duchenne muscular dystrophy (DMD). Also, the long-term clinical outcomes of patients with DMD and cardiomyopathy is unknown. This study investigated long-term clinical outcomes and their associated factors in patients with late-stage DMD.

Clinical and genetic spectra in patients with dystrophinopathy in Korea: A single-center study.

Dystrophinopathy is a group of inherited phenotypes arising from pathogenic variants in DMD. We evaluated the clinical and genetic characteristics of Korean patients with genetically confirmed dystrophinopathy. We retrospectively reviewed medical records (January 2004-September 2020) from the myopathy database maintained at the study hospital and found 227 patients from 218 unrelated families with dystrophinopathy.

Clinical Course of Amyotrophic Lateral Sclerosis according to Initial Symptoms: An Analysis of 500 Cases.

Purpose: One obstacle in early diagnosis of amyotrophic lateral sclerosis (ALS) is its vague initial presentation, which is generally classified into limb- and bulbar-dominant types and may be mistaken for other musculoskeletal conditions. We analyzed clinical data from patients in relation to their initial presentation and prognosis from symptom onset to diagnosis.

Materials And Methods: We retrospectively analyzed the medical records of patients with ALS who were admitted for pulmonary rehabilitation between January 2007 and December 2019.

Analysis of Pneumothorax in Noninvasive Ventilator Users With Duchenne Muscular Dystrophy.

Background: With the advancement of cardiorespiratory interventions, the survival rate among patients with Duchenne muscular dystrophy (DMD) has increased. Subsequently, pneumothorax has become a significant problem in patients with prolonged ventilatory support.

Research Question: What are the frequency, recurrence rate, risk factors, and prognosis of pneumothorax in patients with DMD requiring noninvasive ventilation (NIV)? Also, are there known risk factors of pneumothorax on chest CT scans?

Study Design And Methods: This retrospective longitudinal cohort study included 176 patients treated between 2006 and 2019.

Safety of Gastrostomy Tube Placement in Patients with Advanced Amyotrophic Lateral Sclerosis With Noninvasive Ventilation.

Background: In patients with amyotrophic lateral sclerosis (ALS), bulbar muscle dysfunction can occur, which eventually requires the initiation of enteral tube feeding. However, there is no consensus on the optimal timing for the gastrostomy or the proper ventilator support method during the procedure. We aimed to investigate the safe range of gastrostomy according to respiratory support status and forced vital capacity (FVC) % of predicted values classification and to compare the safety of noninvasive and invasive mechanical ventilation during the procedure in ALS patients with FVC < 30% of predicted.

Precise Pulmonary Function Evaluation and Management of a Patient With Freeman-Sheldon Syndrome Associated With Recurrent Pneumonia and Chronic Respiratory Insufficiency.

Freeman-Sheldon syndrome (FSS) is a rare distal arthrogryposis syndrome. There are few reports on the respiratory insufficiency of FSS. Additionally, there is no detailed information on pulmonary functional evaluation.

Correlation of Bone Mineral Density with Pulmonary Function in Advanced Duchenne Muscular Dystrophy.

Background: A relationship between bone mineral density (BMD) and physical function has been revealed in the general population and various diseases. However, there is a lack of research investigating the correlation between BMD and respiratory function, one of few measurable physical parameters in patients with advanced Duchenne muscular dystrophy (DMD).

Objective: To determine whether pulmonary function parameters, including respiratory muscle strength, are related to BMD.

Relationship between Eating and Digestive Symptoms and Respiratory Function in Advanced Duchenne Muscular Dystrophy Patients.

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 and various eating and digestion related symptoms in patients with advanced Duchenne muscular dystrophy (DMD).

Methods: Eating and digestive symptoms, including loss of appetite, nausea, vomiting, diarrhea, constipation, swallowing difficulty, mastication difficulty, early satiety, and aspiration, were evaluated among patients with advanced DMD who were nonambulatory and required noninvasive mechanical ventilatory support.

Clinical implication of maximal voluntary ventilation in myotonic muscular dystrophy.

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

Improvement of Peak Cough Flow After the Application of a Mechanical In-exsufflator in Patients With Neuromuscular Disease and Pneumonia: A Pilot Study.

Objective: To investigate and demonstrate persistent increase of peak cough flow after mechanical in-exsufflator application, in patients with neuromuscular diseases and pneumonia.

Methods: A mechanical in-exsufflator was applied with patients in an upright or semi-upright sitting position (pressure setting, +40 and -40 cmH2O; in-exsufflation times, 2-3 and 1-2 seconds, respectively). Patients underwent five cycles, with 20-30 second intervals to prevent hyperventilation.

Epidemiology and diagnostic process of amyotrophic lateral sclerosis as distinct from myelopathy: 5-year cohort study of whole-population in South Korea.

Objective: To investigate the incidence, prevalence, and demographic factors of all amyotrophic lateral sclerosis (ALS) patients diagnosed in South Korea from 2011 to 2015, and to analyze cases misdiagnosed as myelopathy.

Methods: The whole population registered under the Korean National Health Insurance Service (KNHIS) was applied. All 4551 patients who were registered as having ALS code from 2011 to 2015 were included.

Remarkable improvement in a very severe chronic obstructive pulmonary disorder patient after use of noninvasive intermittent positive pressure ventilator: A case report.

Rational: Chronic obstructive pulmonary disease (COPD) impairs lung function and induces systemic effects, resulting in impaired quality of life. Skeletal muscle dysfunction-characteristic of advanced COPD patients-limits a patient's ability to perform activities of daily living (ADL). In addition, dysphagia is commonly observed in COPD patients.

Can radial bone mineral density predict spinal bone mineral density in patients with advanced Duchenne muscular dystrophy?

In advanced Duchenne muscular dystrophy (DMD), patients with high bone fracture risk due to osteoporosis, it is difficult to measure spinal bone mineral density (BMD) because of maintaining proper posture. This study began with the idea that if we diagnose and manage osteoporosis by predicting spinal BMD through easily testable radial BMD, we could prevent fracture and improve quality of life in DMD patients. In 61 DMD patients aged 20 years or older who were admitted to Gangnam Severance Hospital from April 2013 to May 2015, radial BMD and spinal BMD were measured to compare their Z-scores.

Long-Term Outcome of Amyotrophic Lateral Sclerosis in Korean Subjects.

Objective: To report the latest long-term outcome of amyotrophic lateral sclerosis (ALS) and to analyze the predictors of prognosis.

Methods: Subjects who were diagnosed with ALS between January 2005 and December 2009 at a single institute were followed up until death or up to December 2014. Data regarding age, sex, date of onset, date of diagnosis, presence of bulbar symptoms on onset, date of initiation of non-invasive ventilation (NIV), and the date of tracheostomy were collected.

Five-Year Follow-Up and Outcomes of Noninvasive Ventilation in Subjects With Neuromuscular Diseases.

Introduction: The purpose of this study was to investigate the 5-year outcomes of noninvasive ventilation (NIV) application in different neuromuscular disease (NMD) groups.

Methods: We categorized 180 subjects who had initiated NIV between March 2001 and August 2009 into 4 groups and followed them for > 5 y. The NIV maintenance rate and average duration, applying time, and forced vital capacity (FVC) were investigated at the time NIV was initiated and 5 y after NIV initiation in each group.