• Title/Summary/Keyword: Neuromuscular disease

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DMSO Improves Motor Function and Survival in the Transgenic SOD1-G93AMouse Model of Amyotrophic Lateral Sclerosis (DMSO 투여된 근위축성 측삭경화증 SOD1-G93A 형질 변환 마우스 모델에서의 근육 기능과 생존 기간 증가 효과)

  • Park, Kyung-Ho;Kim, Yeon-Gyeong;Park, Hyun Woo;Lee, Hee Young;Lee, Jeong Hoon;Patrick, Sweeney;Park, Larry Chong;Park, Jin-Kyu
    • Journal of Life Science
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    • v.32 no.8
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    • pp.611-621
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    • 2022
  • Dimethyl sulfoxide (DMSO) is commonly used as control or vehicle solvent in preclinical research of neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) due to its ability to dissolve lipophilic compounds and cross the blood brain barrier. However, the biochemical effects of DMSO on the outcomes of preclinical research are often overlooked. In the present study, we investigated whether the long-term oral administration of 5% DMSO affects the neurological, functional, and histological disease phenotype of the copper/zinc superoxide dismutase glycine 93 to alanine mutation (SOD1-G93A) mouse model of amyotrophic lateral sclerosis. SOD1-G93A transgenic mice showed shortened survival time and reduced motor function. We found that administration with DMSO led to increased mean survival time, reduced neurological scores, and improved motor performance tested using the rotarod and grip strength tests. On the other hand, DMSO treatment did not attenuate motor neuron loss in the spinal cord and denervation of neuromuscular junctions in the skeletal muscle. These results suggest that DMSO administration could improve the quality of life of the SOD1-G93A mouse model of ALS without affecting motor neuron denervation. In conclusion, the use of DMSO as control or vehicle solvent in preclinical research may affect the behavioral outcomes in the SOD1-G93A mouse model. The effect of the vehicle should be thoroughly considered when interpreting therapeutic efficacy of candidate drugs in preclinical research.

Elderly Sarcopenia and Vitamin B Deficiency: A Relationship? (비타민 B 결핍에 의한 노인성 근감소증)

  • Kisang Kwon;Hye-Jeong Jang;Sun-Nyoung Yu;Soon-Cheol Ahn;O-Yu Kwon
    • Journal of Life Science
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    • v.33 no.7
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    • pp.574-585
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    • 2023
  • Sarcopenia is a leading cause of increased medical and nursing care costs among the elderly. In Korea, preventive measures for sarcopenia are mostly targeted toward the general elderly population without specific diseases. However, it is also necessary to implement measures for elderly individuals living in nursing homes and hospitals, where the prevalence of sarcopenia is high. Currently, computed tomography and/or magnetic resonance imaging are considered standard diagnostic tools. However, their complexity and time-consuming nature make them unsuitable for clinical use. The exact pathophysiological mechanisms of sarcopenia are unclear, as they involve various molecular biological pathways, including decreased exercise, protein and nutrient intake, changes in testosterone and growth hormone, and inflammation. Sarcopenia symptoms can lead to several diseases, such as osteoporosis, fractures, dementia, diabetes, and cardiovascular disease. Vitamin B deficiency is a significant factor in sarcopenia induction, with B vitamins being directly involved in energy and protein metabolism and nerve function. Vitamin B deficiency can lead to neuromuscular and neurogenic disorders, which often overlap with sarcopenia. Suboptimal intake of B vitamins, malabsorption, and anorexia are common among the elderly. This study aims to provide information on the role of water-soluble B vitamins in preventing and controlling muscle mass loss and deterioration among the elderly with sarcopenia. In addition, we discuss the potential of myokines from the B vitamin family in modulating sarcopenia.

The Effect of Nasal BiPAP Ventilation in Acute Exacerbation of Chronic Obstructive Airway Disease (만성 기도폐쇄환자에서 급성 호흡 부전시 BiPAP 환기법의 치료 효과)

  • Cho, Young-Bok;Kim, Ki-Beom;Lee, Hak-Jun;Chung, Jin-Hong;Lee, Kwan-Ho;Lee, Hyun-Woo
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.2
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    • pp.190-200
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    • 1996
  • Background : Mechanical ventilation constitutes the last therapeutic method for acute respiratory failure when oxygen therapy and medical treatment fail to improve the respiratory status of the patient. This invasive ventilation, classically administered by endotracheal intubation or by tracheostomy, is associated with significant mortality and morbidity. Consequently, any less invasive method able to avoid the use of endotracheal ventilation would appear to be useful in high risk patient. Over recent years, the efficacy of nasal mask ventilation has been demonstrated in the treatment of chronic restrictive respiratory failure, particularly in patients with neuromuscular diseases. More recently, this method has been successfully used in the treatment of acute respiratory failure due to parenchymal disease. Method : We assessed the efficacy of Bilevel positive airway pressure(BiPAP) in the treatment of acute exacerbation of chronic obstructive pulmonary disease(COPD). This study prospectively evaluated the clinical effectiveness of a treatment schedule with positive pressure ventilation via nasal mask(Respironics BiPAP device) in 22 patients with acute exacerbations of COPD. Eleven patients with acute exacerbations of COPD were treated with nasal pressure support ventilation delivered via a nasal ventilatory support system plus standard treatment for 3 consecutive days. An additional 11 control patients were treated only with standard treatment. The standard treatment consisted of medical and oxygen therapy. The nasal BiPAP was delivered by a pressure support ventilator in spontaneous timed mode and at an inspiratory positive airway pressure $6-8cmH_2O$ and an expiratory positive airway pressure $3-4cmH_2O$. Patients were evaluated with physical examination(respiratory rate), modified Borg scale and arterial blood gas before and after the acute therapeutic intervention. Results : Pretreatment and after 3 days of treatment, mean $PaO_2$ was 56.3mmHg and 79.1mmHg (p<0.05) in BiPAP group and 56.9mmHg and 70.2mmHg (p<0.05) in conventional treatment (CT) group and $PaCO_2$ was 63.9mmHg and 56.9mmHg (p<0.05) in BiPAP group and 53mmHg and 52.8mmHg in CT group respectively. pH was 7.36 and 7.41 (p<0.05) in BiPAP group and 7.37 and 7.38 in cr group respectively. Pretreatment and after treatment, mean respiratory rate was 28 and 23 beats/min in BiPAP group and 25 and 20 beats/min in CT group respectively. Borg scale was 7.6 and 4.7 in BiPAP group and 6.4 and 3.8 in CT group respectively. There were significant differences between the two groups in changes of mean $PaO_2$, $PaCO_2$ and pH respectively. Conclusion: We conclude that short-term nasal pressure-support ventilation delivered via nasal BiPAP in the treatment of acute exacerbation of COPD, is an efficient mode of assisted ventilation for improving blood gas values and dyspnea sensation and may reduce the need for endotracheal intubation with mechanical ventilation.

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Clinical Study of Children Using Home Mechanical Ventilation (가정용 인공 호흡기를 사용하는 소아의 임상적 고찰)

  • Ahn, Young Joon;Lee, Seung Hyeon;Kim, Hyo-Bin;Park, Seong Jong;Ko, Tae Sung;Hong, Soo Jong
    • Clinical and Experimental Pediatrics
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    • v.48 no.4
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    • pp.401-405
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    • 2005
  • Purpose : The use of mechanically-assisted ventilators at home reduces morbidity and improves the quality of life in children with chronic respiratory failure. But in Korea there is no clinical data of children with home mechanical ventilation. We investigated ventilator types, duration, the causes of failure or death, and the cost needed for care. Methods : We retrospectively analyzed the medical records of 21 children who were admitted and who applied for home mechanical ventilation at the Pediatric Intensive Care Unit in Asan Medical Center. Phone interviews took place after discharge. and interviewed by phone after discharge. Results : The median age was 31 months; the median duration with ventilator was 25 months. Underlying diseases were 16 neuromuscular diseases, one metabolic disease and four chronic respiratory diseases. The types of ventilator were pressure and volume type(16 and five patients, respectively). The frequency of ventilation failure was once per 19 months. Weaning could be performed in three cases. Frequencies of admission after receiving ventilators were 1.7 times per year; the most common cause was pneumonia. Nine patients(43%) died; four of them died because of endotracheal tube obstruction. The costs for medical care were about 1,110,000 won per month. Conclusion : There is an increment in the numbers of individuals who need mechanical ventilation support. The most common cause of death was endotracheal tube obstruction. The most important problem for the patients was medical cost. There needs to be more interest in patients with ventilator and social welfare systems to support their families need to be prepared.

Clinical Investigation about the Result of Surgically Treated Myasthenia Gravis (중증 근무력증의 수술적 치료결과에 대한 임상적 고찰)

  • 김대현;황은구;조규석;김범식;박주철
    • Journal of Chest Surgery
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    • v.36 no.1
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    • pp.15-20
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    • 2003
  • Myasthenia gravis is a rare autoimmune disease involving acetylcholine receptor and its autoantibody on neuromuscular junction. The methods of treatment are medical treatment and surgical thymectomy. In this paper we analyzed the result of thymectomy and the factors affecting the postoperative symptom improvement. Material and method : This study obtained medical records of 37 patients who received the thymectomy for myasthenia gravis from March 1986 to December 1998. Result Out of 37 cases, 21 cases(57%) showed improvement, of which 8 cases (50%) in the group of thymoma(n=16), and 13 cases (62%) in the group of thymic hyperplasia(n=21) showed the improvement of symptoms. Postoperative complications were respiratory insufficiency due to aggravation of symptoms after operation, including tracheal intubation for ventilator support in 9 cases, pneumonia in 3 cases, pneumothorax in 2 cases and left vocal cord palsy in 1 case. There was one postoperative mortality. The relation between postoperative improvement and sex(P=0.3222), age(P=0.7642), thymic pathologic variants,(P=0.4335) and classification of thymoma(P=0.20) showed no statistically significant correlation. However, the lower grade of preoperative symptoms can predict the lower grade of postoperative symptoms significantly(P=0.0032). Follow up study to 36 postoperative survivors was performed in October 2002 based on the out-patient records and call with patients. Out of 36 cases, 33 cases(91.7%) could be investigated and 3 cases could not. Mean follow up period was 83.2 months. Out of 33 cases, 25 cases(75.8%) showed symptomatic improvement, of which 8 cases(53.3%) in the group of thymoma(n=15) and 17 cases(94.4%) in the group of thymic hyperplasia(n=18) showed the improvement of$\boxUl$ symptoms. Conclusion : In myasthenia gravis, thymectomy showed the good improvement, and more important factor affecting the improvement of symptoms was the grdae of preoperative symptoms. Also midterm and long term follow up results showed good symptomatic improvement.

The Usefulness of Noninvasive Positive Pressure Ventilation as a New Weaning Method (새로운 이탈방법으로서 비침습적 양압환기법의 유용성)

  • Shim, Tae-Sun;Koh, Youn-Suck;Lee, Sang-Do;Kim, Woo-Sung;Kim, Dong-Soon;Kim, Won-Dong;Lim, Chae-Man
    • Tuberculosis and Respiratory Diseases
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    • v.46 no.4
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    • pp.500-511
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    • 1999
  • Background: Noninvasive positive pressure ventilation (NPPV) using facial or nasal mask have been widely used for several years in stable patients with chronic neuromuscular disease or central alveolar hypoventilation, and recently have been tried in patients with acute respiratory failure. In a few studies, NPPV was also used to rescue the patients with post-extubation respiratory failure. However, yet it has not been adopted as a weaning method in patients on long-term mechanical ventilation. So we performed this prospective clinical study to evaluate the usefulness of NPPV as a weaning method after removing endotracheal tube intentionally in patients on long-term mechanical ventilation. Method: Twelve patients who had been on invasive mechanical ventilation over 10 days were enrolled and 14 trials of NPPV were done. All had failed at least one weaning trial and showed ventilator dependence(pressure support requirement between 8-15cm $H_2O$, and PEEP requirement between 5-10cm $H_2O$), so tracheostomy was being considered. After removing the endotracheal tube, NPPV was applied using facial mask. Respiratory rate, arterial blood gas, pressure support level, and PEEP level were monitored just before intended extubation, at 30 minutes, 1 to 6, 6 to 12, 12 to 24 hours, 2nd day, and 3rd day following initiation of NPPV, and just before weaning from NPPV. The successful weaning was defined as spontaneous breathing off the ventilator for 48 hours or longer without respiratory distress. Results: The weaning through NPPV after intended extubation was successful in 7(50%) of 14 trials, and tracheostomy could be avoided in them. There were no differences in age, sex, APACHE III score, duration of invasive mechanical ventilation, baseline respiratory rate, $PaCO_2$ $PaO_2/FiO_2$, and ventilatory requirement(PS and PEEP) between the success and failure groups. In the success group, respiratory rate, pH, $PaCO_2$, and $PaO_2/FiO_2$ were not different between invasive MV and NPPV period. But in the failure group, pH decreased after 30 minutes of NPPV initiation compared with that of invasive MV($7.40\pm0.08$ vs. $7.34\pm0.06$, p<0.05). The causes of failure were worsening of ABG(n=3), retained tracheal secretion(n=2), mask intolerance(n=1), and flail chest(n=1). Conclusion: NPPV may be worth trying as a bridge method in weaning patients on long-term invasive mechanical ventilation.

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