Myasthenia gravis is a functional neuromuscular disorder with characteristic voluntary muscle weakness. The role of thymus in pathogenesis of this disorder has become apparent that thymectomy in treatment has gained acceptance. Between January 1976 and June 1987, twenty patients underwent thymectomy for myasthenia gravis at the Department of Thoracic and Cardiovascular Surgery, College of Medicine, Korea University. A clinical study that is focused on the factors affecting the operative results was analyzed and the following results were obtained. Among the 20 patients, male to female ratio was 8:12 and the age of onset was ranged from 3 years to 67 years. The chief complaints in order of frequency were as follows; ocular symptoms such as ptosis and diplopia[7 cases], general weakness[4 cases], swallowing difficulty[3 cases], dyspnea[3 cases], dysphasia[1 case], headache[1 case] and dizziness[1 case]. The severity of disease was classified by modified Osserman`s method that Group IIa was 8 cases, Group IIb; 7 cases, Group IIc; 3 cases and Group I; 2 cases. In histopathology of thymus, the most frequent finding was hyperplasia[11 cases] followed by thymoma[4 cases], normal tissue[3 cases] and malignant` thymoma[2 cases]. There were two cases of postoperative complications; one case was wound infection and the other was mediastinitis. One case of malignant thymoma died due to respiratory failure with pulmonary metastasis. There was 16 cases[80%] of improvement after thymectomy as follows; complete remission was 4 cases[20%], marked improvement was 9 cases[45%] and subjective improvement only was 3 cases[15%]. The effect of severity and duration of disease on operative result has statistically significant. The effect of thymus histopathology on operative result was not statistically significant. But there were comparable results between thymoma cases and non-thymoma cases.
Spinal muscular atrophy (SMA) is the second most common fatal disease of childhood with autosomal dominant mode of inheritance, and in its less severe form the third most common neuromuscular disease of childhood after Duchenne muscular dystrophy. The genetic defect was found to be on the long arm of chromosome 5 (5q11.2-q13.3) where many genes and microsatellite markers were missing. One of the most important genes is the Survival Motor Neuron (SMN) gene which is homozygously missing in 90% of SMA patients. Another important gene, the Neuronal Apoptosis Inhibitory Protein (NAIP) gene was found to be defective in 67% of SMA type I patients. Studies so far suggest SMA occurs when the genes on the long arm of chromosome 5 are mutated or deleted. Recently our hospital encountered 2 SMA patients of type I and II respectively. These patients both had homozygously defective SMN genes but intact NAIP genes. We are reporting these cases with bibliographic review and discussion. Korean SMA patients presumably have defects in SMN genes similar to those found in European patients, although the significance of NAIP genes remains to be established. SMN gene defects can be easily diagnosed using PCR and restriction enzymes, and this method could be applied towards convenient prenatal diagnosis and towards screening for family members at risk.
Purpose: The purpose of this study was to investigate the effect of chest mobilization and stretching exercises on maximal inspiratory pressure and maximal expiratory pressure in healthy adults who use computers for extended periods of time each day due to coronavirus disease 2019. Methods: Twenty-five healthy adults in their 20s and without respiratory disease (15 female, 10 male) took part in this study. Two types of thoracic mobilizing exercises using a Theraband and three types of stretching exercises using a foam roller were performed. Maximum inspiratory pressure and maximum expiratory pressure were measured three times each before and after the interventions. In terms of statistical methods, the maximum inspiratory pressure due to chest mobility and stretching was compared with the maximum expiratory pressure using parametric paired t-test and non-parametric Wilcoxon signed-rank test. Results: Maximum inspiratory pressure (p = .012) and maximum expiratory pressure (p = .006) showed significant differences before and after chest mobilization exercise and stretching among the participants. Conclusion: The results of this study suggest that chest mobilization and stretching exercises are effective exercise methods for improving maximal inspiratory and expiratory pressure. They suggest that these exercises can prevent respiratory muscle weakness and improve aerobic fitness in healthy people as well as those in need of cardiorespiratory physiotherapy.
This study investigated the effect of community-based health intervention program to improve metabolic disease in Jeju island. There were 50 obese local residents in the experimental group (body mass index, $BMI{\geq}25kg/m^2$). They participated in cooking therapy with nutrition education (4 times), exercise program with proprioceptive neuromuscular facilitation (PNF) and stretching training (9 times), and alternative medicine program with healing touch massage (3 times) during a 3-week period. To evaluate the effectiveness of the program, body composition, blood lipid profiles, blood glucose, and waist circumference were assessed before and after the intervention program. After the program, the value of total cholesterol (TC), glycosylated hemoglobin (HbA1C), diastolic blood pressure, percent body fat, and waist circumference were decreased, and became the normal value. Especially, HbA1C, percent body fat, and waist circumference were significantly decreased (P<0.001) in the experimental group, while HbA1C, percent body fat, and waist circumference were significantly increased (P<0.001) in the control group (N=50), who had no intervention. Our results suggest that metabolic syndrome associated markers need intervention program for improving them. In conclusion, although this study did not analyze the effect of the health intervention program and cooking therapy separately, considering the result of this 3-week, short term program, the effect will be more profuse if cooking therapy and exercise program were performed concurrently.
The Journal of Korea Assosiation for Disability and Oral Health
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v.15
no.1
/
pp.50-54
/
2019
Muscular dystrophy (MD) is a heterogeneous group of inherited neuromuscular disorders, characterized by progressive muscle weakness. Severity of the disease ranges from mild to severe, and the disease is mostly caused by mutations in a number of genes. These genetic mutations cause lack of proteins which are essential for muscle cell stability. Muscle fibers are gradually replaced by fat and fibrous tissue. The muscles of the head and neck are affected in several types of MD that manifest as altered craniofacial morphology and dental malocclusion. A 3-year-10-month old, 15.0 kg boy with MD presented to Seoul National University Dental Hospital, Seoul, South Korea because of extensive carious teeth. A number of dental caries in primary dentition were identified during clinical oral examination. Due to dental anxiety and underlying systemic disease, general anesthesia was considered. General anesthesia was induced and maintained with intravenous anesthetics, propofol and remifentanil. Caries treatments - resin restoration, pulpectomy, zirconia crown restoration, stainless steel crown restoration - were performed. Under general anesthesia, successful dental procedure was done. Total intravenous anesthesia (TIVA) was performed instead of inhalation anesthesia in order to avoid risk of complications such as malignant hyperthermia and life-threatening rhabdomyolysis. With decreasing muscle function, plaque control becomes more difficult and leads to gingivitis. Especially, the open-mouth posture worsens gingivitis and can leads to malocclusions and problems in swallowing. Regular and periodic dental care is essential for maintaining oral health for patients with MD.
Journal of the Korean Academy of Clinical Electrophysiology
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v.5
no.2
/
pp.35-45
/
2007
The purpose of this study was to examine spiral way movement of a trunk exerts on the movement ability. The details established to achieve for this article. This examination confirmed the weight, weight/height2 index, ratio of lumbar to pelvic, musculoskeletal quantity, push up for 2 minute, pitch a ball and voluntary isometric contraction with flexion and extension of knee joint of the subjects with spiral direct movement. Healthy eighteen subjects who understand fully the significance of procedure, consented to a plan, without neuromuscular disease were participated in two groups of experiment. The group were a spiral movement(9), rectilinear movement(9). Trunk movement tested 2 sessions of a spiral movement and rectilinear movement with a push up for 2 minute, 5days per a week, for the 4 weeks. This experiment tested 3 times with a sufficient rest for fatigue limitation. An analysis of the results used a paired samples t-test for difference from before and after experiment. The following results were obtained; At an internal change of the body, the musculoskeletal quantity was increased significantly to spiral movement group, but the weight was increased significantly, the musculoskeletal quantity was not significant to rectilinear movement. The movement ability evaluation for a external change was increased significantly in a push up for 2 minute, pitch a ball, isometric contraction with extension of knee joint of a spiral movement group, but a push up for 2 minute was increased significantly in a push up for 2 minute on the abdominal muscle training of a rectilinear movement group. As compared with a rectilinear movement, a spiral movement was more effect by cooperation with nerve and musculoskeletal system and an increase in movement ability was caused by learning acknowledgment, muscular reeducation. These results lead us to the conclusion that a spiral movement of trunk was more effect than a rectilinear movement, the coordination of nerve and musculoskeletal system was of importance of Multi-direction movement. Therefore, A further studies concerning the therapeutic exercise intervention and active-dynamic analysis could enhance the development of the most effect on the trunk.
Purpose: Respiratory is an essential vital component for conservation of life in human, which is controlled by respiratory muscles and its related neuromuscular regulation. The purpose of this study is to assess lung capacity and respiratory pressure in healthy children, and to investigate relationship and predictability between respiratory pressure and other related respiratory functions. Methods: A total of 31 healthy children were recruited for this study. Demographic information and respiratory related factors were assessed in terms of body surface area (BSA), chest mobility, lung capacity, and respiratory pressure. Correlation between respiratory pressure and the rested variables was analyzed, and multiple regression using the stepwise method was performed for prediction of respiratory muscle strength, in terms of respiratory pressure as the dependent variable, and demographic and other respiratory variables as the independent variable. Results: According to the results of correlation analysis, respiratory pressure showed significant correlation with age (r=0.62, p<0.01), BSA (r=0.80, p<0.01), FVC (r=0.80, p<0.01), and FEV1 (r=0.70, p<0.01). In results of multiple regression analysis using the backward elimination method, BSA and FVC were included as significant factors of the predictable statistical model. The statistical model showed a significant explanation power of 71.8%. Conclusion: These findings suggest that respiratory pressure could be a valuable measurement tool for evaluation of respiratory function, because of significant relationship with physical characteristics and lung capacity, and that BSA and FVC could be possible predictable factors to explain the degree of respiratory pressure. These findings will provide useful information for clinical assessment and treatment in healthy children as well as those with pulmonary disease.
Purpose: The Oral-anal Transit Test (OTT) is a simple method of obtaining information about colonic transit. We aim to assess the correlation of OTT with the neuromuscular integrity of the colon determined by colonic manometry (CM). Methods: All patients who had OTT followed by CM were evaluated. Less than 6 of 24 markers remaining on OTT was considered normal. CM was performed per previously published guidelines. A normal CM was defined as at least one High Amplitude Propagating Contraction progressing from the most proximal sensor through the sigmoid colon. Results: A total of 34 patients underwent both OTT and CM (44% male, age 4-18 years, mean 11.5 years, 97% functional constipation +/- soiling, Hirschsprung's Disease). Of normal and abnormal OTT patients, 85.7% (6/7) and 18.5% (5/27) respectively had normal CM. When all markers progressed to at least the sigmoid colon, this was 100% predictive against colonic inertia. Greater than 50% of patients with manometric isolated sigmoid dysfunction had markers proximal to the recto-sigmoid. Conclusion: OTT and CM are both valuable studies that assess different aspects of colonic function. OTT can be used as a screening test to rule out colonic inertia. However, the most proximal extent of remaining markers does not predict the anatomical extent of the manometric abnormality, particularly in isolated sigmoid dysfunction.
Journal of The Korean Dental Society of Anesthesiology
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v.2
no.2
s.3
/
pp.101-106
/
2002
Background: The management of the behavior of handicapped children when providing required dental care is often a problem, whether in the dental office or in a hospital setting. Because of the high incidence of poor cooperation, many of these patients are scheduled for dental care under general anesthesia with preoperative medical assessment. The purpose of this study was to carry out a clinico-statistical survey on dental treatment for handicapped children under general anesthesia. Methods: After approval from the institutional review board, the medical records of 64 handicapped children between 1997 and 2002 were reviewed to determine the patient profiles, anesthesia management, and complications. The charts of these patients, who underwent dental examination, scaling and prophylaxis, and restoration and extraction of teeth under general anesthesia, were reviewed. Results: The mean age was 12.8 years old, and males (53%) predominated females (47%). Twenty-four patients had mental retardation, twelve had autism, six had cerebral palsy, 4 had behavior disorder, others had heart disease, convulsive disorder, etc. Sixty-two had intravenous thiopental with neuromuscular blocker, 2 had intravenous ketamine induction. Nasotracheal intubation was uneventful in 55 patients, nine had orotracheal intubation because of difficult visualization of the larynx. Twenty-one patients experienced postoperative complications in the recovery room, including epistaxis, nasal obstruction, vomiting, airway obstruction, respiratory depression. Conclusions: General anesthesia is a very effective way of completing the dental treatments for disabled children. We emphasize the need to train anesthesiologists in the care of disabled patients.
Kim Bong-Ok;Chae Su-Sung;Kim Yong-Gun;Han Dong-Uck
The Journal of Korean Physical Therapy
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v.11
no.2
/
pp.43-50
/
1999
The purpose of this study was to evaluate the change of the energy consumption when loading to leg of the 60persons who don't have past history of cardiopulmonary and neuromuscular disease, To evaluate the change or energy consumption, heart rate was measured in sitting position for 5minute, during walking for 3minute at for 4.8km on treadmill, and during resting state after walking with 1Kg loading to right ankle, and the other 1Kg loading was added to left ankle and then heart rates were measured in the The results were as follow; 1. PCI value without loading to Ankle were significantly increased compared to 1Kg, and 2Kg. (p<0.05) 2. Female Subjects showed mon increased PCI value in without leading and 2Kg loading compared to male subjects. ( p<0.05) 3. When 1Kg ana 2Ka loading to ankle significantly differences were showed between them. (p<0.05) 4. In the case of 1Kg and 2Kg loading, the difference among age groups was observed and the significant difference among PCI, PCI 1kg, PCI 2kg was showed in the only group that is less than 30 years old. 5. In every PCI condition the difference among height groups was observed and the significant difference among PCI conditions was showed in the only group that is less than 165cm. 6. The difference among weight groups in each PCI condition was not observed, but the significant differences among PCI conditions was showed in every group except the group that h from 60kg to 69kg. These results showed that energy consumption was increased according to loading on the ankle during Sate so weight of orthosis or prosthesis met be considered when choosing them and during gait training with these ones.
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