• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.898-902
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• 2003

Purpose : To determine the clinical association of diagnostic criteria and the prevalence of autoantibodies in newly diagnosed children with juvenile dermatomyositis(JDM). Methods : Thirty-two children with JDM were identified at Seoul National University Children's Hospital from March 1985 to March 1999 by retrospective review. The diagnosis of JDM was based on the criteria proposed by Bohan and Peter. We investigated for the presence of several autoantibodies: antinuclear(ANA), double-stranded DNA, anti-Sm, anti-ribonucleoprotein(RNP), anti-SSA/ SSB, anti-Jo1, anti-Scl-70 antibodies and rheumatoid factor(RF). Results : Sex ratio and age at diagnosis were similar to data published in other studies. All the newly diagnosed children with JDM had a typical rash(100%) and proximal muscle weakness(100%); 17(53%) had muscle pain or tenderness; 10(31%) calcinosis; eight(25%) dysphagia; eight(25%) arthritis, and seven(22%) fever. Muscle enzymes were elevated in 90% of the patients. Of the 27 patients who had an electromyogram, 20(70%) had diagnostic results. Sixteen(70%) of biopsied patients had appropriated results for JDM. Patients were negative for all autoantibodies except ANA and RF. ANA and RF were detected in 47% and 7% of the patients respectively. Conclusion : Although the sensitivity of the criteria proposed by Bohan and Peter is superior, each of these criteria has possible confounding factors. Additional criteria may be needed for early diagnosis of JDM. Based on our findings of autoantibodies in JDM, we do not recommend routine testing for autoantibodies in children with typical JDM.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.93-101
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• 2016

Purpose: McArdle disease, glycogen storage disease type V (GSD V), is one of the most common adolescent-onset glycogen storage diseases. It is caused by recessive mutations in PYGM encoding myophosphorylase, which is critical to glycogen metabolism. Since only a few korean patients have been reported, we will observe the clinical and genetic features of three korean patients with McArdle disease. Methods: We retrospectively reviewed the medical records of three patients with genetically confirmed McArdle disease, including the results of forearm ischemic exercise test, electromyogram, nerve conduction velocity, muscle biopsy, and PYGM analysis in peripheral leukocytes. Results: All three cases were males and their age of symptom onset was 12, 5, 14 years old, respectively. A high basal level of serum creatine kinase was noted in all three patients. They experienced the recurrent episodes of rhabdomyolysis, but second wind phenomenon was not definite. In muscle biopsy, subsarcolemmal space vacuoles including periodic acid schiff stained materials were found in two patients, while no evidence of glycogen storage disease was found in the other. A total of five different mutations, $p.Arg50^*$, p.Trp798Arg, $p.Arg50^*$, p.Glu779del, $p.Asp511Thrfs^*28$ and p.Phe710del, were found in three patients. Avoidance of isometric exercise, aerobic exercise and glucose intake before each exercise were recommended for all patients. Conclusion: The three Korean patients with McArdle disease showed the typical manifestations of the condition. The most mutations were private. Therefore, identification of more cases with long-term follow-up will be required to understand the clinical and genetic features of this disease among Korean population.

• 한국노년학
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• v.29 no.4
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• pp.1247-1259
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• 2009

Muscle mass is reduced by aging. There seems to be no direct relationship between sarcopenia(muscle loss) and medical cost in the elderly, but lowering muscle mass results in increase risk of fall and decrease of strength, fitness, physical activity, and independent life. This is coupled with physical trouble and chronic degenerative disease such as diabetes, obesity, hyperlipidemia, and hypertension. Thus, sarcopenia is potential risk factor increasing mortality. The purpose of this study was to investigate the effects of elastic band exercise and detraining on sarcopenia prevention related variables, body composition and fitness. The subject of this study was 60~70 aged 14 seniors who participated in exercise program in J-welfare senior center at J-gu in S-city. Elastic band exercise was performed twice per week for 12 weeks. The body composition and fitness variables were measured before 12 weeks of control, after control(before exercise), after 12 weeks of exercise(before detraining), and after 12 weeks of detraining. There was no significant difference in body composition and fitness variables before and after 12 weeks of control, but elastic band exercise before and after 12 weeks has effect on body composition variables such as weight (t=2.978, p=0.001), body mass index (t=3.502, p=0.004), percent body fat (t=2.216, p=0.045), muscle mass (t=-3.837, p=0.002), visceral fat area (t=5.186, p<0.001), and waist-hip ratio (t=3.045, p=0.009) and on fitness variables such as 2-minutes step (t=-6.891 p<0.001), arm curl (t=-4.702, p<0.001), chair stand (t=-4.860, p<0.001), chair sit and reach (t=-5.910, p<0.001), back scratch (t=-3.835, p=0.002), and 8-ft up and go (t=7.560, p<0.001). This exercise effect was continued after 12 weeks of detraining on body composition variables such as weight (t=2.323, p=0.037), body mass index (t=2.503, p=0.026), muscle mass (t=-3.137, p=0.008) and on fitness variables such as 2-minutes step (t=-6.489 p<0.001), chair stand (t=-4.694, p<0.001), chair sit and reach (t=-3.690, p=0.003), and 8-ft up and go (t=7.539, p<0.001). It was found that the elastic band exercise has positive effect on body composition and fitness in the elderly and the effect was maintained over 12 weeks of detraining.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.8 no.2
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• pp.127-133
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• 2012

Ehlers-Danlos syndrome (EDS), an inherited connective tissue disorder, is caused by mutations in genes encoding different types of collagen or collagen-processing enzymes. EDS most typically affects the joints, ligaments, skin, and blood vessels. Oral health may be severely compromised in EDS as a result of specific alterations of collagen in orofacial structures. Dental hard tissue defects, root dilaceration, pulp stones, ectopic or delayed eruption, impaction, and periodontal disease could be observed. Therefore, a number of tissue responses related to collagen and precautions should be anticipated when considering dental treatment in EDS. Long-term and comprehensive dental management is required. The purpose of this report is to describe a clinical case of eruption disorders in a patient with EDS.

• Journal of Life Science
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• v.21 no.3
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• pp.400-405
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• 2011

Autophagy, a highly conserved mechanism of internal quality control, is essential for the maintenance of cellular homeostasis and for the orchestration of an efficient cellular response to stress. During aging, the efficiency of autophagic degradation declines and intracellular waste products accumulate. Therefore, the aim of this study is to investigate the effects of exercise on autophagic response in skeletal muscle. Twenty-four Young (4 month) and Old (12 month) ICR-type white male mice were divided into a control group (CON: n=6) and exercise training group (Tr: n=6) after an adaptation period of 1 week. Exercise consisted of treadmill running at 16.4 m/min with a 4% incline, 40 min/day and 5 days/week for 8 weeks. Cervical dislocation was performed at 48 hours after the last round of exercise, after which the gastrocnemius skeletal muscle were immediately collected. The results of verifying autophagy formation showed that the Sarcopenia index was decreased in the Old mice compared to the Young. However, it increased with exercise training in the Old. Lipidation LC3-II, Becline-1, and Atg7 were decreased in the Old mice compared to the Young. However, Lipidation LC3-II was significantly increased in the trained Old mice (Young:1 Vs Old:$1.32{\pm}0.042$, p<0.05). Based on these data, we suggest that autophagy regulatory events are the attenuated in Old mice, but that they are enhanced with exercise training.

• Journal of the Korean Chemical Society
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• v.63 no.6
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• pp.415-419
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• 2019

Fipronil is an insecticide that belongs to the N-phenylpytazole and has been used mainly for an insect pest control. However, it is known that acute poisoning of the human body causes various symptoms such as dizziness, muscle weakness, dyspnea, skin irritation, and increased heart rate. Lately, eggs containing fipronil have been distributed and toxic problems are spreading around the world. In this study, we tried to develop analytical methods to evaluate the exposure of fipronil and fipronil sulfone in animal serum samples. The differences according to mobile phase and the results of liquid - liquid extraction and solid phase extraction pretreatment method were compared. Distilled water (A) and acetonitrile (B) were selected for the mobile phase, and the pretreatment method was determined by solid phase extraction. As a result of the method validation, the intra-day / inter-day accuracies were 82.2~114.1% and the precisions were less than 20%. The detection limit was 0.027 ng/ml for fipronil and 0.087 ng/ml for fipronil sulfone. The linearity obtained was satisfying, with a coefficient of determination (r²) higher than 0.99. The concentrations in some animal sera were determined using the methods of analysis for fipronil and fipronil sulfone in animal sera developed in this study. Using the method developed in this study, it could be used as an analytical method for human bio-monitoring of fipronil and fipronil sulfone as well as animal serum.

• Journal of Korean Orthopaedic Sports Medicine
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• v.9 no.1
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• pp.7-15
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• 2010

Anterior knee pain syndrome would best be defined as a painful condition that arises in or around the patellofemoral joint and is insidious in onset and bilateral, with an enigmatic entity with multiple causes. Although its etiology is uncertain, the cause is often considered to be abnormal lower limb biomechanics, pathology of extensor mechanism, disorder of patellofemoral joint, malalignment or lateral tracking of the patella, soft tissue tightness, muscle weakness. The measurement of patellar alignment has come to be accepted as an integral part of the examination of anterior knee pain syndrome. Various measurement techniques exist, both clinical and radiological, and these have been frequently used in the diagnosis and treatment of the condition.?Treatment depends on the underlying cause of anterior knee pain and should be directed to the cause rather than to the results. Most often, this involves non-surgical measures, such as anti-inflammatory medications, quadriceps exercises, and hamstring stretching. Shin splint, or medial tibial stress syndrome refers a syndrome of pain running along the inner distal 2/3 of tibia shaft. Shin splint is a common problem for athletes whose sport involves a repeated, jarring impact to the leg. A major factor determining the efficacy of the treatment is that correct diagnosis be made of the problem. The varied etiology has led to the development of several theories as to the cause, treatment, rehabilitation and prevention of shin splint. The management is rest, ice massages, pain relief by medication, and muscle strengthening exercise. Proper rehabilitation and preventative measures can ensure that there is no further recurrence.

• Korean Journal of Food Science and Technology
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• v.49 no.6
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• pp.685-692
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• 2017

Mitochondrial biogenesis-a process that leads to an increment in the number and density of mitochondria, improves physical performance and body health by enhancing exercise capacity. In the present study, we investigated the stimulatory effect of Ashitaba and red ginseng complex (ARC) on exercise capacity in L6 skeletal muscle cells and mice. In L6 skeletal muscle cells, ARC increased the mitochondrial contents and ATP production by activating AMP-activated protein kinase (AMPK), sirtuin 1 (SIRT1), and peroxisome proliferator-activated receptor gamma coactivator-1 alpha (PGC-$1{\alpha}$) and up-regulating the mRNA expression of nuclear respiratory factor-1 (NRF-1) and mitochondrial transcription factor A (TFAM). In the animal experiments, mice treated with ARC showed an increment in exercise capacity as compared with mice treated with Ashitaba extract or red ginseng extract alone. These studies indicate that ARC might serve as a potential natural candidate for enhancing exercise capacity by stimulation of mitochondrial biogenesis.

• Journal of Digital Convergence
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• v.12 no.7
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• pp.365-370
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• 2014

The present study was aimed to comprehend the effect of the Russian current stimulation on the muscular performance and activity of quadriceps femoris muscle of the convalescent patient after the leg fracture.This study conducted test based on 20 patients after leg fracture. Russian current was applied for six weeks as three times in a week, between January 9, 2013 and 15 March 16, 2013. The electromyogram was used to measure the muscle activity of the quadriceps femoris, and the isokinetic equipment was used to measure the muscular performance. The muscular performance difference by duration depending on the application of the Russian current showed the significant increase in all muscular performances. The activity of all muscles of the quadriceps femoris muscle by duration showed significant increase. These results showed that the Russian current can be the effective treatment method for enhancing the muscular strength of the weakened lower limbs muscle due to the problem such as the leg fracture.

• Clinical and Experimental Pediatrics
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• v.50 no.2
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• pp.213-217
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• 2007

Pompe disease is a genetic disorder caused by a deficiency of acid ${\alpha}$-glucosidase (GAA). Infantile onset Pompe disease is uniformly lethal. Affected infants generally present in the first few months of life with hypotonia, generalized muscle weakness, and a hypertrophic cardiomyopathy, which is rapidly followed by death, usually by the age of one. The late-onset form is characterized less severe symptoms and prognosis. Therapy for Pompe disease is intended to directly address the underlying metabolic defect via intravenous infusions of recombinant human GAA to replace the missing enzyme. We report a case of atypical infantile-onset Pompe disease that presented symptoms in infancy but had less severe clinical manifestations and improved after GAA enzyme replacement ($Myozyme^{(R)}$, Genzyme Co., MA, USA) therapy. It is very important that pediatricians become aware of signs and symptoms of Pompe disease, such as a nasal voice or a waddling gait at an early stage so that these patients can benefit from appropriate GAA replacement therapy as soon as possible.