염증성 근병증에서 자기공명영상의 임상적 의의

고재현 ( Jae Hyun Koh ),홍석주 ( Suk Joo Hong ),안홍준 ( Hong Joon Ahn ),안중경 ( Joong Kyung Ahn ),전찬홍 ( Chan Hong Jeon ),차훈석 ( Hoon Suk Cha ),안중모 ( Joong Mo Ahn ),김현숙 ( Hyun Sook Kim ),서연림 ( Yeon Lim Seo ),고은미 대한류마티스학회 2002 대한류마티스학회지 Vol.9 No.3

Objective: The purpose of the study is to describe the magnetic resonance imaging (MRI) findings and to investigate useful sequences of MRI in inflammatory myopathies. A third goal is to correlate MRI findings with the grade of histopathologic severity. Methods: Sixteen patients (13 women, 3 men), aged 21~74 years (median age of 49) with inflammatory myositis (examined with both MRI and muscle biopsy) were studied. Ten patients were diagnosed with polymyositis and 6 with dermatomyositis. Conventional T1-weighted (SE 366/12) and T2-weighted (SE 4766/90) fast spin-echo and fat-suppressed T1-weighted MR images with contrast enhancement (FSAT T1 CE) were obtained. Ratios between the signal intensities of a muscle and the signal intensities of subcutaneous fat in the same tomographic sections were calculated to objectively represent the degree of signal intensities. Semi-quantitative grading of severities in muscle biopsy specimen was examined based on invasion of inflammatory cells and necrosis, degeneration and regeneration of muscle fiber by a neuromuscular pathologist. Results: FSAT T1 CE could objectively describe the severity of involvement in inflammatory myopathies. The quadriceps muscle group, especially the vastus muscle tended to be most severely and frequently involved in inflammatory myositis with FSAT T1 CE (statistically insignificant). The vastus intermedius muscle was more severely involved in dermatomyositis than polymyositis. The signal intensity of abnormal muscle sampled by biopsy correlated positively with the grade of muscle biopsy. Conclusion: MRI shows promise in identifying pathologic muscle in patients suspected of having one of the inflammatory myopathies. The degree of signal intensity on MRI may reflect the severity of grade in muscle biopsy.

High Grade Myxofibrosarcoma in the Paravertebral Muscle: A Case Report

남동혁,김태완,박관호,김재오 대한척추신경외과학회 2010 Neurospine Vol.7 No.4

Many different abnormalities, such as, neoplasm, infection, traumatic hematoma, or congenital or immune myopathy, may be found in the paravertebral muscles. However, neoplasms of paravertebral muscle are an uncommon cause of back pain. Such neoplasms may arise from local lesions or due to metastatic spread from a distant malignancy. The differential diag- noses of a primary soft tissue malignancy and metastatic spread from a skeletal muscle tumor are important. In cases of soft tissue sarcoma, histopathological findings and surgical margins are both related to local recurrence and metastasis, therefore, percutaneous needle biopsy may be helpful before surgical excision. A degree of surgical excision is decided based on considerations of muscular function and histopathological findings.

Intramuscular Hemangioma in the Anterior Scalene Muscle Diagnosed by Core Needle Biopsy

조재근,차원재,성명훈 대한이비인후과학회 2015 Clinical and Experimental Otorhinolaryngology Vol.8 No.3

Intramuscular hemangioma (IMH) is a rare, benign vascular lesion that frequently develops within skeletal muscles. Preoperatively, accurate diagnosis of IMH is often extremely difficult because of nonspecific clinical findings and the inaccuracy of fine-needle aspiration cytology. IMH is suspected in only 8% of preoperative diagnoses before surgical exploration. Here, we report a case of a 44-year-old man with a huge IMH in the anterior scalene muscle that was preoperatively diagnosed using ultrasonography-guided core needle biopsy, and was successfully treated based on preoperative clinical information.

12주간의 β-alanine 보충과 운동 훈련이 근육 내 Carnosine 농도와 운동수행능력에 미치는 영향

손희정 ( H. J. Son ),하영일 ( Y. I. Ha ),박노환 ( N. H. Park ),육현철 ( H. C. Youk ),김효정 ( H. J. Kim ),김창근 ( C. K. Kim ) 한국운동생리학회(구-한국운동과학회) 2010 운동과학 Vol.19 No.3

Carnosine(beta-alanyl-L-histidine)은 베타알라닌과 히스티딘으로 이루어진 다이펩다이드로서 근육 내에 많이 분포하고 있다. Carnosine은 체액의 산성화가 일어날 때 수소이온을 효과적으로 완충시키는 작용을 하며 식이를 통해 근육 내 carnosine 농도를 변화시킬 수 있다. 이 연구는 엘리트 수영선수(22-25세, n=10)를 대상으로 12주간 β-alanine 보충과 수영 훈련이 근육 내 carnosine 농도와 운동수행능력에 미치는 영향을 구명하였다. 집단은 β-alanine 보충 집단(n=5)과 위약 집단(n=5)으로 무선배정하였다. 12주간 β-alanine보충집단은 β-alanine을 3.2g/d씩 섭취하였으며 수영 훈련은 주당 6회, 1일 7km의 복합트레이닝을 실시하였다. 근육 내 carnosine 농도는 대퇴 외측광근의 근생검 샘플을 HPLC로 분석하였다. 12주간의 β-alanine 섭취와 수영훈련에 의해 보충 집단의 근육 내 carnosine 농도는 11% 증가한 반면(p<.05), 위약집단에서는 유의한 변화가 나타내지 않았다. 한편, β-alanine 보충집단의 반복적인 수영 시행(100m×7회 자유형, 매 시행직후, 안정시, 회복기 3, 6, 9분)에서 운동수행능력의 변화를 살펴본 결과, 위약집단과 비교할 때 β-alanine 보충집단의 수영 기록이 유의한 향상(p<.05)을 나타내었으며 심박수 변화에서도 집단 간 유의한 차이(p<.05)를 발견할 수 있었다. 이상의 결과는 운동 훈련 시 β-alanine 보충은 세포내 carnosine 합성을 증가시킬 수 있으며 이러한 근육 내 carnorsine 농도 증가는 고강도 운동 시 운동수행능력에 긍정적인 영향을 미침으로써 운동선수를 위한 영양보조물로서의 가능성을 시사하고 있다. Carnosine(Carn) is a dipeptide of β-alanine and histidine and occurs in high concentrations in skeletal muscle. Carn is highly effective on buffering H+ over the physiological pH range and probably the only such active compound of which concentration can be changed by diet. This study was performed to investigate the effect of 12 wk β-alanine supplementation on muscle carnosine and lactate concentration, heart rate, and speed during the repeated high intensive swimming trials. Ten young elite swimmers(22-25yrs) participated in this study. They were divided into β-alanine group (n=5, 3.2g/d of β-alanine) and placebo group(n=5) and performed combined exercise training for 12 weeks. Subjects performed 7 repetitions of 100m freestyle swimming with blood samplings before, after, and during the recovery. Percutaneous muscle biopsies were obtained before and after the supplementation. Carnosine concentration was determined by high performance liquid chromatography(HPLC). From the result of this study, we found Carnosine content(11%, p<.05), heart rate(p<.05), and the speed of 7 repetitions 100m freestyle swimming(p<.05) were positively changed with the supplementation. These findings suggest that the increased carnosine concentration with β-alanine supplementation is likely contribute to exercise performance and a possibility that β-alanine may be used as an useful ergogenic aid for athletes.

The Usefulness of Muscle Biopsy in Initial Diagnostic Evaluation of Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes

백민성,김세훈,이영목 연세대학교의과대학 2019 Yonsei medical journal Vol.60 No.1

Purpose: The disease entity mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is characterizedby an early onset of stroke-like episodes. MELAS is the most dominant subtype of mitochondrial disease. Molecular genetictesting is important in the diagnosis of MELAS. The mitochondrial DNA (mtDNA) 3243A>G mutation is found in 80% of MELASpatients. Nevertheless, molecular analysis alone may be insufficient to diagnose MELAS because of mtDNA heteroplasmy. Thisstudy aimed to evaluate whether muscle biopsy is useful in MELAS patients as an initial diagnostic evaluation method. Materials and Methods: The medical records of patients who were diagnosed with MELAS at the Department of Pediatrics ofGangnam Severance Hospital between January 2006 and January 2017 were reviewed. The study population included 12 patients. They were divided into two subgroups according to whether the results of muscle pathology were in accordance with mitochondrialdiseases. Clinical variables, diagnostic evaluations, and clinical outcomes were compared between the two groups. Results: Of the 12 patients, seven were muscle pathology-positive for mitochondrial disease. No statistically significant differencein clinical data was observed between the groups that were muscle pathology-positive and muscle pathology-negative for mtDNA3243A>G mutation. Additionally, the patients with weakness as the initial symptom were all muscle pathology-positive. Conclusion: The usefulness of muscle biopsy appears to be limited to an initial confirmative diagnostic evaluation of MELAS. Muscle biopsy can provide some information in MELAS patients with weakness not confirmed by genetic testing.

A combination of Korean mistletoe extract and resistance exercise retarded the decline in muscle mass and strength in the elderly: A randomized controlled trial

Lim, N.J.,Shin, J.H.,Kim, H.J.,Lim, Y.,Kim, J.Y.,Lee, W.J.,Han, S.J.,Kwon, O. Pergamon Press ; Elsevier Science Ltd 2017 Experimental Gerontology Vol.87 No.1

<P>Given the increased concerns about the degenerative decline in the physical performance of the elderly, there is a need for developing effective strategies to suppress the age-related loss of skeletal muscle mass and functional capacity through a lifestyle intervention. This randomized controlled trial examined whether a combination of Korean mistletoe extract (KME) supplement and exercise affected muscle mass, muscle function, and targeted molecular expressions. Sixty-seven subjects aged 55-75 years were assigned to placebo, low-dose (1 g/d), or high-dose (2 g/d) of KME for 12 weeks. The body composition was significantly changed in the high-dose group during the intervention period as determined by skeletal muscle mass (P = 0.040), fat free mass (P = 0.042), soft lean mass (P = 0.023), skeletal muscle index (P = 0.041), fat-free mass index (P = 0.030), percent body fat (P = 0.044), and fat mass to lean mass ratio (P = 0.030). Knee strength was measured by Cybex, demonstrating a significant effect in the KME groups compared to the placebo group (P = 0.026 for peak torque and P = 0.057 for set total work), which was more pronounced after adjusting for age, gender, protein, and energy intake (P = 0.009 for peak torque and P = 0.033 for set total work). The dynamic balance ability was remarkably improved in the high-dose group over a 12-week period as determined by Timed 'Up and Go' (P = 0.005 for fast walk test and P = 0.024 for ordinary walk test). Consistent with these results, RT-PCR, multiplex analyses, and immunocytofluorescence staining revealed that a high-dose KME supplementation was effective for suppressing intracellular pathways related to muscle protein degradation, but stimulating those related to myogenesis. In particular, significant differences were found in atrogin-1 mRNA (P = 0.002 at a single administration and P = 0.001 at a 12-week administration), myogenin mRNA (P < 0.0001 at a single administration and P = 0.040 at a 12-week administration), and insulin growth factor 1 receptor phosphorylation (P = 0.002 at a 12 week administration). These results suggest that KME supplementation together with resistance exercise may be useful in suppressing the age-related loss of muscle mass and strength in the elderly. (C) 2016 Elsevier Inc. All rights reserved.</P>

단기간의 고강도 저항성 트레이닝에 의한 근섬유의 MHC phenotype 변화

하재여(Ha Jae-Yeo),김효정(Kim Hyo-Jeong),김창근(Kim Chang-Keun) 한국체육과학회 2011 한국체육과학회지 Vol.20 No.3

The purpose of this study was to examine the effect of 4 week resistance training on myosin heavy chain (MHC; Ⅰ, Ⅰ/Ⅱa, Ⅱa, Ⅱa/Ⅱx, Ⅱx, Ⅰ/Ⅱa/Ⅱx) isoforms in single muscle fiber in young men (age 21.20±2.25, n=10). Subjects were subjected to 4 weeks of isokinetic training and each training session consisted of 10 × 10 maximal 90°/s and 180°/s extension and flexion contractions using a Kin-Com isokinetic dynamometer. To maintain the effective control of the differences of training impact on individuals, the present study adopted the one-leg exercise training or within subject model. Muscle biopsies were obtained from right/left vastus lateralis before and after training. We analyzed MHC isoforms in single muscle fiber by electrophoretic analysis (100 fibers analyzed to each biopsy sample; 4,000 fibers analyzed in total). Mean MHC IIa isoforms in the trained leg were significantly increased by 10% (p<.05), whereas the number of MHC IIx isoforms was decreased by 8% (p<.01). MHC Ⅱa was significantly increrased by 5% (p<.05) in untrained leg. MHC Ⅱx and MHC Ⅰ/Ⅱa/Ⅱx were decreased by 7% (p<.05) and 1% (p<.05) respectively in untrained leg. However, MHC IIa expression in trained leg was increased by 5%(p<.05) than untrained leg. From the result of this study we confirmed 4 week of training result in significant changes in MHC isoforms in within subject resistance exercise model.

Activation of the wnt/β-Catenin Signaling Pathway in Polymyositis, Dermatomyositis and Duchenne Muscular Dystrophy

Fuchen Liu,Zonglai Liang,Jingwen Xu,Wei Liu,Dandan Zhao,Yuying Zhao,Chuanzhu Yan 대한신경과학회 2016 Journal of Clinical Neurology Vol.12 No.3

Background and PurposezzTe wnt/β-catenin signaling pathway plays a critical role in embryonic development and adult-tissue homeostasis. Recent investigations implicate the importance of wnt/β-catenin signaling in normal wound healing and its sustained activation being associated with fbrogenesis. We investigated the immunolocalization and activation of wnt/β-catenin in polymyositis (PM), dermatomyositis (DM), and Duchenne muscular dystrophy (DMD). MethodszzImmunofuorescence staining and Western blot analysis of β-catenin were performed in muscle specimens from 6 PM, 8 DM, and 6 DMD subjects. Te β-catenin/Tcf4 DNA-binding activity in muscle was studied using an electrophoretic mobility shif assay (EMSA), and serum wnt/β-catenin/Tcf transcriptional activity was measured using a luciferase reporter gene assay. ResultszzImmunoreactivity for β-catenin was found in the cytoplasm and nuclei of muscle fbers in PM, DM, and DMD. Te protein level of β-catenin was elevated, and EMSA analysis confrmed the activation of wnt/β-catenin signaling. Te transcriptional activities of β-catenin/ Tcf in the circulation were increased in patients with PM, DM, and DMD, especially in those with interstitial lung disease, and these transcriptional activities decreased when PM or DM patients exhibited obvious clinical improvements. ConclusionszzOur fndings indicate that wnt/β-catenin signaling is activated in PM, DM, and DMD. Its activation in muscle tissue and the circulation may play a role in modulating muscle regeneration and be at least partly involved in the process of muscle and pulmonary fbrosis.

Continuous Retrogressive Degeneration of Postnatal Cleft Lip

박정민,박영욱,김광수,유영재,김연숙,이상신,이석근 대한구강악안면병리학회 2009 대한구강악안면병리학회지 Vol.33 No.4

In order to know the degenerating state of postnatal cleft lip tissue, total 23 cases of lip biopsy obtained from cleft lip surgery were collected and examined pathologically. The cleft lip tissues characteristically disclosed epithelial hyperplasia (13/23), stromal myxoid degeneration (20/23), salivary gland degeneration (1/23), muscular degeneration (11/23), and sebaceous gland hyperplasia (15/23), melanocytic infiltration (5/23). The epithelial hyperplasia was marked with hyperkeratosis and basal hyperplasia, which was usually coincident with the myxoid degeneration of underlying connective tissue. The myxoid degeneration was diffuse in the deep connective tissue with chronic inflammatoryreaction, and followed by extensive muscular degeneration. The sebaceous gland hyperplasia was usually predominant in the skin area of the cleft lip. In this study the lip biopsy from 30years old patient still showed remarkable retrogressive degeneration of lip tissue. Therefore, it is considered that the postnatal cleft lip tissue is continuously degenerative, and its retrogressive change gradually affects the deterioration of perioral muscular structures, consequently resulted in the failure of lip functions as well as further bizarre malformation of oro-facial shape during the postnatal period. These data also indicate that the biopsy of postnatal cleft lip should be recommended to know its variable degenerating status and to perform the proper rehabilitation surgery of cleft lip.

태생 후 구순열의 지속적인 퇴행성 변성

박정민,박영욱,김광수,유영재,김연숙,이상신,이석근 대한구강악안면병리학회 2009 대한구강악안면병리학회지 Vol.33 No.4

In order to know the degenerating state of postnatal cleft lip tissue, total 23 cases of lip biopsy obtained from cleft lip surgery were collected and examined pathologically. The cleft lip tissues characteristically disclosed epithelial hyperplasia (13/23), stromal myxoid degeneration (20/23), salivary gland degeneration (1/23), muscular degeneration (11/23), and sebaceous gland hyperplasia (15/23), melanocytic infiltration (5/23). The epithelial hyperplasia was marked with hyperkeratosis and basal hyperplasia, which was usually coincident with the myxoid degeneration of underlying connective tissue. The myxoid degeneration was diffuse in the deep connective tissue with chronic inflammatoryreaction, and followed by extensive muscular degeneration. The sebaceous gland hyperplasia was usually predominant in the skin area of the cleft lip. In this study the lip biopsy from 30years old patient still showed remarkable retrogressive degeneration of lip tissue. Therefore, it is considered that the postnatal cleft lip tissue is continuously degenerative, and its retrogressive change gradually affects the deterioration of perioral muscular structures, consequently resulted in the failure of lip functions as well as further bizarre malformation of oro-facial shape during the postnatal period. These data also indicate that the biopsy of postnatal cleft lip should be recommended to know its variable degenerating status and to perform the proper rehabilitation surgery of cleft lip.