• Journal of Oral Medicine and Pain
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• v.35 no.2
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• pp.155-163
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• 2010

Dermatomyositis (DM) is an idiopathic inflammatory connective tissue disorder and a systemic autonomic immune disease which shows a progressive muscle weakness and characteristic rash. It is identified by a characteristic rash accompanying, or more often preceding muscle weakness. Pathognomonic skin lesions are a blue-purple discoloration on the upper eyelids with edema (heliotropic rash), a flat red rash on the face and upper trunk, and erythema of the knuckles with a raised violaceous scaly eruption (Gottron's papule). The myopathy represents inflammatory and degenerative changes primarily affecting proximal muscles. DM often involves GI tract and respiratory system with as risk of 15-25% internal malignancy. It's managed with sun protection since muscle weakness as well as a rash could be aggravated by sun exposure. Systemic corticosteroid is an initial therapy and other immunosuppressive agent has been used as alternatives. Facial muscles are unaffected and masticatory muscles are rarely affected in DM. We present trismus close to muscle contracture in a patient with DM. Therefore, it needs continuous mouth-opening exercise to prevent progressive muscle contracture and to ensure normal mouth opening.

• 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.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1116-1124
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• 2007

Purpose : Juvenile dermatomyositis (JDM) is the most common of the idiopathic inflammatory myopathies in children. The purpose of this study is to observe demographic, initial presentations, duration of time between disease onset and diagnosis, clinical manifestations and laboratory findings at diagnosis of patients with JDM. Methods : Forty seven patients identified at Seoul National University Children's Hospital from January 1986 to May 2007. Medical records were reviewed retrospectively focusing on initial presentations, clinical manifestations and laboratory findings at the time of diagnosis of patients with JDM. Results : Male and female patients were 25 and 22, respectively and sex ratio was 1.14:1. The average age at the time of diagnosis was 6.51 years. Skin rash (94%) was the most common symptom, followed by the proximal muscle weakness (89%). The disease activity score was 10.8. The duration between the onset of the skin rash and the muscle weakness and diagnosis was 7.18 and 4.70 months, respectively. The serum muscle enzymes, LDH, AST, CK and aldolase, were elevated in the patient with JDM. Autoimmune antibodies, antinuclear antibody, anti SSA antibody and anti SSB antibody, were negative findings. Electromyography findings were consistent with JDM in 88% of the patients, the muscle biopsy was in 91% and all MRI findings were compatible with those of patients with JDM. The most common symptom besides musculocutaneous lesions was the calcinosis (62.5%). The most common site of calcinosis was the pelvic area and buttocks. Conclusion : This study shows that the major symptoms are proximal muscle weakness and cutaneous lesion, and they are important to diagnose JDM.

• Tuberculosis and Respiratory Diseases
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• v.64 no.2
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• pp.138-143
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• 2008

Pneumatosis intestinalis or spontaneous pneumomediastinum are rarely associated with nonspecific interstitial pneumonia (NSIP). However, the development of both conditions in the same patient simultaneously has not been reported previously. A 56-year-old man with NSIP developed spontaneous pneumomediastinum accompanied by subcutaneous emphysema and pneumatosis intestinalis after the treatment with intravenous high dose steroid. The development of spontaneous pneumomediastinum, subcutaneous emphysema and pneumatosis intestinalis in this patient was possibly due to the factors such as NSIP, high dose steroid therapy and subclinical dermatomyositis. Treatment with corticosteroid and cyclosporin gradually improved his exacerbated NSIP and pneumomediastinum, subcutaneous emphysema, pneumatosis intestinalis.

• 대한핵의학회:학술대회논문집
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• 1988.05a
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• pp.125.1-125.1
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• 1988

• Tuberculosis and Respiratory Diseases
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• v.51 no.4
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• pp.354-363
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• 2001

Background : Although lung involvement has been reported in 5 to 46% of dermatomyositis/polymyositis(DM/PM) patients, reports of the condition in Korea are rare. This study evaluated the clinical features of lung involvement in DM/PM patients. Methods : The medical records, laboratory results and radiologic findings of 79 DM/pM patients, who attended the Seoul National University Hospital (SNUH) between 1989 and 1999, were reviewed retrospectively. Results : A total 79 patients of whom 24 patients(33%) showed lung involvement, were enrolled in this study. More patients with lung involvement were female(F:M=11:1), and older compared with those without lung involvement. Patients with lung involvement presented with dyspnea(79%), coughing(67%), an elevated ESR, and CK/LD. Anti-Jo 1 antibody test was positive in 30%, which is significantly higher in patients with lung involvement. A simple chest X-ray of the patients with lung involvement exhibited reticular opacity(50%), reticulonodular opacity(30%), patchy opacity(29%), nodular opacity(13%) and linear opacity(4%). HRCT(n=24) showed ground glass opacity(75%), linear or septal thickening(50%), patchy consolidation(42%), honey-combing(33%) and nodular opacity(17%). The pulmonary function test showed a restrictive ventilatory pattern(77%) and a lower diffusing capacity(62%). The patients were followed up during a mean duration of $30{\pm}28$ months. They were treated with steroid only(50%) or a combination of steroids and cytotoxic agents(46%). Muscle symptoms were improved in 89% with treatment, but an improvement in the respiratory symptoms or in the pulmonary function test was rare. Patients with lung involvement had a higher mortality rate(21%) than those without lung involvement(10%) during the follow-up periods. Conclusion : DM/PM patients with lung involvement were mostly female, older and had a higher positive rate Anti-Jo 1 antibodies, but there was no significant difference in prognosis.

• The Journal of the Korean bone and joint tumor society
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• v.15 no.1
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• pp.81-86
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• 2009

An extraskeletal osteosarcoma is a rare malignancy. A small number of cases and studies have been reported in the world and only two cases have been reported in Korea. We experienced an extraskeletal osteosarcoma around the knee joint of 91-year-old male who was the oldest case in the literatures. It was developed without history of trauma, irradiation, myositis ossificans, and heterotopic ossification of dermatomyositis. This patient was treated with excision alone, however he was alive and there were no sign of local recurrence or distant metastasis and functional loss during 1-year follow-up.

• The Journal of Korean Medicine
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• v.23 no.2
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• pp.225-230
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• 2002

Dermatomyositis is an inflammatory cutaneo-muscular disease of unknown etiology, characterized by symmetrical proximal muscle weakness and typical dermatologic manifestation. Henoch-Schonlein purpura is most common in children. In Korea, dermatomyositis or Henoch-Schonlein purpura associated with herbal medicine has not been reported. The subject patient is a seven year-old girl diagnosed with dermatomyositis in August 1999 and Henoch-Schonlein purpura in January, July 2001. There were symptoms of dermatomyositis such as headache, arthralgia, eruption, itching and abdominal pain. I think the herb-prescription composed or Radix astragali (Huangqi), Herba agrimoniae (Xianhecao), Fructus jujubae (Dazao) had an ameliorating effect on Henoch-Schonlein purpura by decreasing these symptoms.

• The Korean Journal of Nuclear Medicine
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• v.23 no.2
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• pp.183-188
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• 1989

Esophageal transit of radionuclide was quantitatively analyzed in 29 patients with dermatomyositis-polymyositis. Fourteen patients (48.3%) showed retention of tracer in oropharynx. The mean value of percent retention of oropharynx was $15.5{\pm}16.6%$. Esophageal dysfunction was found in 19 patients (65.5%). Among them 4 showed mild, 12 showed moderate and 3 showed severe esophageal dysfunction. Dysphagia was found in 11 patients (37.9%), which was closely related to percent retention of oropharynx.Quantitative analysis of esophageal transit of radionuclide seemed to be a useful technique for evaluation of dysphagia in patients with dermatomyositis-polymyositis.

• The Korean Journal of Nuclear Medicine
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• v.16 no.1
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• pp.15-22
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• 1982

To evaluate the diagnostic usefulness of radioisotope bone scan and clinical activity of myositis, bone scan using $^{99m}Tc-Methylene$ diphosphonate was serially done before and after treatment with prednisolone in 10 patients with well-documented dermatomyositis-polymyositis. The observed results were as follows. 1. In all 10 patients before treatment with prednisolone, the bone scans showed evidence of increased muscle uptake. Muscle uptake was markedly increased in 4 patients, moderately increased in 3 patients and minimally increased in 3 patients. 2. The site of increased muscle uptake was consistent with the site of clinically involved muscle which was weak and tender. 3. The degree of muscle uptake correlated with the severity of the muscle weakness and tenderness at the scan was done. In all 10 patients treated with high dose prednisolone, muscle uptake was decreased following therapy. Above results suggest the radioisotope bone scanning may be useful in the diagnosis and treatment of patient with dermatomyositis-polymyositis.