• Journal of Chest Surgery
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• v.25 no.4
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• pp.412-417
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• 1992

Pulmonary artery aneurysm is Behcet`s disease is rare and can be fatal due to rupture. We experienced a case of pulmonary artery aneurysm in Behcet`s disease. The patient was 21 year old woman who was adimitted with three month history of dyspnea, fever and cough. On examination, she had aphthous ulcer in the mouth and erythema nodosum on the left popletial fossa and forearm, but didn`t have any lesion at eyes and genitalia. The latex fixation test for rheumatoid factor, VDRL test for syphillis, antinuclear antibody and LE cell test were all negatives. The third and fourth components of complement in the serum, serum immunoglobulin concentrations[IgG, IgM, IgA] were within normal range. The chest radiography revealed a 5x6cm sized radiopaque mass density in the left hilar region. Two months later, the mass was enlarged to 6x7cm. The IV-DSA showed a single aneurysm at the proximal part of left lower lobe artery with lingular segment artery and no distal perfusion by thombotic obstruction. The steroid therapy was done for a month, but symptoms not improved. We performed resection of lingular segment and lower lobe including the aneurysm. The microscopic findings of the operative specimen were intimal hyperplasia and fragmentation of the internal elastic fibers. She was improved without remarkable event, except infection of the operative wound.

• Archives of Plastic Surgery
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• v.33 no.1
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• pp.124-126
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• 2006

Pachydermodactyly is a rare distinct form of fibromatosis characterized by acquired, asymptomatic dense fibrosis and fibroblastic proliferation around one or more proximal interphalangeal joints. Intralesional triamcinolone injection may be helpful in improving the clinical appearance. A 20-year-old unemployed female patient gave a four years history of progressive swelling of the fingers affecting predominantly the proximal interphalangeal joints of index fingers of left hand and index, middle fingers of right hand. There was no pain and tenderness. Physical examination, complete blood count, antinuclear antibody, rheumatoid factor were unremarkable and radiograph of both hands showed soft tissue swelling only without bony abnormality. We excised the redundant soft tissue from the both sides of proximal interphalangeal joint with longitudinal elliptical fashion. After operation, the fingers of the patient showed marked improvement cosmetically. There were no motor or sensory changes. Pachydermodactyly was first reported in 1996 in Korea. Histologic features include an increased dermal accumulation of collagen and occasionally an increased number of fibroblast. We report for one patient with pachydermodactyly treated with longitudinal elliptical excision and obtained an improvement of clinical appearance.

• Journal of Veterinary Clinics
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• v.32 no.4
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• pp.366-369
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• 2015

A 12-year-old, castrated male, mixed-breed dog was referred to us with a history of depression, lameness, neck and multiple joints pain. Clinical signs had been deteriorating from rear limbs lameness leading to pain on all four limbs lameness for 7 days. Mild leukocytosis and increased C-reactive protein concentration were revealed in blood work. On radiography, degenerative change of humerus and cervical intervertebral space narrowing were found. The results of synovial fluid analysis revealed severe neutrophilic pleocytosis, decreased viscosity, increased turbidity and bacterial culture was negative. The antinuclear antibody test was negative and MRI results revealed mild cervical intervertebral disk disease (IVDD). Based on all tests, we diagnosed this case as idiopathic immune-mediated polyarthritis (IMPA). Prednisolone and mycophenolate mofetil were administered and clinical signs were resolved after 7 days. This case report demonstrated that clinical, diagnostic imaging and synovial fluid analysis findings and successful treatment result with prednisolone and mycophenolate mofetil in canine idiopathic IMPA.

• Tuberculosis and Respiratory Diseases
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• v.65 no.4
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• pp.323-327
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• 2008

Systemic lupus erythematosus (SLE) is a multisystem disorder where the etiology is not clearly known. Symptomatic chronic interstitial pneumonitis is an uncommon manifestation, with a reported prevalence of 3~13%. Achalasia is rare disease that presents with failure in the relaxation of the esophagus sphincter. A 22-year-old woman was admitted to our hospital because of fever, cough and dyspnea. The patient had a history of pericardial effusion and Raynaud's phenomenon. The results of laboratory tests indicated the presence of lymphopenia and included positive antibody tests for antinuclear antibody and anti Sm antibody. A chest X-ray demonstrated the presence of peribronchial infiltration on both lung fields. A Chest CT image showed interlobar septal thickening, ground-glass opacity and a honeycomb appearance in both lung fields and esophageal dilatation with air fluid level. An esophagogram showed the presence of dilated esophagus ends that represented the non-relaxed lower esophageal sphincter. Manometry demonstrated incomplete sphincter relaxation. The case was diagnosed as systemic lupus erythematosus associated with interstitial pneumonia and achalasia.

• 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.43 no.4
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• pp.637-644
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• 1996

Interstitial pneumonitis associated with interferon alpha therapy for chronic hepatitis C was first describe6 in 1994 by Kazoo et al In Japan. The mechanism of interstitial pneumonitis developed by interferon alpha was still unknown but immunologic, allergic of direct lung toxicity were suggested. We experienced a case of interstitial pneumonitis developed during interferon alpha therapy for chronic hepatitis C in a 52-year-old male patient. He was treated with 6 million units of interferon alpha intramuscularly 3 times per week for 4 weeks and noted progressive dyspnea and cough. These symptoms were subsided after 6 weeks' discontinuation of interferon alpha therapy. And so, he was retreated with 3 million units of interferon alpha 3 times per week for 8 weeks and felt dyspnea again. He was admitted to our hospital for further evaluation of progressive dyspnea. Arterial blood gas(ABG) values were $PaO_2$ 90.7 mmHg and $PaCO_2$ 31.9 mmHg, and antinuclear antibody(ANA) was negative. A chest X-ray film revealed diffuse reticulo-nodular shadows in bilateral lung fields, suggesting a diagnosis of interstitial pneumonitis. A marked increase in lymphocyte count and suppressor T cell were observed in bronchoalveolar lavage(BAL) fluid. Lymphocyte stimulation test with interferon alpha was positive. Interstitial pneumonitis was confirmed by transbronchial lung biopsy. After discontinuation of interferon alpha, we gave oral steroid in the condition that clinical symptoms were being improved gradually.

• Annals of Clinical Neurophysiology
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• v.9 no.1
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• pp.11-15
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• 2007

Background: The term "overlap syndromes" designates a group of diseases in which polymyositis (PM) or dermatomyositis (DM) is associated with some other disorders of connective tissues. The aim of this study was to delineate the clinical features, laboratory findings, and outcome of treatment of "overlap syndromes" Methods: We analyzed the medical records of 16 patients (PM in 10, DM in 6) with well documented "overlap syndromes" between 1997 and 2004. The diagnosis was made when the criteria for two different disorders were fulfilled. Results: All patients were female. Age of onset ranged from 14 to 52 years (mean 29.8 years) with peak incidence in the third and fourth decades. Systemic lupus erythematosus (SLE) was associated in 10, systemic sclerosis in 7, and rheumatoid arthritis in 3 patients. Four of the patients had two different connective tissue diseases simultaneously. The characteristic clinical features were muscle weakness, arthralgia, Raynaud's phenomenon, and myalgia. In laboratory tests, creatine kinase (CK), lactic dehydrogenase (LDH), and transaminases were usually abnormal. Positive antinuclear antibody (ANA), rheumatoid factor (RF), and cryoglobulin were found in 100%, 69%, and 67% of the patients, respectively. Needle electromyography (EMG) showed abnormal findings compatible with myopathy in 15 patients. The pathology of muscle biopsy from 14 patients revealed findings compatible with inflammatory myopathy. Glucocorticoids were administered to 15 patients. The muscle strength improved in all the treated patients, which was well correlated with repeat CK level and EMG findings. Conclusions: The presence of autoantibodies such as ANA, RF, and cryoglobulin in patients with PM or DM highly suggests the possibility of an overlap syndromes. These syndromes reveal a strong female predominance. The myositis associated with them usually shows a good response to glucocorticoids treatment.

• Journal of Veterinary Clinics
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• v.40 no.5
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• pp.336-340
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• 2023

A dog (2-year old, female, Shih-Tzu) presented with hyperthermia and right-sided facial paralysis characterized by the inability to close the right eye and drooling from the right side of the mouth after H3N2 influenza vaccination [A/Canine/Korea/01/07(H3N2) strain; Caniflu-Max, Bionote, Hwaseong, Gyeonggi-do, ROK]. To determine the cause of the fever and neurological symptoms, physical examination, ophthalmic examination, thoracic and abdominal radiography, abdominal ultrasonography, complete blood counts, serum chemistry values, and electrolyte levels were determined. In addition, Cerebrospinal fluid analysis, antinuclear antibody test, fever of unknown origin polymerase chain reaction (PCR) panel, tick-borne pathogen PCR panel were performed. As a result, hyperthermia, leukocytosis, and elevated C-reactive protein were confirmed. In addition, neurological examination revealed decreased right eyelid reflexes, corneal reflexes, threat response, and facial sensation, it was possible to suspect problems with the trigeminal and facial nerves of the cranial nerve. Magnetic resonance imaging revealed a lesion suggestive of myositis in the right muscular lesion at atlanto-occipital junction level on site of vaccine injection. Therefore, right-sided facial paralysis was tentatively determined to be a secondary cause of nerve damage caused by myositis. The patient was treated with immunosuppressants such as prednisolone and mycophenolate mofetil. After 3 months of immunosuppressant therapy, the patient's symptoms improved.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.424-430
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• 2006

Purpose : Juvenile rheumatoid arthritis(JRA) is one of the most common rheumatic diseases of childhood and is an important cause of short- and long-term disability. The purpose of this study was to determine the disease course and outcome in childhood patients with JRA. Methods : Fifty nine patients with JRA who were diagnosed and treated in the Department of Pediatrics, Asan Medical Center from August 1990 to November 2004 were enrolled in this study. Sex, age, type, affected joints, extra-articular manifestations, laboratory and radiologic findings, treatments, and outcomes of JRA patients were reviewed retrospectively. Results : Among JRA patients, 32.2 percent had pauciarticular type, 30.5 percent had polyarticular type and 37.3 percent had systemic type. The ratio of boys to girls was 1.7 : 1 and the mean age at onset was $9.3{\pm}3.7$(1.3-15.9) years. The most commonly affected joints were knee, ankle and wrist. The extra-articular manifestations observed were fever, rash, myalgia and lymph node enlargement, etc. The main laboratory findings observed were leukocytosis, anemia, thrombocytosis, elevated ESR, and elevated CRP. Rheumatoid factor and antinuclear antibody(ANA) were positive in 5.3 percent and 18.0 percent. Nonsteroid anti-inflammatory drugs(NSAID) were used most frequently and methotrexate with or without steroids was added in 27.1 percent of patients unresponsive to NSAID. 88.1 percent of patients were cured without functional disability and only one patient was in functional status IV. One patient, who had pulmonary involvement, died. Conclusion : Our results showed an even distribution in type of onset, male predominance, older age of onset, low incidence of iridocyclitis, and low positivity of ANA in JRA patients; this differs from occidental data. This study may suggest regional differences and variability in disease groups of JRA among different racies, but further multi-center trials and large scale epidemiological studies are needed to confirm our conclusion.

• Tuberculosis and Respiratory Diseases
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• v.42 no.6
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• pp.878-887
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• 1995

Background: It is reported that frequency of pulmonary involvement in the patients with rheumatoid arthritis reaches 10 to 50% and pulmonary involvement is a principal cause of death. As immunology and radiology has developed, interest for the early diagnosis of pulmonary involvement is increasing. Method: Among the patients at Hanyang University Hospital from March, 1990 to July, 1995, we compared the 29 patients having pulmonary involvement with the 18 patients controls in clinical and chest high resolution computed tomography(HRCT) findings by immunological markers and findings of pulmonary function test. We sought useful markers for early diagnosis of pulmonary involvement with noninvasive investigations. Results: The ratio of males to females was 14 : 15 in the group of pulmonary involvement, and all of the 18 patients were females in the control group. Smoking history was 31%(9/29) in the former group and none in the latter group. Rheumatoid factor(RF) was positive in 95.5%(28/29) of the pulmonary involvement group and in 100%(18/18) of the control group(p=0.12). Antiperinuclear factor(APF) showed a significant difference: 86.9%(20/23, average value: 2.0) was positive in the pulmonary involvement group and 50%(8/16, average value: 1.1) in the control group(p=0.04). Antinuclear antibody(ANA) was positive in 60.7%(17/28) of the pulmonary involvement group and in 72.2%(13/18) of the control group(p=0.33). Cryoglobulin also showed a significant difference: 72%(18/25) in the pulmonary involvement group was positive and 56.2%(9/16) in the control group was positive(p=0.02). Bony erosion was positive in 61.5%(16/26) of the pulmonary involvement group and in 77.7%(14/18) of the control group(p=0.8). On the pulmonary function test, the average value of alveolar volume corrected diffusion capacity and residual volume in the pulmonary involvement group and in the control group were 1.07mmol/rnin/KPa(predicted value: 64.2%), 1.32L(predicted value: 70%) and 1.44mmol/min/KPa, 3.75L(predicted value: 86.6%), respectively(p=0.003, p=0.004), showing a significant difference. Conclusion: APF or cryoglobulin on the serological test, the measurement of residual volume and alveolar volume corrected diffusion capacity may be used as effective markers in the diagnosis of pulmonary involvement of the patients with rheumatoid arthritis.