• Title/Summary/Keyword: Connective tissue diseases

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Recurrent Secondary Pneumothorax Caused by Bronchiolitis Obliterans Due to Chronic Graft Versus Host Disease in a Patient with Chronic Myelogenous Leukemia after Allogenic Bone Marrow Transplantation (골수이식 후 만성 이식편대숙주질환으로 발생한 폐쇄성 세기관지염에 의한 이차성 재발성 기흉 1례)

  • Ahn, Chul Min;Hwang, Sang Yun;Byun, Min Kwang;Lee, Jin Hyoung;Chung, Wou Young;Moon, Jin Wook;Park, Moo Suk;Min, Yoo Hong;Kim, Se Kyu;Chang, Joon;Kim, Sung Kyu;Kim, Haeryoung;Kim, Hoguen;Kim, Young Sam
    • Tuberculosis and Respiratory Diseases
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    • v.57 no.2
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    • pp.183-187
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    • 2004
  • Bronchiolitis obliterans (BO) is a nonspecific inflammatory injury affecting primarily the small airways. Its inflammatory process is characterized by fibrotic obliteration of the lumen of bronchioles. BO can be idiopathic or associated with connective tissue disease, inhaled toxins, infections, drugs, and chronic graft-versus-host-disease (GVHD). Pulmonary complications occur in 40~60% of patients who undergo allogeneic bone marrow transplantation (BMT), causing 10~40% of transplant-related deaths. BO is a characteristic pulmonary complication which occurs usually within a few years after BMT. Documented complications of BO include air-leak syndromes such as pneumomediastinum, subcutaneous emphysema and pneumothorax. We report a case of a 30-year-old male patient with BO due to chronic GVHD after allogenic BMT who presented with recurrent bilateral pneumothoraces.

Fat Embolism Syndrome with Pulmonary Hemorrhage of Unknown Origin (원인을 알 수 없는 폐출혈을 동반한 지방색전 증후군 1예)

  • Jeong, Hye-Cheol;Jung, Ki-Hwan;Kim, Byung-Gyu;Kim, Kyung-Kyu;Lee, Sang-Youb;Park, Sang-Myun;Lee, Sin-Hyung;Shin, Chol;Cho, Jae-Youn;Shim, Jae-Jeong;In, Kwang-Ho;Kim, Han-Gyum;Yoo, Sa-Hwa;Kang, Kyung-Ho
    • Tuberculosis and Respiratory Diseases
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    • v.48 no.3
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    • pp.383-387
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    • 2000
  • Fat embolism syndrome is a rare but serious complication occurring mostly in patients with long bone fractures and occasionally in patients who have had an underlying disease. For example, pancreatitis, diabetes mellitus, alcoholic liver disease and connective tissue disease can be risk factors. The 44-year old woman with a sudden dry cough, blood tinged sputum, and exertional dyspnea visited the Korea University Hospital. Petechiae on her anterior chest wall was found. Chest X-ray and CT showed patchy opacities and multifocal ground-glass opacities in both lung fields. An open lung biopsy demonstrated diffuse pulmonary hemorrhage and intravascular macrovesicular fat bubbles. After conservative management, her symptoms and radiologic findings were significantly improved. A case of fat embolism syndrome without any known risk factors is reported.

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Interstitial Lung Disease Associated with Polymyositis: Response to Cyclophosphamide and Prednisolone Combination Treatment (Cyclophosphamide와 Prednisolone 병합요법에 치료반응을 보인 다발성근염에 동반된 간질성폐질환 1예)

  • Moon, Jong-Ho;Park, Jun-Young;Lee, Sang-Moo;Kim, Hyeon-Tae;Uh, Soo-Taek;Chung, Yeon-Tae;Kim, Yong-Hoon;Park, Choon-Sik;Lee, Kyung-Soo;Kang, Dae-Young
    • Tuberculosis and Respiratory Diseases
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    • v.40 no.2
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    • pp.197-202
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    • 1993
  • Polymyositis (PM) is a inflammatory connective tissue disease involving predominantly skeletal muscles, characterized by symmetrical, proximal muscle weakness, inflammation, and frequently, degeneration. Interstitial lung disease in association with PM occurs in 5~10% of cases and carries an especially grave prognosis. Although the cause of lung involvement in PM is not known, the underlying pathologic process in the lung is an immune mediated inflammation of alveolar structures, alveolitis. It is of interest, therefore, that cyclophosphamide, an immune modulating agent, has been reported to be effective in the treatment of PM. We report a case of corticosteroid resistant PM associated with interstitial lung disease, successfully treated with cyclophosphamide. A 37-year-old female was presented with 8 months duration of cough, exertional dyspnea, and muscle weakness. She had typical symptoms, physical findings, and elevated muscle enzyme levels in serum with characteristic findings of muscle biopsy. She also had typical interstitial lung disease pattern on chest X-ray and high resolution CT with restrictive pattern on pulmonary function test. The findings of transbronchial lung biopsy was compatible with interstitial lung disease. She failed to respond to corticosteroid initially. Subsequently steroids and cyclophosphamide were given with excellent clinical improvement.

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The Etiology of the Diffuse Infiltrative Disease of the Lung in Korea (한국에서의 미만성 침윤성 폐질환의 원인별 분포)

  • Kim, J.H.;Choi, S.J.;Kim, D.S.;You, J.H.;Kang, H.M.;You, S.H.;Jo, D.I.;Kim, J.W.;Han, S.K.;Kim, K.Y.;Kim, Y.S.;Park, C.S.;Kim, W.S.;Kim, W.D.;Jang, J.;Lee, W.Y.;Choi, B.H.;Hue, S.H.;Shin, D.H.;Lee, J.H.
    • Tuberculosis and Respiratory Diseases
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    • v.38 no.1
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    • pp.1-7
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    • 1991
  • To study the frequency of the underlying disease of the diffuse pulmonary infiltrates in korea, we ansalyzed retrospectively 982 patients who were seen at nine university hospitals and one general hospital in Seoul area. The results are folloing: 1) Among the total 982 patients, 490 patients were male and 492 patients were female. The mean age was 44.3 years. 2) The most common etiology was milliary tuberculosis (38%), which was followed by, idopathic pulmonary fibrosis (27%), pulmonary fibrosis associated with collagen-vascular disease (15%), and diffuse pulmonary infiltrates by malignancy (10%). 3) Amon the connective tissue disease which was accompanied by the interstitial lung disease, rheumatoid arthritis was the most common disease (43%), systemic lupus erythematosus was the 2nd (28%), and progressive systemic sclerosis was the 3re (16%). 4) Among 101 cases of malignant disease, lung was the most frequent primary site (31%), which was followed by stomach (28%), thyroid (16%), and breast (6%). 5) For the diagnosis of the underlying disease of pulmonary infiltrates, the transbronchial lung biopsy was performed in 21% of the patients and open lung biopsy was done in 7%.

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Controversial issues in the legal restriction for prenatal genetic testing in Korea (산전검사 대상 질환에 대한 법적 규제의 문제점에 대한 고찰)

  • Choi, Ji-Young;Jeong, Seon-Yong;Kim, Hyon-J.
    • Journal of Genetic Medicine
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    • v.4 no.2
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    • pp.186-189
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    • 2007
  • More than 6,000 rare disorders including genetic diseases have been reported. Of them, 1,500 diseases (1,211 for clinical diagnosis and 289 for research only) are technically possible for genetic testing. In Korea, since 2005, only 63 genetic diseases is permitted for prenatal genetic testing by the "Bioethics and Biosafety Law". The article 25 in the law prescribes 63 genetic diseases without clear indication for its selection and inclusion criteria. In EU, USA, and other foreign countries, however, there is no provision in the statute on prenatal genetic testing; it is not restricted by a law. Recently, a woman (Mrs. L, 38y) who is a carrier for Menkes disease made an appeal to a government for an amendment of the "Bioethics and Biosafety Law" prohibiting the prenatal diagnosis of her pregnancy at risk for Menkes disease. Menkes disease (MNK) is an X-linked recessive disorder characterized by neurodegeneration, connective tissue defects and hair abnormalities, and no effective treatment is available yet. The prevalence rate of MNK is one in about 250,000 live births. Menkes syndrome patients fail to absorb copper from the gastrointestinal tract in quantities adequate for meeting nutritional needs. These needs seem particularly acute during the initial 12 month of life, when the velocity of brain growth and motor neurodevelopment. Most of pts. die around 3yrs. of age. Mrs. L had a boy with Menkes disease who died at 2y.o. in 2001. Subsequent pregnancy in 2003, she was able to have prenatal genetic testing for mutation of the Menkes (ATP7A) gene and delivered a healthy baby boy. Now, She is pregnant again and wants to have prenatal diagnosis. however, this time, she was not allowed to have any more because Menkes disease is not included in 63 genetic diseases permitted by the law for prenatal genetic testing, in spite of the fact that she is a Menkes disease carrier and her pregnancy is at risk to have an affected baby. This case shows the practical problem of the legal restriction for prenatal genetic testing in Korea. In this study, we report a arguable case and discuss the controversial issues in the legal restriction for prenatal genetic testing in Korea.

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Clinical Study of the Patients, in Whom Pulmonary Embolism was Suspected by Lung Perfusion Scan (폐 관류주사검사상 폐동맥 색전증 소견을 보인 환자의 임상적 고찰)

  • Lee, Gwi-Lae;Kim, Jae-Yeol;Park, Jae-Suk;Yoo, Chul-Gyu;Kim, Young-Whan;Shim, Young-Soo;Han, Sung-Koo
    • Tuberculosis and Respiratory Diseases
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    • v.44 no.4
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    • pp.889-898
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    • 1997
  • Pulmonary Embolism can develop in variable conditions, and presents with nonspecific symptoms and signs. If diagnosis is delayed, it can be resulted in catastrophic results. Therefore, early diagnosis and adequate treatment is crucial in Pulmonary Embolism. Lung Perfusion Scan is useful screening test. Negative result can exclude pulmonary embolism. But, perfusion defects don't always mean pulmonary embolism. To find the better methods of interpretation of lung perfusion scan and To evaluate the clinical course and outcomes of the patients, in whom pulmonary embolism was suspected by lung perfusion scan, we reviewed the clinical records of 49 cases suspected by lung perfusion scan at Seoul National University Hospital during the period of January, 1995 to July, 1996. The results are as follows. First impression of cases in which PE was present at time of admission were pulmonary embolism (63%), heart diseases (26%), and pneumonia (11%) in orders. Underlying diseases of cases in which PE developed during admission were malignancy (36.5%), ICH (22.7%), sepsis (13.7%), and SLE (9.1%) in orders. The predisposing factors were operation (20%), cancer (16%), immobility (16%), connective tissue disease (16%), heart dis. (10%), old age (10%), and preg/pelvic dis. (8%) The results, of lung perfusion scan were HPPE 40 cases(26.8 %), IPPE 21 cases(14.1%), LPPE 88 cases (59.1%), and cases(%) of treatment in these cases were HPPE 34 cases(85%), IPPE 9 cases(42.9%), LPPE 0 case(0.0%). Treatments were heparin and warfarin (69.5%), heparin alone (8.2%), warfarin alone (2.0%), embolectomy (4.1%), thrombolytics (2.0%), IVC filter (2.0%), and no treatment (12.2%) In 34 cases (69.4%), follow up could be done, and 5 cases were recurred (10.2%). The causes of recurrence was incomplete anticoagulant therapy (3 cases) and recurrence of predisposing factor (2 cases). Expired case due to pulmonary embolism was one who was expired just before trial of thrombolytic therapy. Conclusion : Efforts should be made to shorten the interval from onset of Sx to Dx, ie, high index of suspision.

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Development of Porcine Pericardial Heterograft for Clinical Application (Microscopic Analysis of Various Fixation Methods) (돼지의 심낭, 판막을 이용한 이종이식 보철편의 개발(고정 방법에 따른 조직학적 분석))

  • Kim, Kwan-Chang;Choi, Chang-Hyu;Lee, Chang-Ha;Lee, Chul;Oh, Sam-Sae;Park, Seong-Sik;Kim, Woong-Han;Kim, Kyung-Hwan;Kim, Yong-Jiin
    • Journal of Chest Surgery
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    • v.41 no.3
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    • pp.295-304
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    • 2008
  • Background: Various experimental trials for the development of bioprosthetic devices are actively underway, secondary to the limited supply of autologous and homograft tissue to treat cardiac diseases. In this study, porcine bioprostheses that were treated with glutaraldehyde (GA), ethanol, or sodium dodecylsulfate (SDS) were examined with light microscopy and transmission electron microscopy for mechanical and physical imperfections before implantation, Material and Method: 1) Porcine pericardium, aortic valve, and pulmonary valve were examined using light microscopy and JEM-100CX II transmission electron microscopy, then compared with human pericardium and commercially produced heterografts. 2) Sections from six treated groups (GA-Ethanol, Ethanol-GA, SDS only, SDS-GA, Ethanol-SDS-GA and SDS-Ethanol-GA) were observed using the same methods. Result: 1) Porcine pericardium was composed of a serosal layer, fibrosa, and epicardial connective tissue. Treatment with GA, ethanol, or SDS had little influence on the collagen skeleton of porcine pericardium, except in the case of SDS pre-treatment. There was no alteration in the collagen skeleton of the porcine pericardium compared to commercially produced heterografts. 2) Porcine aortic valve was composed of lamina fibrosa, lamina spongiosa, and lamina ventricularis. Treatment with GA, ethanol, or SDS had little influence on these three layers and the collagen skeleton of porcine aortic valve, except in the case of SDS pre-treatment. There were no alterations in the three layers or the collagen. skeleton of porcine aortic valve compared to commercially produced heterografts. Conclusion: There was little physical and mechanical damage incurred in porcine bioprosthesis structures during various glutaraldehyde fixation processes combined with anti-calcification or decellularization treatments. However, SDS treatment preceding GA fixation changed the collagen fibers into a slightly condensed form, which degraded during transmission electron micrograph. The optimal methods and conditions for sodium dodecylsulfate (SDS) treatment need to be modified.

Antimicrobial Effect of Mouthwash against Streptococcus mutans by Visual Staining Method (시각적 염색 방법을 이용한 마우스워시의 구강균에 대한 항균효과 확인)

  • Park, Taehun;Cho, Jeong Hun;Sung, Youngeun;Cho, Jun-Cheol;Shin, Kyeho
    • Journal of the Society of Cosmetic Scientists of Korea
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    • v.40 no.2
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    • pp.187-193
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    • 2014
  • Dental caries are one of the most common oral diseases and Streptococcus mutans (S. mutans) plays an important role in the initiation and progression of dental caries. Oral malodor is primarily the result of microbial metabolism such as Porphyromonas gingivalis (P. gingivalis) that produce volatile sulfur compounds (VSCs), causing oral malodor. Prevotella intermedia (P. intermedia) is known as typical periodontopathic bacteria, and periodontal disease is chronic inflammatory disease that leads to damage of gingival connective tissue and alveolar bone, eventually loss of teeth. In this study, we investigated antimicrobial effect of mouthwash containing cetylpyridinium chloride (CPC), sodium fluoride (NaF), green tea water extract and pine needles water extract against cariogenic and periodontopathic bacteria sucn as S. mutans, P. gingivalis and P. intermedia. As a result, the reduction ratios of S. mutans and P. gingivalis were 4.00 Log and 4.68 Log reduction for 30 s, and P. intermedia were 2.40 Log reduction for 30 s and 2.70 Log reduction for 60 s. Dentocult SM Strip mutans (SM Strip) provides easy detection of visual data showing a significant inhibition on S. mutans. In conclusion, we expected that mouthwash containing CPC, NaF, green tea water extract and pine needles water extract could help preventing the dental disease like dental caries and oral malodor.

CASE REPORTS OF FASCIAL SPACE ABSCESS CAUSED BY ODONTOGENIC INFECTION (치성 감염에 의한 근막간극 농양의 치험례)

  • Choi, Ji-Eun;Yang, Kyu-Ho;Choi, Nam-Ki;Kim, Sun-Mi
    • Journal of the korean academy of Pediatric Dentistry
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    • v.35 no.1
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    • pp.136-143
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    • 2008
  • Infections involved with the oral and maxillofacial area are associated with various anatomical structures. If the proper treatment is not done in an immediate period, the infections will be quite fatal. The causes of the infections are numerous, but the most common cause of odontogenic infections in children is a dental caries. It is known to lead to some kinds of diseases such as periapical abscess, cellulitis, osteomyelitis, Ludwig's angina, toxic shock syndrome and so on. The common pathogenic sequence of fascial abscess is a necrotic pulpal inflammation in the form of dentoalvelor abscess which spreads over and gradually penetrates into the fascial membranes through the cortical bones and finally contracts the potential fascial spaces. If the infections of oral maxillofacial area were penetrated into the surrounding soft tissues, then they would diffuse into the directions of the least tissue resistance along with the connective tissues and the fascial spaces. These infections can be properly cured by tooth extraction, endodontic therapy, surgical treatment including Incision & drainage and antibiotics. The purpose of the cases is to report the satisfactory treatment results in the patients derived from the canine fascial space abscesss or buccal fascial space ones of the odontogenic origin.

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Exendin-4 Improves Nonalcoholic Fatty Liver Disease by Regulating Glucose Transporter 4 Expression in ob/ob Mice

  • Kim, Seok;Jung, Jaehoon;Kim, Hwajin;Heo, Rok Won;Yi, Chin-Ok;Lee, Jung Eun;Jeon, Byeong Tak;Kim, Won-Ho;Hahm, Jong Ryeal;Roh, Gu Seob
    • The Korean Journal of Physiology and Pharmacology
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    • v.18 no.4
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    • pp.333-339
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    • 2014
  • Exendin-4 (Ex-4), a glucagon-like peptide-1 receptor (GLP-1R) agonist, has been known to reverse hepatic steatosis in ob/ob mice. Although many studies have evaluated molecular targets of Ex-4, its mechanism of action on hepatic steatosis and fibrosis has not fully been determined. In the liver, glucose transporter 4 (GLUT4) is mainly expressed in hepatocytes, endothelial cells and hepatic stellate cells (HSCs). In the present study, the effects of Ex-4 on GLUT4 expression were determined in the liver of ob/ob mice. Ob/ob mice were treated with Ex-4 for 10 weeks. Serum metabolic parameters, hepatic triglyceride levels, and liver tissues were evaluated for hepatic steatosis. The weights of the whole body and liver in ob/ob mice were reduced by long-term Ex-4 treatment. Serum metabolic parameters, hepatic steatosis, and hepatic fibrosis in ob/ob mice were reduced by Ex-4. Particularly, Ex-4 improved hepatic steatosis by enhancing GLUT4 via GLP-1R activation in ob/ob mice. Ex-4 treatment also inhibited hepatic fibrosis by decreasing expression of connective tissue growth factor in HSCs of ob/ob mice. Our data suggest that GLP-1 agonists exert a protective effect on hepatic steatosis and fibrosis in obesity and type 2 diabetes.