• Title/Summary/Keyword: Vasculitis

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A Case of Behcet's Disease with Multiple Cavitary Lung Lesion (다발성 폐공동을 형성한 베체트병 1예)

  • Yoon, Se Hee;Son, Ji Woong;Joung, Chung Il;Choi, Eu Gene
    • Tuberculosis and Respiratory Diseases
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    • v.61 no.1
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    • pp.65-69
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    • 2006
  • Behcet's disease is a systemic vasculitis of an unknown etiology involving the arteries and veins of all sizes. There are reports showing that a pulmonary artery aneurysm or thromboembolism and superior vena cava thrombosis are present in 5-10% of patients with Behcet's disease and that lung parenchymal lesions are mainly airway consolidations resulting from hemorrhage or infarction. We encountered a patient with increasing pulmonary cavitary changes and localized aspergilloma. The patient was a 43-year-old man diagnosed with Behcet's disease with a history of recurrent oro-genital ulceration and uveitis, and who was administered methotrexate, colchicines, prednisolone. During the follow up he developed progressive dyspnea upon exertion and finger clubbing. Therefore further evaluations were performed. Chest computed tomography showed more advanced consolidations and cavitations than the previous film with the previously known aspergilloma still observable. An open lung biopsy was carried out to determine the presence of malignant changes, which revealed nonspecific vasculitis. Azathioprine was added resultion in an improvement of symptoms.

Clinical Manifestations of the Lung Involvement in Behçet's Syndrome (Behçet 증후군에서 폐침범의 임상양상에 관한 고찰)

  • Park, Kwang Joo;Park, Seung Ho;Kim, Sang Jin;Kim, Hyung Jung;Chang, Joon;Ahn, Chul Min;Kim, Sung Kyu;Lee, Won Young
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.5
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    • pp.763-773
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    • 1996
  • Background : Behçet's syndrome is a chronic multisystemic disease affecting many organs such as skin, mucosa, eye, joint, central nervous system and blood vessels. Lung involvement occurs in 5% of Behçet's syndrome and is thought to be due to the pulmonary vasculitis leading to thromboembolism, aneurysm and arteriobronchial fistula. Pulmonary vasculitis in Behçet's syndrome is a unique clinical feature, differing from other vasculitis affecting the lung and is one of the major causes of death. Therefore, we examined the incidence, the clinical features, the radioloic findings and the clinical courses of the lung involvement in Behçet's syndrome. Methods: We retrospectively reviewed the medical records and radiologic studies of 10 cases of the lung involvement in Behçet's syndrome diagnosed at Yongdong Severance Hospital and Severance Hospital from 1986 to 1995. We analysed the clinical features, the radiological findings, the treatment modalities and the clinical courses. Results: 1) The incidence of the lung involvement in Behçet's syndrome was 2%(10/487). The male to female ratio was 8 : 2 and the mean age was 34 years. The presenting symptom was hemoptysis in 5 of 10 cases, and massive hemoptysis was noted in 2 cases. Other pulmonary symptoms were cough(6/10), dyspnea(4/10), and chest pain(2/10). Other manifestations were oral ulcers(10/10), genital ulcers(9/10), skin lesions(7/10), and eye lesions(6/10). 2) The laboratory findings were nonspecific. The posteroanterior views of chest radiographies showed multiple infiltrates(6/10), nodular or mass-like opacities(4/10), or normal findings(2/10). The chest CT scans showed multifocal consolidations(6/8), and aneurysms of the pulmonary aneries(4/8). The pulmonary angiographies were performed in 3 cases, and showed pulmonary artery aneurysms in 2 cases. The ventilation-perfusion scans in 2 cases of normal chest x-ray showed multiple mismatched findings. 3) The patients were treated with combination therapy consisting of corticosteroids, cyclophosphamide, and colchicine or anticoagulant agents. Surgical resection was performed in one case with a huge aneurysm. 4) We have followed up nine of ten cases. Three cases are well-being with medical therapy, two cases are severely disabled now and four cases died due to massive hemoptysis, massive pulmonary embolism, or sepsis. Conclusion : Pulmonary vasculitis is a main feature of the lung involvement of Behçet's syndrome, causing hemorrhage, aneurysmal formation, and/or thromboemboism. The lung involvement of Behçet's syndrome is uncommon but is one of the most serious prognostic factors of the disease. Therefore, an aggressive diagnostic work-up for early detection and proper treatment are recommended to improve the clinical course and the survival.

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Feline infectious peritonitis : A case report (고양이 전염성복막염의 자연감염 예)

  • ;;;;;;Manfred Reinacher
    • Korean Journal of Veterinary Service
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    • v.26 no.1
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    • pp.67-71
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    • 2003
  • A 8 months old, female domestic Shorthair cat with long-term signalment of anorexia, lacrimation, uveitis and coughing was submitted to the Pathology and Diagnosis Reference Division, NVRQS, Korea, for necropsy. Main gross lesions were characterized by ascities, some grayish-white nodular formation and fibrous adhesion on the surface of visceral organs including liver and kidney. Principle histopathological findings were fibrinous serositis, multifocal granuloma and necrosis, vasculitis, perivasculitis in various pharenchymal organs. Paraffin-embedded tissue sections taken from most of organs with granulomatous lesions were confirmed specific reaction to the monoclonal antibody of feline infectious peritonitis virus in the cytoplasm of many infiltrating macrophages by immunohistochemistry. The report was to describe the pathological lesions of the first naturally-occuring FIP case in companion cat of Korea.

A Case of Subcortical Dementia After Cerebral Malaria (말라리아 후유증으로 초래된 피질하성 치매 1례)

  • Chung, Hyo-Kyung;Lee, Young-Ho;Chung, Young-Cho;Kim, Su-Sie;Park, Byoung-Kwan
    • Korean Journal of Biological Psychiatry
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    • v.2 no.2
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    • pp.301-305
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    • 1995
  • Subcortical dementia may occur as a complication of cerebral malaria via deep white matter demyelination with encephalitis and diffuse small vessel vasculitis. In subcortical dementia, impairment in attention and frontal executive function are predominant. Patients are often inert, indifferent, and uninterested. They appear characteristically 'slowed up' with a marked deficit in the retrieval of information. Changes in mood, personality, and social conduct are very common. We describe a case of subcortical dementia, who has definite changes in brain MRI after cerebral malaria.

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A Case of Gas Forming Enterocolitis Associated with Kawasaki Disease (가와사끼병에 동반된 Gas Forming Enterocolitis 1례)

  • Kim, Ji-Young;Yang, Tae-Jin;Song, Min-Seop;Kim, Chul-Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.4 no.2
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    • pp.233-237
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    • 2001
  • Kawasaki Disease, an acute systemic vasculitis of unknown etiology, is the leading cause of acquired heart disease in children in many parts of the world. It predominantly affects children under 5 years of age and has many clinical symptoms. We experienced a case of gas forming enterocolitis associated with Kawasaki Disease. Aeromonas hydrophilia was isolated from her stool culture. So, we report the case with a brief review of its literature.

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A Case of Nephrotic Syndrome in Behcet Syndrome (신 증후군을 동반한 베체트 증후군(Behcet syndrome) 1례)

  • Juhng Woo-Suk;Kim Hye-Young;Lee Dae-Yeol
    • Childhood Kidney Diseases
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    • v.2 no.1
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    • pp.73-76
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    • 1998
  • Behcet syndrome is a multisystem disease complex of unknown cause. It is usually manifested by aphthous oral and genital ulcers, uveitis and skin eruption. Less frequently, CNS involvement, colitis, large vessel vasculitis, and myocarditis occur. Recently, several studies have reported renal manifestations of amyloidosis and focal necrotizing glomerulonephritis in Behcet syndrome. We describe a patient with Behcet syndrome who experienced nephrotic syndrome with focal effacement of epithelial foot process in glomeruli. A brief review of literature ensues.

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The Inhibitory Effect of Bamboo Culm Extract on the Development of Pulmonary Inflammation in Pristane-Induced Lupus Mice

  • Chae, Byeong-Suk;Kim, Dae-Keun;Eun, Jae-Soon;Kwon, Gi-Sung;Shin, Tae-Yong
    • Natural Product Sciences
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    • v.16 no.4
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    • pp.245-250
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    • 2010
  • Pulmonary pathogenesis in lupus is characterized by interstitial inflammation and vasculitis in lungs. We investigated whether bamboo culm extract (BC) attenuates pulmonary inflammation and lung injury in pristane-induced lupus mice. The pristane-induced lupus mice and healthy mice were administrated with BC 0.5 ml/kg or PBS orally once a day for 14 days. Our results demonstrated that BC significantly attenuated levels of bronchoalveolar lavage (BAL) IL-6, IL-10, IFN-$\gamma$, $PGE_2$ and VEGF, and pulmonary vascular permeability in pristane-induced lupus mice. Therefore, these findings suggest that BC may inhibit development of pulmonary inflammation and lung injury in lupus.

Diffuse alveolar hemorrhage and recombinant factor VIIa treatment in pediatric patients

  • Park, Jeong A
    • Clinical and Experimental Pediatrics
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    • v.59 no.3
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    • pp.105-113
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    • 2016
  • Diffuse alveolar hemorrhage (DAH) is a life-threatening pulmonary complication in patients with hematologic malignancies or autoimmune disorders. The current treatment options, which include corticosteroids, transfusions, extracorporeal membrane oxygenation (ECMO), and immunosuppressants, have been limited and largely unsuccessful. Recombinant activated factor VII (rFVIIa) has been successfully administered, either systemically or bronchoscopically, to adults for the treatment of DAH, but there are few data on its use in pediatric patients. The current literature in the PubMed database was reviewed to evaluate the efficacy and risk of rFVIIa treatment for DAH in pediatric patients. This review discusses the diagnosis and treatment of DAH, as well as a new treatment paradigm that includes rFVIIa. Additionally, the risks and benefits of off-label use of rFVIIa in pediatric patients are discussed.

The Occurrance of Velogenic Viscerotropic Newcastle Disease Virus in an Adult Peacock (성숙 공작(Pavo cristatus)에서 발생한 내장 친화형 뉴캣슬병 바이러스 강독주)

  • 조경오;박남용;강문일;고홍범;이근우
    • Journal of Veterinary Clinics
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    • v.18 no.2
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    • pp.152-155
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    • 2001
  • A two-year-old male peacock (Pavo cristatus) showed acute watery green diarrhea, followed by neurological signs including torticollis and muscular tremor. By the hemagglutination inhibition test for detecting the antibody against the Newcastle disease virus (NDV), the peacock serum inhibited the agglutination of chicken red blood cells. Grossly distinctive hemorrhagic lesions were found in the mucosa of proventiculus and intestine and lung. The spleen revealed multiple variable sized necrotic foci. Histologically, the mucosa of gastrointestinal track had hemorrhagic lesions and some of them underwent ulceration. The spleen exhibited multiple variable sized necrotic foci in which fibrin exudation was marked. Central nervous system had mild non-suppulative menin-goencephalitis consisting of vasculitis, perivascular hemorrhage, gliosis and meningitis. The cells particularly in the cerebellum were degenerative to necrotic. Some of these nerve cells revealed characteristic peripheral chromatolysis. From the present serological and pathological findings, it is suggested that NDV causing death of peacock was velogenic viscerotropic strain. This is the first report of the occurrence of velogenic viscerotropic NDV in an adult peacock in Korea.

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Epidemiology of Kawasaki disease in Korea (한국 가와사끼병의 역학)

  • Park, Yong Won
    • Clinical and Experimental Pediatrics
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    • v.51 no.5
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    • pp.452-456
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    • 2008
  • Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and young children. Initially described in 1967 by Dr. Tomisaku Kawasaki, it is now the most common cause of acquired heart disease among children in developed nations. Although KD has been reported across all racial and ethnic groups, the incidence of KD is more common among Asians, which suggests differences of race-specific susceptibility. The prevalence of the disease varies considerably among different Asian countries, and there is a higher rate of KD reported in Asian countries such as Japan and Korea than in other countries. In Korea, a nationwide epidemiological study has been conducted every three years since the 1990s by the Korean Pediatric Heart Association to determine the epidemiologic patterns and incidence rate of KD in Korea. It was thus found in a recent survey (2003-2005) that the average annual incidence of 105.0/100,000 Korean children under the age of five years was the second-highest reported rate in the world, after Japan.