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장현규 ( Hyun Kyu Chang ) 대한류마티스학회 2003 대한류마티스학회지 Vol.10 No.2
Behcet`s disease is a chronic inflammatory disease with various organ involvements, its pathogenesis being unclear. It runs the clinical course of unpredictable exacerbations and remissions. In addition to a triple syndrome of oral ulceration, genital ulceration, and uveitis, there may be the involvement of other systems, such as joints, gastrointestinal tract, central nervous system, vessels, heart, or lungs. Although we have more information about the agents, through the controlled studies, that have been already available to us, the considerable portions of managements have been still done empirically. In addition, the major goal of treatment is to suppress an inflammatory status of the disease and therapy should be tailored according to the disease activity and involved organ. In these days, promising data have also been accumulated in the tumor necrosis factor inhibitors, especially in Behcet`s patients with severe manifestations refractory to conventional treatments.
장현규 ( Hyun Kyu Chang ),허승호 ( Seung Ho Hur ),정행섭 ( Haing Sub R. Chung ) 대한류마티스학회 2000 대한류마티스학회지 Vol.7 No.1
Giant cell arteritis is an inflammatory disease that mainly involves the cranial branches of the arteries originating from the aortic arch. It affects the white populations over the age of 50 almost exclusively who live in Northern Europe and in the United states. There is close relationship between giant cell arteritis and polymyalgia rheumatica. So far, there have been a few reports of temporal arteritis in Korea. However, giant cell arteritis with typical pathological findings in temporal artery has not been reported. We describe a case who showed typical pathological findings of giant cell arteritis in superficial temporal artery. She had been diagnosed as polymyalgia rheumatica in several months ago.
장현규 ( Hyun Kyu Chang ) 대한류마티스학회 2004 대한류마티스학회지 Vol.11 No.3
Behcet`s disease (BD) is a chronic inflammatory disorder of unknown cause, characterized by recurrent oral ulcerations, genital ulcerations, ocular and skin lesions. Although the exact pathogenesis for BD is not completely understood, it has been suggested that the disease is triggered in genetically susceptible individuals by environmental factors, such as microbial agents. It is noted that multiple genes, including MHC and non-MHC genes, are implicated in the pathogenesis of BD. Although the HLA-B51 is known to be the candidate gene showing the strongest association with BD, it is necessary to be determined whether this HLA molecule is directly involved in the pathogenesis of BD. Cross-reactivity between microbial 65-kD and human 60-kD heat shock proteins is demonstrated to cause an increased T cell (particularly γδ T cell) response. The resultant overexpression of pro-inflammatory cytokines (mainly Th-1 type) from several immune cells seems to be responsible for the enhanced inflammatory reaction, and this may be associated with the genetic factors.
장현규 ( Hyun Kyu Chang ),김승용 ( Seung Yong Kim ),박양순 ( Yang Soon Park ) 대한류마티스학회 2000 대한류마티스학회지 Vol.7 No.4
Hepatic involvement of systemic lupus erythematosus (SLE) has been considered to occur rarely. Viral hepatitis or previous treatment with potentially hepatotoxic drugs has usually been implicated as the major causes of liver diseases in patients with SLE. After careful exclusion of these etiologies, the difference between the hepatic involvement of SLE and autoimmune hepatitis is not clear due to similarities in clinical and biochemical features. We describe a 37-yearold female with SLE who simultaneously satisfied the criteria of autoimmune hepatitis.
항-RNP 항체가 있는 전신성 경화증 환자에서 Sulindac 투여 후 발생한 담즙울체성 간염과 급성췌장염
장현규 ( Hyun Kyu Chang ),김연석 ( Yeon Suk Kim ),정훈 ( Hoon Jeong ),박성오 ( Sung Oh Park ),김수희 ( Su Hee Kim ),고혁 ( Hyuk Ko ),강길현 ( Gil Hyun Kang ),김정욱 ( Jeong Uk Kim ) 대한류마티스학회 2000 대한류마티스학회지 Vol.7 No.2
Sulindac is a commonly used nonsteroidal anti-inflammatory drug (NSAID). It is relatively economical arid seems to be less nephrotoxic due to its property of minimal renal prostaglandin inhibition. Although hepatic injury has been infrequently reported for nearly all NSAIDs currently on the market, sulindac has more commonly been associated with hepatotoxicity. In addition, it has been known that sulindac is the agent to have a definite association with pancreatitis. We describe a 37-year-old woman who developed cholestatic hepatitis and acute pancreatitis in association with sulindac. This patient met the diagnostic criteria of both mixed connective tissue disease and systemic sclerosis, in which the disease classification needs to be clarified. While being treated with sulindac, close observation and periodic liver function test seem to play an important role.
다발성 식도궤양과 회장말단부궤양을 동반한 베체트병 2예
장현규 ( Hyun Kyu Chang ),김연석 ( Yeon Suk Kim ),김완수 ( Wan Soo Kim ),정행섭 ( Haing Sub R. Chung ),정승문 ( Seung Mun Jung ) 대한류마티스학회 1999 대한류마티스학회지 Vol.6 No.3
Behcet`s disease is a chronic polysymptomatic disease of recurrent systemic vasculitis. The etiology remains unclear. This disease affects several organs of the body concurrently or consecutively and sometimes has a serious outcome, depending upon the system involved. Though gastrointestinal symptoms are relatively common in Behcet`s disease, ulcerative change of the intestine is infrequent. The most common sites for the intestinal Behcet`s disease are terminal ileum and cecum. Only a few literatures report the esophageal ulcers in Behcet`s disease. We describe 2 cases of Behcet`s disease who have multiple ulcers in esophagus and terminal ileum. Esophageal ulcers have improved with low-dose prednisolone, colchicine and sulfasalazine in these patients.
원발성 담즙성 견경변증과 Isolated pulmonary hypertension을 동반한 미만성 경피증(Diffuse scleroderma)
장현규 ( Hyun Kyu Chang ),정상식 ( Sang Sig Chung ),정행섭 ( Haing Sub R. Chung ),이성희 ( Sung Hee Lee ),허환 ( Hwan Herr ) 대한류마티스학회 1997 대한류마티스학회지 Vol.4 No.2
Primary biliary cirrhosis is frequently associated with a variety of disorders presumed to be autoimmune in nature, such as Sjogren`s syndrome, scleroderma, rheumatoid arthritis, systemic lupus erythematosus, and autoimmune thyroiditis. Scleroderma has been recognized in association with primary biliary cirrhosis. Most cases present as the CREST (calcinosis cutis, Raynaud`s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. Isolated pulmonary hypertension develops in a small proportion of patients, nearly all of whom have limited cutaneous involvement. We report a case who has diffuse scleroderma associated with primary biliary cirrhosis and isolated pulmonary hypertension.
장현규 ( Hyun Kyu Chang ),정승문 ( Seung Mun Jung ),정행섭 ( Haing Sub R. Chung ) 대한류마티스학회 1998 대한류마티스학회지 Vol.5 No.1
The antiphospholipid syndrome is characterized by arterial thrombosis, venous thrombosis, pregnancy wastage, and thrombocytopenia associated with a persistently positive lupus anticoagulant and/or moderate to high positive anticardiolipin antibodies(IgG or IgM). The antiphospholipid antibodies have been detected in many medical conditions, but the antiphospholipid syndrome (APS) has mainly been restricted to the primary antiphospholipid syndrome and APS associated with systemic lupus erythematosus. Rarely, the APS has been reported in other autoimmune disorders in the literature. We describe a woman with a limited form of scleroderma and the APS manifested by complete occlusion of left axillary artery with probable thrombotic occlusive nature, thrombocytopenia, prolonged aPTT, and persistently positive lupus anticoagulant.
장현규 ( Hyun Kyu Chang ),김정욱 ( Jeong Uk Kim ) 대한류마티스학회 2000 대한류마티스학회지 Vol.7 No.1
Objective: Behcet`s disease (BD) is a chronic inflammatory disorder affecting several organs. The etiology of BD remains unclear, although genetic factors, infectious agents, and immune mechanisms have been studied. The association of BD with certain genetic factors, especially HLA-B51 antigen, is well known in some geographical areas. Nevertheless, the familial occurrence of BD is rare. In this paper, HLA phenotype was evaluated in one family member showing the clustering of BD. Methods: The serological tissue typing of HLA class I and class II antigens was performed by standard National Institutes of Health microlymphocytotoxicity method in seven family members in which four siblings were affected by BD. The diagnosis of BD was established by the criteria of the International Study Group of BD in these four siblings. Results: In this family study, all members had HLA-A2 and DQ3 antigens. Although HLA-B51 antigen was positive in six out of seven family members, BD was developed in three of the six having HLA-B51 antigen. Three siblings had the exact same HLA phenotype. However, only one person had BD among three siblings with identical HLA phenotype. In addition, all siblings who developed erythema nodosum-like lesion had HLA-B51 antigen. Conclusion: This family suggests that the familial clustering of BD may not be explained solely by a susceptible HLA phenotype. The environmental factor or other genetic factors besides HLA-B51 might play a role in the development of BD. Furthermore, more studies and information will be needed to clarify the role of A2 and DQ3 antigens in BD.