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임철현 ( Churl Hyun Im ),이은봉 ( Eun Bong Lee ),강은하 ( Eun Ha Kang ),박정진 ( Jeong Jin Park ),김만호 ( Man Ho Kim ),송영욱 ( Yeong Wook Song ) 대한류마티스학회 2004 대한류마티스학회지 Vol.11 No.4
Systemic sclerosis is a rare, autoimmune, connective tissue disorder which involves skin and other internal organs. However, central nervous system involvement including seizure, is a very rare event. This 29-year-old woman experienced repeated episodes of typical generalized tonic clonic seizure. T2 weighted and flare images of magnetic resonance image showed a mass-like high signal intensity lesion in the left temporal lobe. Atypical spikes and waves were present in a eclectroencephalogram. Her seizure episodes were controlled with anticonvulsants, corticosteroids and immunosuppresants. We report a rare case of systemic sclerosis which showed central nervous system involvement.
증례 : 류마티스 ; Sweet 증후군으로 발현한 파종성 임균감염
김준완 ( Joon Wan Kim ),송란 ( Ran Song ),임철현 ( Churl Hyun Im ),김진현 ( Jin Hyun Kim ),이은영 ( Eun Young Lee ),이은봉 ( Eun Bong Lee ),송영욱 ( Yeong Wook Song ) 대한내과학회 2011 대한내과학회지 Vol.80 No.4
Sweet 증후군은 특발성 이외에도 악성종양, 염증장병, 감염, 임신, 약물 등과 관련되어 발생할 수 있다. 파종성 임균 감염은 발열, 백혈구 증가, 동통을 동반한 피부 발진 등의 임상양상과 피부 조직소견이 Sweet 증후군과 유사할 수 있다. 본 저자들은 전형적인 피부 소견을 보이지 않고 Sweet 증후군으로 발현한 파종성 임균감염을 경험하였기에 문헌고찰과 함께 보고하는 바이다. The salient features of Sweet syndrome, also known as acute febrile neutrophilic dermatosis, are pyrexia, elevated neutrophil count, painful red papules, nodules, plaques, and an infiltrate that consists mainly of mature neutrophils diffusely distributed in the upper dermis. In addition to idiopathic forms of the syndrome, malignancies, inflammatory bowel disease, infections, pregnancy, and drugs have been associated with Sweet syndrome. Disseminated gonococcal infection (DGI) results from bacteremic dissemination of Neisseria gonorrhoeae. The most common presentation of DGI is arthritis-dermatitis syndrome. About 75% of DGI patients display a characteristic dermatitis consisting of discrete papules and pustules, often with a hemorrhagic component. We report a case of DGI presenting as Sweet syndrome. (Korean J Med 2011;80:486-489)
박정진 ( Jeong Jin Park ),이정찬 ( Jung Chan Lee ),김진현 ( Jin Hyun Kim ),임철현 ( Churl Hyun Im ),강은하 ( Eun Ha Kang ),배영덕 ( Young Deok Bae ),이윤종 ( Yun Jong Lee ),이은봉 ( Eun Bong Lee ),송영욱 ( Yeong Wook Song ) 대한류마티스학회 2005 대한류마티스학회지 Vol.12 No.3
Objective: To investigate the clinical characteristics of relapsing polychondritis (RP) in Korea. Methods: We retrospectively reviewed 16 patients with RP who were diagnosed at Seoul National University Hospital and Gyeongsang National University Hospital in the period from July 1987 to August 2003. Results: There were 9 males and 7 females, the mean age was 45.4±12.8 years. The frequency of clinical manifestation was as follows; auricular chondritis (81.3%), nasal chondritis (68.8%), ocular inflammation (56.3%), respiratory tract involvement (56.3%), arthritis (50%) and audio-vestibular damage (25%). Westergren erythrocyte sedimentation rate (ESR) was elevated in 53.3% and antinuclear antibody (ANA) was positive in 20% of study patients. Rheumatoid factor was negative in all cases. Conclusion: Clinical manifestations were comparable to the reports of Western countries. However, the frequency of arthritis was lower in Korean patients with RP than Western reports.
증례 : 류마티스 ; 파미드로네이트 불응성 다발 장기 전이성 석회화를 동반한 고칼슘혈증
배기범 ( Gi Bum Bae ),은정수 ( Jung Su Eun ),김나리 ( Na Ri Kim ),임철현 ( Churl Hyun Im ),남언정 ( Eon Jeong Nam ),강영모 ( Young Mo Kang ) 대한내과학회 2013 대한내과학회지 Vol.85 No.5
저자들은 외상으로 인한 골절 수술 이후 중증 고칼슘혈증과 다발 장기의 전이성 석회화가 동반된 파제트병이 의심되는 환자에서 고칼슘혈증, 다발 장기 전이성 석회화와 신부전이 파미드로네이트 치료에 부분적인 반응과 재발 소견이 반복되었으나 졸레드로닉산에 극적인 치료효과를 나타낸 증례를 보고하는 바이다. 중증 고칼슘혈증과 다발 장기 전이성 석회화가 동반된 파제트병은 현재까지 세계적으로 문헌보고가 없는 상태이다. 중증 고칼슘혈증과 다발 장기 전이성 석회화가 동반된 파제트병 환자에서 지속적인 임상적 호전을 위해서는 뼈 대사 억제 치료로서 졸레드로닉산 사용을 고려해 볼 수 있을 것으로 사료된다. Paget`s disease of bone (PDB) is a disorder featuring high-level bone turnover associated with the presence of disorganized and immature bone tissue with excessive levels of fibrosis. The risk of deformity is very high. The etiology of PDB is not well understood, but includes both genetic and environmental factors among which is bone trauma. Hypercalcemia can occur as a complication of PDB in patients who are immobilized and dehydrated. However, to date, no case of severe hypercalcemia with metastatic calcifications in multiple organs has been reported in any PDB patient. The drugs of choice for treatment of PDB are bisphosphonates. These drugs effectively suppress bone turnover. Patients with extensive PDB may require higher doses of bisphosphonates, and acquired resistance to a particular bisphosphonate may be overcome by use an alternative drug. Here, we report a case of suspicion of PDB. The patient presented with hypercalcemia and metastatic calcifications and his condition improved dramatically after treatment with zoledronic acid. (Korean J Med 2013;85:545-550)
류마트스 : 항인지질 증후군을 동반한 전신홍반루푸스에서 진단된 May-Thurner 증후군
김지훈 ( Ji Hun Kim ),강종완 ( Jong Wan Kang ),배기범 ( Gi Bum Bae ),이종민 ( Jong Min Lee ),임철현 ( Churl Hyun Im ),남언정 ( Eon Jeong Nam ),강영모 ( Young Mo Kang ) 대한내과학회 2012 대한내과학회지 Vol.82 No.6
항인지질 증후군이 동반된 전신홍반루푸스 환자에서 May-Thurner 증후군이 진단된 예는 아직 세계적으로 보고가 없다. 본 증례와 같이 전신홍반루푸스를 가진 젊은 환자에서 좌측 하지에 심부정맥 혈전증이 발생한 경우 항인지질 증후군을 감별하여야 하며 동시에 May-Thurner 증후군도 감별진단에 포함시켜야 한다. 그리고 진단 이후의 적극적인 혈관중 재시술과 지속적인 항응고제 치료를 통해 재발을 방지하기 위한 전략이 필요할 것이다. May-Thurner syndrome, which refers to an iliofemoral venous thrombosis caused by chronic compression of the left common iliac vein by the right common iliac artery, usually manifests as left lower extremity pain and swelling. The syndrome is particularly evident in patients with health conditions including obesity, smoking, pregnancy, surgery, or prolonged immobility. Antiphospholipid syndrome consists of arterial or venous thrombosis and the association of lupus anticoagulant or anticardiolipin antibodies. Most common clinical manifestations include pregnancy loss and deep vein thrombosis. We experienced a rare case of May-Thurner syndrome concurrent with antiphospholipid syndrome, secondary to systemic lupus erythematosus. The patient was treated successfully by catheter-directed percutaneous thrombectomy, venous thrombolysis, and stent insertion, followed by oral anticoagulant therapy.
최효진 ( Hyo Jin Choi ),신기철 ( Ki Chul Shin ),배영덕 ( Young Deok Bae ),이정찬 ( Jung Chan Lee ),김진현 ( Jin Hyun Kim ),강은하 ( Eun Ha Kang ),임철현 ( Churl Hyun Im ),이은봉 ( Eun Bong Lee ),송영욱 ( Yeong Wook Song ) 대한류마티스학회 2004 대한류마티스학회지 Vol.11 No.1
1. Protein-losing enteropathy is a rare complication of autoimmune diseases. We report a case of primary Sjogren`s syndrome with protein-losing enteropathy in 50-year-old female who complained of generalized edema. Protein-losing enteropathy of the small intestine was demonstrated by Tc-99m labeled albumin abdominal scintigraphy. Duodenal biopsies showed chronic inflammatory cell infiltration without lymphangiectasis or vasculitis. The patient received oral prednisolone (60 mg/day) for 4 weeks with subsequent clinical improvement. Primary Sjogren`s syndrome or other autoimmune diseases should be considered in cases of protein-losing enteropathy.
증례 : 류마티스 ; 원위신세관산증이 선행한 전신홍반루푸스
강진영 ( Jin Young Kang ),강종완 ( Jong Wan Kang ),김지훈 ( Ji Hun Kim ),진미경 ( Mi Kyung Jin ),임철현 ( Churl Hyun Im ),남언정 ( Eon Jeong Nam ),강영모 ( Young Mo Kang ) 대한내과학회 2011 대한내과학회지 Vol.81 No.4
원위신세관산증은 세관내 산 분비의 저하로 소변의 산성화 장애와 전신적인 산혈증 및 저칼륨혈증을 특징으로 하는 질환이다. 루푸스를 비롯한 자가면역질환에 동반되는 원위신세관산증은 대부분 자가면역질환 발현 후에 나타나며 원위신세관산증이 먼저 발생하고 자가면역질환이 수개월에서 수년 후 발생하는 경우는 세계적으로 매우 드물게 보고되어 있다. 저자들은 원위신세관산증으로 진단되고 21년 후에 전형적인 루푸스 임상 증상들이 나타난 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Distal renal tubular acidosis (RTA) is characterized by a decreased net H+ secretion in the collecting tubules, which results in a failure of urine acidification and results in metabolic acidosis, hypokalemia, and nephrocalcinosis. The acquired form of distal RTA is associated with tubulointerstitial involvement of immune-mediated disorders such as Sjogren`s syndrome and systemic lupus erythematosus (SLE). Only a few case reports have indicated that distal RTA precedes SLE by months to years. We present a 39-year-old woman who had manifestations of distal RTA for 21 years before the development of overt symptoms of SLE. (Korean J Med 2011;81:533-536)