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루푸스 환자에서 비전형적 용혈요독증후군에 합병된 사지 말단부 괴저
이혜연 ( Hea Yon Lee ),장진선 ( Jin Sun Jang ),김형욱 ( Hyung Wook Kim ),김영수 ( Young Soo Kim ),김영옥 ( Young Ok Kim ),윤선애 ( Sun Ae Yoon ) 대한신장학회 2010 Kidney Research and Clinical Practice Vol.29 No.3
Atypical hemolytic uremic syndrome (HUS) in adults is a life-threatening disorder characterized by acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia, which is not related to E. coli. Digital gangrene is one of the grave signs in emergency medicine because it requires prompt evaluation and treatment. We describe a 37 year-old Thailand woman, initially treated for suspected Neisseria septicemia, who went on to develop renal complications, thrombocytopenia and hemolytic anemia, which made the diagnosis of atypical hemolytic uremic syndrome. The patient was complaining of dysesthesia on all extremities and severe pain in both legs. Serologically, she was diagnosed as systemic lupus erythematosus (SLE). She was treated with plasma exchanges using fresh frozen plasma, parenteral steroid, anticoagulant and antibiotics, successfully. This is the first report of digital gangrene complicated by atypical HUS in a patient with SLE in Korea.
김진진 ( Jin Jin Kim ),권순석 ( Soon Seog Kwon ),이현정 ( Hyun Jeong Lee ),이혜연 ( Hea Yon Lee ),정명희 ( Myung Hee Jeong ),김용현 ( Yong Hyun Kim ) 대한결핵 및 호흡기학회 2009 Tuberculosis and Respiratory Diseases Vol.67 No.4
The incidence of pulmonary thromboembolism increases with age. The risk factors of pulmonary thromboembolism include surgery, malignancy, obesity, lupus anticoagulants, and vascular conditions such as deep vein thrombosis. Thromboembolism in younger patients or in unusual locations, the possibility of primary thrombophilic conditions should be considered. Primary thrombophilic states include myeloproliferative disorders (MPD). JAK2 V617F mutation is found commonly in patients diagnosed with MPD, in 90∼95% of polycythemia vera (PV) and in 50∼60% of essential thrombocytosis (ET) patients. Sometimes the JAK2 V617F mutation is found in cases without MPD. The relationship between JAK2 V617F mutation and thrombosis has not been defined. Recently, clinical evidence suggests that this mutation may be variably associated with thrombosis. We present one case of pulmonary thromboembolism in a young patient, who was positive for the JAK2 V617F mutation and did not have MPD.
즐례 : 골수형성이상증후군에 속발한 비전형 소견의 폐포단백증 1예
김재영 ( Jae Young Kim ),김석찬 ( Seok Chan Kim ),강지영 ( Ji Young Kang ),이혜연 ( Hea Yon Lee ),김유진 ( Yoo Jin Kim ),김예니 ( Yae Ni Kim ),한지원 ( Ji Won Han ) 대한내과학회 2014 대한내과학회지 Vol.86 No.1
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of lipoproteinaceous material in the distal air spaces. Secondary forms of PAP are caused by underlying diseases, in particular tumorous conditions, the most common of which are hematologic malignancies, such as chronic myeloid leukemia and myelodysplastic syndrome (MDS). We herein describe a case of atypical manifestation of PAP caused by MDS, which initially mimicked military tuberculosis with severe thrombocytopenia because of radiologic features showing necrotic mediastinal lymphadenopathies and diffuse bronchiolitis. A 46-year-old male visited the hospital complaining of coughing and general weakness and was presumptively diagnosed with military tuberculosis combined with MDS. He was treated with antituberculous medication, but the lung lesions did not improve and dyspnea only progressed. Finally, he underwent lung biopsy and was diagnosed with atypical pattern of PAP associated with MDS. (Korean J Med 2014;86:74-78)