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고용량의 면역 글로불린 주사 치료로 호전된 Salmonella 그룹 D 균혈증과 골수섬유증을 동반한 전신성 홍반성 루푸스
이혜순 ( Hye Soon Lee ),배상철 ( Sang Cheol Bae ),윤형란 ( Hyung Ran Yun ),이지현 ( Ji Hyun Lee ),정유성 ( Yu Seong Jeong ),배윤상 ( Yun Sang Bae ),김태환 ( Tae Hwan Kim ),전재범 ( Jae Bum Jun ),정성수 ( Sung Soo Jung ),이인홍 ( I 대한류마티스학회 2000 대한류마티스학회지 Vol.7 No.1
A 22-year-old woman presented with fever and pancytopenia. One year ago, she was diagnosed as Salmonella group D bacteremia and myelofibrosis associated with SLE at another hospital. She was placed on high dose steroid, however, there was no improvement. Two months ago, she was diagnosed as recurrent Salmonella group D bacteremia. After admission to our hospital, she was placed on intravenous antibiotics and high dose intravenous immunoglobulin. A significant improvement in laboratory and clinical condition occurred and bone marrow biopsy showed complete resolution of fibrosis. We report a case of SLE with myelofibrosis and recurrent Salmonella group D bacteremia.
류마티스 관절염 환자에서 HLA-DRB1의 유전적 변이와 Etanercept 치료 반응과의 연관성: 예비 연구
윤혜련 ( Hye Ryeon Yun ),강창수 ( Chang Soo Kang ),이경화 ( Kyung Wha Lee ),이혜순 ( Hye Soon Lee ),김태환 ( Tae Hwan Kim ),배상철 ( Sang Cheol Bae ) 대한류마티스학회 2006 대한류마티스학회지 Vol.13 No.1
Objective: To investigate the roles of genetic variation in the HLA-DRB1 as predictors of response to etanercept treatment in rheumatoid arthritis (RA) patients. Methods: Clinical responses of 66 patients treated with etanercept were determined according to the ACR criteria (ACR20 and 70). HLA-DRB1 typing and further subtyping of all alleles were performed by polymerase chain reaction, sequence-specific oligonucleotide probe hybridization, and direct DNA sequencing analysis. We tested whether genetic variation in the HLA-DRB1 influenced on the responses to 12 weeks of etanercept therapy. Univariate and multivariate analyses were performed to compare allele and genotype distribution between responders and nonresponders. Results: When allelic association with etanercept response was analyzed with ACR20 and ACR 70 criteria for shared epitope alleles (HLA-DRB1 *0101, *0401, *0404, *0405, *0410, *1001, and *1406 alleles) and protective alleles (HLA-DRB1*0701, *0802, *1301, *1302, *1403, and *1405 alleles), there was no association with etanercept efficacy. When ACR20 nonresponders were compared with ACR70 responders, there was no significant association. Next, we tested genotypic association for shared epitope carriage status. The presence of HLA-DRB1 alleles encoding the shared epitope (1 and 2 copies) was marginally associated with nonresponse effect for ACR 70 response (OR=0.27, 95% CI=0.08∼0.93, P=0.045). Conclusion: There was no influence of genetic variation in the HLA-DRB1 on the response to treatment of RA with etanercept.
류마티스 관절염과 전신성 홍반성 루푸스가 공존하는 Rhupus 증후군의 임상적 고찰
윤형란 ( Hyung Ran Yun ),전재범 ( Jae Bum Jun ),이명호 ( Myong Ho Lee ),이혜순 ( Hye Soon Lee ),이지현 ( Ji Hyun Lee ),김태환 ( Tae Hwan Kim ),정성수 ( Sung Soo Jung ),이인홍 ( In Hong Lee ),배상철 ( Sang Cheol Bae ),유대현 ( Dae 대한류마티스학회 1999 대한류마티스학회지 Vol.6 No.4
Objective: This study was designed to evaluate clinical features of 20 patients with rhupus syndrome and compare its characteristics with systemic lupus erythematosus(SLE) and rheumatoid arthritis(RA). Methods: Patients considered to have rhupus who met the American College of Rheumatology(ACR) 1997 and 1987 revised criteria for SLE and RA, respectively and age, sex, and disease duration matched 64 patients with RA and 56 patients with SLE were selected for comparison. Results: Twenty patients were all female and their mean age was 43.7±9.6 years (range 25~68). They had 5.5 ACR criteria for RA and 5 criteria for SLE. The mean age at onset of RA was 35.2±10.5 years(19~63) and that of SLE was 38.2±10.0 years(20~63), giving a mean interval between the diagnoses of the two diseases of 3.0±5.7 years (14~(-6)). There were 2 patients(10%) with rheumatoid nodule, and 18 patients(90%) with rheumatoid factor, and 16 patients(80%) with bony erosions on hand or wrist joints. The criteria for SLE included malar rash(20%), discoid rash(0%), photosensitivity(30%), oral ulcer(45%), arthritis(100%), serositis(35%), renal disorder(15%), neurologic disorder(0%), hematologic disorder(100%), immunologic disorder(90%), and positive antinuclear antibody(100%). Anti-dsDNA was more than 7.0U/ml in 15 patients(75%). The patients with rhupus syndrome showed lower amount of 24 hour urine protein, more severe radiologic involvement, younger age at onset of arthritis, higher titer of rheumatoid factor, lower frequency of low C3, and less complicated clinical course when compared with the patients with SLE(p<0.05). More frequent anemia, Rayanud`s phenomenon, and more complicated clinical course in rhupus when compared with the patients with RA(p<0.05), but the radiologic stage of hand and wrist was similar between rhupus and RA. Conclusion: Rhupus syndrome showed something different clinical characteristics and clinical course when compared with SLE and RA.
김태종 ( Tae Jong Kim ),오승일 ( Seung Il Oh ),박준성 ( Joon Sung Park ),김태엽 ( Tae Yeob Kim ),박재홍 ( Jae Hong Park ),엄완식 ( Wan Sik Uhm ),이혜순 ( Hye Soon Lee ),전재범 ( Jae Bum Jun ),정성수 ( Sung Soo Jung ),배상철 ( Sang 대한류마티스학회 2003 대한류마티스학회지 Vol.10 No.1
Objective: To study the clinical features and outcomes in 10 cases of secondary amyloidosis with rheumatic disease. Methods: The secondary amyloidosis associated with rheumatic disease was studied clinically in 10 patients (4 men and 6 women), who have suspicious tissue dysfunction. The diagnosis of amyloidosis was established by histological examination of biopsy materials. We evaluated them by medical record review; clinical features at onset, organ involvement, cause of death. Results: The mean age and mean duration of rheumatic disease were 52.9±17.27 years and 10.9±10.33 years respectively. The common initial clinical features were generalized edema, diarrhea and abdominal pain. The mainly involved organs were kidney and colon. Causes of death were DIC due to sepsis and renal failure. Conclusion: Renal and gastrointestinal symptoms are cardinal features of secondary amyloidosis with rheumatic disease in Korea.
이민규 ( Min Kyu Lee ),김병식 ( Byung Sik Kim ),정석현 ( Suk Hyun Jung ),이건화 ( Gun Hwa Lee ),김진옥 ( Jin Ok Kim ),임동휘 ( Dong Hwi Rim ),이유화 ( Yu Hwa Lee ),김웅준 ( Woong Jun Kim ),방소영 ( So Young Bang ),이혜순 ( Hye Soo 대한류마티스학회 2012 대한류마티스학회지 Vol.19 No.3
Klinefelter`s syndrome which is characterized by hypogonadism with karyotype abnormality (47 XXY or 46 XY/47 XXY) in males has been reported to be associated with autoimmune diseases including rheumatoid arthritis and systemic lupus erythematosus. However, Klinefelter`s syndrome accompanying with polymyositis has rarely been reported. We report a case of KFS with polymyosits in a 38-year old man for the first time in Korea.
이혜순,김명환,서동진,이성구,최호순,민영일,고문수,송일한,한덕종,최승목 대한내과학회 1996 대한내과학회지 Vol.51 No.2
An epidermoid cyst which is a beign tumor with a stratified squamous epithelium that lacks hair or skin appendage have been described as the most frequent tumorous malformation of central nervous system. Also epidermoid cyst may develop at any other organ, i.e. spleen, pancreas, testis, scalp, skull, vaginal wall, or epiglottis. Almost of epidermoid cyst were diagnosed postoperatively because they have no specific radiologic finding and have difficulty in diagnosis by percutaneous needle aspiration. We report a case of epidermoid cyst which was diagnosed preoperatively as pancreatic head mass but was diagnosed postoperatively as epidermoid cyst at retroperitoneum separated from pancreas.