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전신홍반루푸스에서 발생한 만성 염증성 수초탈락성 다발성 신경병증
문기원 ( Ki Won Moon ),송영욱 ( Yeong Wook Song ),송란 ( Ran Song ),윤찬영 ( Chan Young Yun ),고재기 ( Jae Ki Koh ),김진현 ( Jin Hyun Kim ),이은영 ( Eun Young Lee ),이은봉 ( Eun Bong Lee ) 대한류마티스학회 2009 대한류마티스학회지 Vol.16 No.3
Chronic inflammatory demyelinating polyneuropathy (CIDP) is characterized by symmetrical weakness, impaired sensation, absent or diminished tendon reflexes, an elevated cerebrospinal fluid protein level and nerve-conduction studies that show demyelination. The occurrence of CIDP in patients with systemic lupus erythematosus (SLE) has been rarely reported. We experienced a case of a 33 year-old woman with SLE and she presented with fever, abdominal pain, a tingling sensation of both of her hands and feet, and symmetrical weakness in both the proximal and distal extremities. Her symptoms had persisted for over 1 months before she visited our department. The CSF examination showed an elevated protein level and the nerve conduction studies revealed demyelination. Her symptoms showed minimal improvement with high dose steroid and immunoglobulin therapy, but she responded to cyclophosphamide therapy.
Microbacterium sp. AL-210이 생산하는 levan fructotransferase의 효소활성에 중요한 아미노산의 동정
성희경,문금옥,최기원,최경화,황경주,김묘정,차재호,Sung, Hee-Kyung,Moon, Keum-Ok,Choi, Ki-Won,Choi, Kyung-Hwa,Hwang, Kyung-Ju,Kim, Myo-Jung,Cha, Jae-Ho Korean Society of Life Science 2007 생명과학회지 Vol.17 No.1
[ $\beta$ ]-Fructofuranosidases, a family 32 of glycoside hydrolases (GH32), share three conserved domains including the W(L/M)(C/N)DP(Q/N), FRDPK, and ECP(D/G) motifs. The functional role of the conserved acidic residues within three domains of levan fructotransferase, one of the $\beta-fructofuranosidases$, from Microbacterium sp. AL-210 was studied by site-directed mutagenesis. Each mutant was overexpressed in E. coli BL21(DE3) and purified by using Hi-Trap chelating affinity chromatography and fast performance liquid chromatography. Substitution of Asp-63 by Ala, Asp-195 by Asn, and Glu-245 by Ala and Asp decreased the enzyme activity by approximately 100-fold compared to the wild-type enzyme. This result indicates that three acidic residues Asp-63, Asp-195, and Glu-245 play a major role in catalysis. Since the three acidic residues are present in a conserved position in inulinase, levanase, levanfructotransferase, and invertase, they are likely to have a common functional role as nucleophile, transition state stabilizer, and general acid in $\beta-fructofuranosidases$. 당 분해효소의 family 32 (GH32)에 속하는 $\beta-fructofuranosidase$는 3차구조를 근거로 볼 때 W(L/M)(C/N)DP(Q/N), FRDPK, 그리고 ECP(D/G) 부위를 포함하는 세 군데의 보전적인 영역을 가지고 있다. 이러한 $\beta-fructofuranosidase$ family에 속하는 Microbacterium sp. AL-210 유래 levan fructotransferase (LFTase)의 보전적인 산성 아미노산들의 역할이 특정위치 돌연변이법으로 검사되었다. 각각의 돌연변이체는 대장균인 E. coli BL21 (DE3)균주에서 발현되어 대량 생산되었고, 금속 친화 크로마토그래피법과 FPLC법으로 순수 정제되었다. wild-type LFTase의 효소의 활성은 0.74 unit 인 반면 네 개의 돌연변이체인 D63A, D195N, E245A, E245D 각각은 specific activity를 측정해 본 결과 원 균주와 비교해서 약 100배 정도 감소한 효소활성을 보여 주었다. 이로써 아미노산 변형의 target이 되었던 Asp-63, Aps-195, 그리고 Glu-245가 모두 효소 활성 및 기질과의 결합에 상당히 중요한 역할을 하고 있음이 판명되었다. 이러한 세 부위의 산성 아미노산들은 inulinase, levan fructotransferase와 invertase에 모두 보전적으로 위치 하므로 이들은 $\beta-fructofuranosidase$ family내 에서 공통된 역할을 할 것으로 사료된다.
TNF 길항제 사용 후 결핵이 발생한 류마티스관절염 환자에서 Rituximab을 사용한 1예
김경현 ( Kyeong Hyun Kim ),문기원 ( Ki Won Moon ),허정원 ( Jeong Won Heo ),이원호 ( Won Ho Lee ),이상훈 ( Sang Hoon Lee ),정철민 ( Cheol Min Jeong ),박현선 ( Hyun Sun Park ) 대한류마티스학회 2012 대한류마티스학회지 Vol.19 No.2
Rituximab has been shown to be effective in rheumatoid arthritis (RA) and is recommended for patients exhibiting an inadequate response to tumor necrosis factor (TNF) inhibitors. To date, there have been no reports of tuberculosis in RA patients treated with rituximab. We report the use of rituximab in a TNF inhibitor-refractory RA patient who had developed tuberculosis. A 52-year-old man with RA had been treated with adalimumab for 3 months, but failed to respond well to the treatment. He reported fever, coughing, sputum, and weight loss. He was diagnosed with pulmonary tuberculosis and started anti-tuberculosis medication. His arthritis was not controlled for despite increasing the dose of prednisolone. He was treated with rituximab without serious adverse effects. Sixteen weeks later, he demonstrated improvement with both arthritis and tuberculosis.
증례 : 류마티스 ; 스테로이드 흡입제와 저용량의 cyclophosphamide로 호전된 다발성 근염에 동반된 폐쇄성 세기관지 기질화 폐렴 1예
윤찬영 ( Chan Young Yun ),송란 ( Ran Song ),문기원 ( Ki Won Moon ),이윤종 ( Yun Jong Lee ),송영욱 ( Yeong Wook Song ) 대한내과학회 2009 대한내과학회지 Vol.77 No.5
저자들은 다발성 근염과 그에 합병된 폐쇄성 세기관지 기질화 폐렴 환자를 고용량의 스테로이드와 cyclosprone A로 치료하던 중 스테로이드 근병증이 발생하여 스테로이드를 감량하게 되었고, 이후 다시 폐쇄성 세기관지 기질화 폐렴 및 근염의 악화를 보여 이에 스테로이드 흡입제와 저용량의 cyclophosphamide를 사용하여 치료한 1예를 경험하였기에 이를 문헌고찰과 함께 보고하는 바이다. Polymyositis is an inflammatory connective tissue disease involving, predominantly, skeletal muscle. Occasionally, it is complicated by serious interstitial pneumonia. The presence of interstitial pneumonia affects the prognosis and contributes substantially to the morbidity and mortality. Although the treatment recommendations for interstitial pneumonia in polymyositis are still not optimal, high-dose steroid or immunosuppressive agents such as methotrexate, azathioprine, cyclosporine A, or cyclophosphamide alone or in combination are effective in myositis-associated interstitial pneumonia in many cases. Nevertheless, the relative frequency of complications, such as opportunistic infection, steroid-induced myopathy, and steroid psychosis, limits the use of high-dose steroid or immunosuppressive regimens. Here, we describe our experience of combination treatment with a steroid inhaler and low-dose cyclophosphamide in a patient with bronchiolitis obliterans organizing pneumonia associated with polymyositis who developed steroid-induced myopathy during initial high-dose steroid and cyclosporine A treatment. (Korean J Med 77:654-658, 2009)
임경리 ( Kyoung Ree Kim ),강명호 ( Myung Ho Kang ),김태석 ( Tae Suk Kim ),문기원 ( Ki Won Moon ),류동열 ( Dong Ryeol Ryu ),이희영 ( Hui Young Lee ),한선숙 ( Seon Sook Han ) 대한결핵 및 호흡기학회 2012 Tuberculosis and Respiratory Diseases Vol.72 No.1
A 37 year old female presented with epigastric pain and weight loss over a period of 3 months. Her abdominal CT finding showed a 4.5 cm size hepatic mass and 4.3 cm size pancreatic head mass with multiple macronodules in the liver. At the same time, her chest CT revealed a 5 cm size necrotic mass in the left lower lobe of the lung with multiple bilateral pulmonary nodules. We diagnosed these lesions as tuberculosis through multiple biopsies. She was treated with anti-tuberculous medication. After taking the medications, her symptoms were improved. Twelve months later, imaging studies indicated an improvement in the patient`s health. Here we report a case report of multi-organ macronodular tuberculosis in lung, liver and pancreas.
증례 : 순환기 ; 출산 후 발생한 가역적 뇌혈관 수축 증후군 1예
이호진 ( Ho Jin Lee ),이동현 ( Dong Hyun Lee ),정호철 ( Ho Chul Jung ),문기원 ( Ki Won Moon ) 대한내과학회 2015 대한내과학회지 Vol.88 No.1
본 증례는 건강했던 젊은 여성이 출산 이후 벼락두통으로내원하였고 뇌자기공명혈관 조영술을 이용하여 RCVS로 진단한 증례로 문헌고찰과 함께 이를 보고하는 바이다. Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by development of a severe thunderclap headache withor without other acute neurological symptoms, and by multifocal or diffuse segmental vasoconstriction of the cerebral arteries thatresolves spontaneously within 3 months. Several precipitating factors have been identified; these include the use of adrenergic orserotonergic drugs and postpartum status. Diagnosis is aided by the dynamic nature of the clinicoradiological features, including a‘beads-on-a-string’ appearance of the cerebral arteries on angiography, and complete (or near-complete) resolution of the conditionevident on repeat angiography performed 3 months after initial onset. Calcium channel blockers such as nimodipine seem to relievethe severe headache within 48 h. Here, we present the case of a female who developed RCVS postpartum.