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증예(症例) : Type 2 Arnold Chiari Malformation의 산전진단 1예
류완희 ( Wan Hee Yoo ),정민 ( Min Jeong ),정영주 ( Young Ju Jeong ),김관식 ( Kwan Sik Kim ),박종관 ( Jong Kwan Park ),조성남 ( Sung Nam Cho ) 전북대학교 의과학연구소 2003 全北醫大論文集 Vol.27 No.2
Arnold-Chiari II형 기형은 뇌실확장증을 일으킬 수 있는 기형 증의 하나이며 대부분 예후가 불량하므로 산전 초음파상 뇌실확장증이 발견되면 반드시 후두와(posterior fossa)의 검사와 척추에 대한 정밀검사가 시행되어야 할 것으로 사료된다. 본원에서 뇌실확장증이 발견되어 전원된 환자에서 정밀 초음파를 통해 Arnold-Chiari II형 기형을 진단한 1예를 경험하였기에 간단한 문헌고찰과 함께 보고하는 바이다. Arnold-Chiari malformation is a complex abnormality, consisting of various combinations of brainstem and cerebellar malformations, usually associated with ventriculomegaly and spinal defects. A 29-year-old pregnant woman at 28 weeks gestation was refered after detection of ventriculomegaly. We also found myelomeningocele and indirect cranial markers: the lemon sign, the banana sign, ventriculomegaly. and then we decided this finding was Arnold Chiari II malformation. We present this cases with a brief review of literatures.
전신홍반루푸스 환자에서 IFN-α에 의한 CD4+ T 림프구의 ERK Phosphorylation의 감소
류완희 ( Wan Hee Yoo ) 대한류마티스학회 2006 대한류마티스학회지 Vol.13 No.3
Objective: CD4+ T cells from patients with systemic lupus erythematosus (SLE) display aberrant TCR signaling and IFN-α plays critical roles in the pathogenesis of SLE; however, the effects of IFN-α on disease-associated TCR signaling defects remain unknown. This study investigated the ERK phosphorylation during TCR triggering and the effects of IFN-α on ERK signaling in CD4+ T cells. Methods: CD4+ T lymphocytes were sorted from PBMC using magnetic beads in patients with SLE who met the 1982 revised ACR criteria for SLE and age-matched healthy controls. The phosphorylation of ERK 1/2 was analyzed by flow cytometry and mean fluorescent intensity was measured to define the degree of phosphorylation of ERK. In some experiments, anti-CD3 stimulation was performed after preincubation with patient or control serum, diluted in tissue culture media, with or without addition of an anti-IFN-α antibody. The serum level of IFN-α was measured by ELISA. Results: ERK-1/2 phosphorylation was decreased in CD4+ T cells of lupus patients than healthy controls and associated with disease activity. Pre-incubation of control CD4+ T cells with allogeneic lupus plasma decreased ERK-1/2 phosphorylation more than allogeneic control and RA plasma and this was reversed by anti-IFN-α Ab. Accordingly, ERK-1/2 phosphorylation was decreased in control CD4+ T cells pre-incubation with lupus plasma with high IFN-α levels more than lupus plasma with non-detectable IFN-α levels. Recombinant IFN-α inhibited TCR-mediated ERK-1/2 phosphorylation dose-dependently. Conclusion: These results suggest that IFN-α stimulation in vivo may underlie the aberrant TCR-mediated MAPK signaling in lupus CD4+ T cells and associated with disease pathogenesis.
류마티스 : 미만성 폐포출혈증후군으로 발생한 척-스트라우스 증후군의 1예
강미희 ( Mi Hee Kang ),이원석 ( Won Seok Lee ),최윤정 ( Yun Jung Choi ),오유정 ( Yoo Jeong Oh ),황주희 ( Joo Hee Hwang ),류완희 ( Wan Hee Yoo ) 대한내과학회 2015 대한내과학회지 Vol.89 No.6
Churg-Strauss syndrome (CSS), known as eosinophilic granulomatosis with polyangiitis, is a rare type of systemic vasculitis characterized by the presence of asthma, peripheral eosinophilia, and necrotizing vasculitis with eosinophilic infiltration of multiple organs. Approximately 3-4% of all CSS cases are associated with alveolar hemorrhage. Untreated CSS may lead to a poor prognosis, but glucocorticoid and cytotoxic agent treatments may result in clinical remission. The careful diagnosis and understanding of CSS is important for making treatment decisions and providing effective care. Here, we report a case of CSS with diffuse alveolar hemorrhage. (Korean J Med 2015;89:738-741)
류마티스 : 원발성 쇼그렌 증후군에서 발생한 다발성 경화증 1예
황주희 ( Joo Hee Hwang ),이원석 ( Won Seok Lee ),최윤정 ( Yun Jung Choi ),오유정 ( Yoo Jeong Oh ),강미희 ( Mi Hee Kang ),류완희 ( Wan Hee Yoo ) 대한내과학회 2015 대한내과학회지 Vol.89 No.6
Sjogren syndrome (SS) is a chronic inflammatory autoimmune disorder involving the exocrine glands, which often presents with salivary and tear gland dysfunction leading to dry mouth and eyes (sicca symptoms). This disease occurs alone as primary SS, or in the background of connective tissue diseases as secondary SS. Neurological involvement is seen in 20-25% of SS cases. Cerebral involvement is generally heterogeneous both in terms of localization (focal or diffuse) and progression (acute, progressive or reversible) and may resemble the clinical and radiological findings of multiple sclerosis (MS). Here we present the case of a patient with primary SS who experienced acute progressive diffuse MS. (Korean J Med 2015;89:733-737)
항핵항체 음성, 항-dsDNA 항체 음성, 항-Ro/SSA 항체 양성인 막성 루푸스 신염 1예
양희찬 ( Hee Chan Yang ),홍명주 ( Myong Joo Hong ),임진한 ( Jin Han Lim ),장슬아 ( Seol A Jang ),전소연 ( So Yeon Jeon ),류완희 ( Wan Hee Yoo ) 대한류마티스학회 2013 대한류마티스학회지 Vol.20 No.2
전신부종으로 내원하였던 환자로 항핵항체와 항-dsDNA 항체가 음성이면서 ACR 진단기준을 만족시키지 못하였으나 신장 조직검사 상에서 SLE가 진단되었고 스테로이드와 면역억제제 치료 후에 증상이 호전되었다. 이러한 비전형적인 혈청학적 검사 소견을 보이는 환자의 진단을 위해서는 의심되는 임상증상이 있는 경우 지속적인 추적관찰이 필요할 것으로 사료된다. Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of diverse autoantibodies with various systemic organ involvements. In patients with SLE, autoantibodies, such as antinuclear antibody (ANA) and anti-dsDNA antibody, play an important role not only in diagnosing the disease, but also representing the pathogenesis of the disease. ANA is the main screening tool in diagnosis and serum complement levels and anti-dsDNA antibody level are closely related to the disease activities. Nevertheless, exceptionally, some patients represent with negative ANA and/or anti-dsDNA antibody leading to difficulties in diagnosing the disease. Here, we report a case of 37-year old female SLE patient with negative ANA, negative anti-dsDNA antibody, and positive anti-Ro/SSA antibody, which manifested with nephrotic syndrome.
저산소증에 의한 활막 섬유모세포의 ICAM-1 발현에 대한 항산화제의 영향
김정렬,류완희,Kim, Jung Ryul,Yoo, Wan Hee 대한면역학회 2002 Immune Network Vol.2 No.1
Background: Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by synovial hyperplasia and joint destruction. The synovial fibroblasts express cell adhesion molecules and have a role in adhesive interation with inflammatory cells in synovial tissue. It has been suggested that hypoxic conditioins are thought to exist in arthritic joints, and several studies indicate that reactive oxygen species (ROS) produced in hypoxic condition can initiate events that lead to pro-adhesive changes via increased expression of adhesion molecules. So, this study wsa designed to examine whether antioxidant can inhibit hypoxia-induced expression of ICAM-1 in cultured human synovial fibroblasts. Methods: Synovial fibroblasts were isolated from synovial tissue in patients with RA and cultured at hypoxic condition. Antioxidant, PDTC (pyrrolidine dithiocarbamate) were pre-treated for an hour before the hypoxic culture and synovial fibroblasts were harvested at 0, 6, 12, 24, 48 hours time points. Cell surface ICAM-1 expression in synovial fibroblasts was examined by the flow cytometric analysis. To analyse the expression of ICAM-1 mRNA, reverse-transcriptase polymerase chain reaction (RT-PCR) was performed. The levels of cytokines in culture supernatants were measured by ELISA, and activation of NF-${\kappa}B$ was analysed by electrophoretic mobility shift assay. The adhesive reaction between synovial fibroblasts and lymphocytes was assayed by measurement of fluorescent intensity of BCECF-AM in lymphocytes. Results: Hypoxic stimuli up-regulated the ICAM-1 expression as well as the adhesive interaction of human synvial fibroblasts to lymphocytes in a time-dependent manner, and PDTC inhibited hpyoxia-induced ICAM-1 expression and cell-cell interaction. PDTC also inhibited the hypoxia-induced activation of intracellular transcription factor, NF-${\kappa}B$. PDTC decreased the amount of hypoxia-induced production of IL-$1{\beta}$ and TNF-${\alpha}$. Conclusion: These studies demonstrate that PDTC inhibit the hypoxia-induced expression of the adhesion molecule, ICAM-1 and activation of NF-${\kappa}B$ in cultured human synovial fibroblasts.