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- 저자 : 장성국 ( Sung Guk Chang ) (검색결과 2 건)
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전신성 홍반성 루푸스에 동반된 Hemophagocytic syndrome
오동호 ( Dong Ho Oh ),송용호 ( Yong Ho Song ),김창수 ( Chang Soo Kim ),김재훈 ( Jea Hoon Kim ),도준형 ( Joon Hyung Doh ),최병렬 ( Byung Reul Choi ),김상경 ( Sang Gyung Kim ),장성국 ( Sung Guk Chang ),최정윤 ( Jung Yoon Choe ) 대한류마티스학회 1999 대한류마티스학회지 Vol.6 No.3
A 45-year-old woman was admitted to our hospital because of a high fever, dyspnea, and myalgia. At the time of admission, a diagnosis of systemic lupus erythematosus(SLE) was made by fulfilling four of the 1982 American College of Rheumatology criteria with increasing levels of anti-nuclear antibody titer(speckled pattern). Prednisolone given orally in an initial dosage of 30mg/day was not effective and she was expired by respiratory failure due to disseminated intravascular coagulation. A diagnosis of hemophagocytic syndrome was made because of the increased number of unusual hemophagocytic cells in the bone marrow. High levels of serum ferritin which are known to reflect macrophage activition, supported the diagnosis of hemophagocytic syndrome. Hemophagocytic syndrome is characterized by activated phagocytosis presumably induced by hypersecretion of cytokines. Malignant lymphoma and infection are the two representative diseases which may cause hemophagocytic syndrome. Recently several acute lupus hemophagocytic syndromes were reported in patients with SLE. Here we report a case of acute lupus hemophagocytic syndrome observed in a patient with SLE with brief review of literatures.
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류마티스 관절염 환자에서 Phospholipase A2 활성도
오동호 ( Dong Ho Oh ),최정윤 ( Jung Yoon Choe ),송용호 ( Yong Ho Song ),손호상 ( Ho Sang Shon ),장성국 ( Sung Guk Chang ),김상경 ( Sang Gyung Kim ),이종명 ( Jong Myung Lee ),김능수 ( Nung Soo Kim ),장현욱 ( Hyeun Wook Chang ) 대한류마티스학회 1999 대한류마티스학회지 Vol.6 No.2
목적: Phospholipase A2(PLA2)는 세포막 구성성분인 인지질로부터 각종 eicosanoid의 전구물질인 arachidonic acid 생성에 관여하는 속도조절효소(rate-limiting enzyme)로서, 저자들은 류마티스 관절염 환자에서 PLA2의 활성도를 측정하고 이를 질병활동도와 비교검토함으로써 그 의의를 조사하였다. 방법: 대상환자군은 1987년 미국류마티스학회가 제정한 류마티스 관절염의 진단기준에 부합되는 총 25명의 환자를 대상으로 하여 그들의 혈청과 슬관절에서 뽑은 관절액내 PLA2 활성도를 측정하였다. 정상대조군은 병력이나 직장신체검사상 특별한 이상이 없는 병원직원 14명을 대상으로 하였다. 결과: 1. 류마티스 관절염 환자군에서 혈청 PLA2의 활성도는 정상대조군에 비하여 유의하게 증가되어 있었다(p<0.001). 2. 류마티스 관절염 환자에서 혈청 PLA2 활성도는 관절액에서 측정한 PLA2와 유의한 상관관계를 보였다(p<0.05). 3. 치료 3개월 후 질병활동도(임상 및 검사실 소견)의 유의한 호전(p<0.001)과 함께 혈청 PLA2 활성도는 유의하게 감소하였다(p<0.001). 4. 혈청 PLA2의 유의한 감소는 임상적 호전이 있는 군에서만 관찰되었다. 또한 혈청 PLA2 활성도는 질병활동도와 관련이 있는 각종 지표들 중, ESR과 가장 좋은 상관관계를 보였다(r=0.493, p<0.05). 결론: 류마티스 관절염 환자군에서 혈청 PLA2의 활성도는 정상 대조군과 비교하여 증가되어 있었으며 이는 관절액에서 측정한 PLA2 활성도와 유의한 상관관계를 보였고, 치료 후에는 임상증상의 호전과 함께 감소하였다. 이상의 소견으로 PLA2는 이질환의 병태생리에 관여하며 질병의 활동도를 반영 할 수 있는 지표의 하나로 생각된다. Objectives: A limited retrospective study of patients with rheumatoid arthritis(RA) found that serum phospholipase A2(PLA2) activity correlates with disease activity. To assess the strength of this relationship we investigated prospectively 25 patients with RA using a double blind approach. Methods: Twenty five patients who fulfilled the 1987 American College of Rheumatology criteria for RA had clinical and laboratory assessments. PLA2 activity was measured before and after treatment of 3 months in patients with RA. Fourteen healthy individuals were also enrolled as controls. PLA2 activity was assayed using E. coli membrane phospholipid substrate labelled with [14C]-oleic acid. Results: 1) Serum PLA2 activity was significantly higher in patients with RA than that of normal healthy controls(p<0.001). 2) In patients with RA, synovial fluid PLA2 activity was higher than serum PLA2 activity, and a positive correlation between PLA2 in synovial fluids and matched sera was found in these patients(p<0.05). 3) After treatment, PLA2 activity was significantly decreased with improvement of clinical(morning stiffness and Ritchie index) and laboratory(ESR, CRP and rheumatoid factor) assessments(p<0.001). 4) Among the clinical and laboratory markers of disease activity, ESR showed the best correlation with serum PLA2 activity(r=0.493, p<0.05). 5) In the patients who did not respond clinically to treatment (n=5), there was no significant decrease in PLA2 activity. Conclusion: PLA2 activity significantly correlates with RA activity and may serve as an index of disease activity in RA.
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