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Henoch-Schonlein 자반증 임상양상으로 나타나 진단이 늦어졌던 웨게너 육아종증
송세빈 ( Se Bin Song ),최혜숙 ( Hye Sook Choi ),김이형 ( Yee Hyung Kim ),최천웅 ( Cheon Woong Choi ),박명재 ( Myung Jae Park ),유지홍 ( Jee Hong Yoo ),강홍모 ( Hong Mo Kang ),김윤화 ( Yoon Hwa Kim ),박주철 ( Joo Cheol Park ) 대한결핵 및 호흡기학회 2007 Tuberculosis and Respiratory Diseases Vol.63 No.6
Wegener`s granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener`s granulomatosis are indistinguishable from those of vasculitis such as Henoch-Schonlein purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-Schonlein purpura, was confirmed as Wegener`s granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature. (Tuberc Respir Dis 2007;63:531-536)
G 단백질 변이를 동반한 McCune - Albright 증후군 1 예
민헌기,윤현구,한인권,장학철,임창훈,박찬문,한기옥,강영순,문인걸,홍성란,정필호,황정규,김윤이,황지주,정호연 대한내분비학회 1999 Endocrinology and metabolism Vol.14 No.4
McCune-Albright syndrome (MAS) is a sporadic disease classically including polyostotic fibrous dysplasia, cafe -au-lait spots, sexual precocity, and other hyperfunctional endocrinopathies. Recent investigations suggest an etiological role for activating embryonic somatic missense mutations in the gene for the a subunit of Gs (Gsa), the G protein that stimulates adenylyl cyclase. DNA from bone, ovary, and blood was analyzed by using polymerase chain reaction and sequenced. A embryological somatic mutation of Gsa gene encoding substitution of a Cys for Arg at amino acid 201 from cells of dysplastic bone and ovary was observed, and the distribution of mutant gene reveals mosaic pattern. We report a case of McCune-Albright syndrome with an activating mutation at codon 201 of Gsa subunit on ovary and bone tissue that was experienced recently(J Kor Soc Endocrinol 14:779-785, 1999).
말초혈액 단핵구 세포에서 IL-1β에 의한 OPG/OCIF mRNA 발현 조절에 관한 연구
최규홍,정미선,오한진,김선우,윤현구,한인권,장학철,임창훈,정호연,한기옥,강영순,문인걸,황창선,김윤이,김의현 대한내분비학회 2000 Endocrinology and metabolism Vol.15 No.2
Background : Osteoprotegerin(OPG) is a soluble member of the tumor necrosis factor(TNF) receptor family and inhibits osteoclastogenesis by interrupting the cell-to-cell interaction between osteoblastic/stromal cells and osteoclast progenitors. OPG is expressed in many tissues including osteoblasts and may act on bone tissues in a paracrine and/or autocrine fashion. Futhermore, many cytokines and growth factors are known to influence the regulation of OPG expression in osteoblastic/stromal cells. The aims of the present study were to examine whether or not OPG was expressed in human peripheral blood mononuclear cells(PBMCs) and to investigate the effects of IL-1?, which were known as potent osteotropic agents, on the regulation of OPG mRNA in PBMCs. Methods : PBMCs were isolated by centrifugation over Ficoll-Hypaque density gradients from postmenopausal women and cultured in 6-well plates containing ?-MEM supplemented with 5% FBS. The expression of OPG mRNA in PBMCs was observed by RT-PCR in adherent and nadherent cells on culture plates. To observe the effect of OPG expression by IL-1?, we measured the concentration of OPG mRNA by altering the concentration and incubation time of IL-1?. The measurement of OPG mRNA was done by semi-quantitative PCR and indicated as OPG/GAPDH. Results : OPG was expressed both in cells attached to the surface of culture plates and in non-adherent cells for the incubation of peripheral blood mononuclear cells. The effect of OPG mRNA by IL-1? tend to increase in accordance with the length of incubation time and maximizes at 12 hours of incubation time and shows 1.2?3.5 times higher than the standard level at the concentration of 0.5ng/ml. However, the increased quantity in concentration varies according to individuals. Conclusion : OPG mRNA is expressed in peripheral blood mononuclear cells and known to be increased by IL-1?(J Kor Soc Endocrinol 15:204-213, 2000).