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루푸스 신염에서 ICAM-1과 VCAM-1의 조직내 발현
박성배 ( Park Seong Bae ),한상미 ( Han Sang Mi ),박관규 ( Park Gwan Gyu ),김현철 ( Kim Hyeon Cheol ) 대한신장학회 2001 Kidney Research and Clinical Practice Vol.20 No.6
루프스 신염에서 ICAM-1과 VCAM-1의 발현 양상을 조사하고, 이러한 접착분자들의 역할을 알아보기 위하여 1997년 8월부터 1999년 2월까지 계명대학교 동산의료원에서 초점성 분절형및 미만성 루프스 신염으로 진단된 39예의 신조직을 대상으로 ICAM-1과 VCAM-1에 대한 면역조직화학 염색을 시행하여 다음과 같은 결과를 얻었다. ICAM-1과 VCAM-1은 근위세뇨관과원위세뇨관에서 대조군에 비해 모두 발현이 증가되었다. ICAM-1은 신사구체 내피세포와 족세포, 벽측 상피세포 및 뇨강에서 발현이 크게 증가되었다. VCAM-1은 내피세포, 벽측 상피세포와 뇨강에서 강하게 발현되었으나, 족세포에서는 발현되지 않았다. 또한 모든 반월체에서 ICAM-1과 VCAM-1이 강하게 발현되었다. 그리고 이러한 신사구체 세포의 ICAM-1과 VCAM-1 발현의 증가는 신사구체 손상과 상관관계가 있었다. 이러한 결과들은 신사구체내 ICAM-1과 VCAM-1의 발현이 신사구체내 염증세포의 침윤을 매개함으로써 신사구체 손상과 반월체 형성에 중요한 역할을 할 것으로 생각된다. Intercellular adhsion molecule-1(ICAM-1) and vascular cell adhension molecule-1(VCAM-1) are important cell-surface glycoprotein regulating interactions among immune cells, such as accessory and T cells. Recently, the cDNA encoding the ICAM-1 and VCAM-1 molecule has been isolated and monoclonal antibodies for ICAM-1 and VCAM-1 have been identified, thus allowing studies of ICAM-1 and VCAM- 1 expression using frozen or paraffin-embedded tissues. To determine whether altered expression of ICAM-1 and VCAM-1 occurs in normal and autoimmune lupus nephritis, we studied the ICAM-1 and VCAM-1 expression in kidneys of five normal human kidney specimens and 39 paraffin-embedded tissues from patients with lupus nephritis. By immunohistochemical staining of our materials, ICAM-1 was expressed in the glomerular cells in all cases with lupus nephritis. It was only expressed in the interstitial cell in one case with normal kidney. VCAM-1 expression was significantly increased in the distal tubules and in the interstitial cells, glomerular endothelial, parietal epithlial cells and urinary spaces with lupus nephritis. It was not expressed in the normal kidney. ICAM-1 and VCAM-1 were strongly expressed in the crescents. These results suggest that ICAM-1 and VCAM-1 play an important role in the pathogenesis of glomerular damage and crescent formation in lupus nephritis.
항호중구 세포질 항체(ANCA) 양성으로 진단된 Wegener 육아종증 3예
허정숙 ( Heo Jeong Sug ),이수형 ( Lee Su Hyeong ),박성배 ( Park Seong Bae ),김현철 ( Kim Hyeon Cheol ),박관규 ( Park Gwan Gyu ),이상숙 ( Lee Sang Sug ) 대한내과학회 1993 대한내과학회지 Vol.44 No.2
WG is a syndrome characterized by necrotizing granulomatous lesions in the upper and lower respiratory tracts, glomerulonephritis, and a generalized vasculitis involving both arteries and veins. Recently ANCA has been reported to be a highly specific test for the diagnosis of WG. We have experienced three patients with ANCA positive WG whose initial clinical presentation mimicked Goodpasture`s syndrome. All three patients were female with mean age of 30 years(16-53). The major presenting signs and symptoms were hemoptysis, fever, general weakness, gastrointestinal symptoms, and conjunctivitis. Persistent rhinorrhea was seen in only one patient. All patients had lung, renal, and eye involvement, but none of the patients had signs of ear, heart or nervous system involvement. All three patients had moderate anemia,elevated ESR and urinary abnormality. One patient had leukopenia, and no patient had thrombocytopenia. All three patients were positive for rheumatoid factor and ANCA. None were positive for antinulear antibody, anti-GBM antibody, or cryoglobulin. Serum complement levels were normal in all three cases. Radiographic studies of chest showed multiple bilateral patches and nodular infilterates in all three patients. Cavitation was seen in one case and pleural effusion in one case. Two patients were treated with steroid pulse therapy, one of them showed dramatic improvement, the other patient expired due to progression of underlying illness. One patient whose renal function was normal at the time of admission recovered, but the other two patients who required hemodialysis treatment expired. In conclusion, earlier diagnosis of WG facilitated by ANCA tests may help reduce the higher morbidity and mortality seen in this type of patient.
성정훈 ( Seong Jeong Hun ),강미정 ( Kang Mi Jeong ),황은아 ( Hwang Eun A ),한승엽 ( Han Seung Yeob ),박성배 ( Park Seong Bae ),김현철 ( Kim Hyeon Cheol ),박관규 ( Park Gwan Gyu ) 대한신장학회 2004 Kidney Research and Clinical Practice Vol.23 No.2
The term type Ⅱ membranoproliferarive glomerulonephritis (MPGN) refers to the histopathologic entity characterized by dense intramembranous deposits. It have a variable clincal courses, frequently occurs in older children and young adult. In comparison with The western, the idiopathic membranoproliferative glomerulonephritis (MPGN) has a lower frequency than secondary MPGN. Especially, of the idiopathic MPGN, the frequency of type 2 MPGN, so called dense deposit disease, is very rare in Korea. We are reporting two cases of type Ⅱ MPGN, which was proven by renal biopsy. The clinical manifestations were recurrent gross hematuria in one patient and persistent nephrotic-ranged proteinuria in the other patient. The biopsy findings are characterized by diffuse wall thickening of capillary walls and focal proliferation of mesangial cell in light microscopy, and by capillary wall and granular basement membrane staining of C3 in immunofluorescence microscopy, and an irregular fusiform swelling of the lamina densa which resulting in a further thickening of basement of basement membrane in electron microscopy. Our two patients were treated conservatively without using steroid or immunosuppressive agents. One patient who had followed-up for 7 years after diagnosis remain stable in renal function, and the other patient who had followed-up for 4 years after diagnosis showed persistent nephrotic-range proteinuria. (Korean J Nephrol 2004;23(2):335-340)
신 이식 후의 재발성 분절성 사구체 경화증 : 임상 및 혈장반출의 치료 효과 Natural Course and Treatment with Plasmapheresis
김현철,박성배,황은아,이기태,박경대,박관규 대한신장학회 2000 Kidney Research and Clinical Practice Vol.19 No.5
The recurrence of focal segmental glomerulosclerosis(FSGS) after renal transplantation has a potentially deteriorating course toward the loss of graft function. To identify risk factors for recurrence and efficacy of plasmapheresis, we evaluated outcome of 20 renal allografts in 18 patients with FSGS who underwent transplantation from March 1992 to September 1999. Recurrence was observed in seven of 18(39%) patients. Patients who had rapid progression to end stage renal disease, young age at the time of onset of the disease and the presence of mesangial proliferation tended to more frequent recurrence, albeit statistically not significant. Five patients underwent plasmapheresis. Proteinuria decreased from 5.3±2.1g to 0.8±0.7g immediately after completion of plasmapheresis. Four patients with an improvement in proteinuria had stable renal function at last follow-up. One patient who had chronic rejection lost graft function at 22 months after renal transplantation. In one in whom plasmapheresis was initiated mediately without allograft biopsy had long-lasting complete remission. Two patients who not receive plasma-Apheresis, lost their graft funtion at 21 and 97 months after renal transplantation. We concluded that plasmapheresis in likely to be effective in the therapy of recurrent FSGS if the diagnosis is made promptly following the appearance of proteinuria, there is no significant hyalinosis on pre-plasmapheresis biopy and plasrnapheresis is initiated imrnediately.