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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)
강미정 ( Kang Mi Jeong ),진규복 ( Jin Gyu Bog ),성정훈 ( Seong Jeong Hun ),황은아 ( Hwang Eun A ),한승엽 ( Han Seung Yeob ),박성배 ( Park Seong Bae ),김현철 ( Kim Hyeon Cheol ) 대한신장학회 2004 Kidney Research and Clinical Practice Vol.23 No.3
배경 : 막성 신염은 성인 특발성 신증후군의 가장 흔한 병리학적 병변으로 임상경과는 대부분 잠행성으로 알려져 있다. 막성 신염 환자에서 장기 예후가 양호할 것으로 보고된 인자로는 젊은 연령, 여성, 비신증후군 범위의 단백뇨, 진단 시의 정상 신기능, 신생검상 경한 사구체 변화, 신간질의 병변이 없을 때 등이다. 이 밖에 단백뇨의 완전 관해가 있을 경우 장기 예후가 양호하다는 보고가 있으나 관해 후의 임상 경과에 대해서는 아직 잘 알려져 있지 않다. 방법 : 1982년 1월부터 2003년 2월까지 계명의대 동산병원에서 경피적 신생검을 통해 특발성 막성 신염으로 진단된 환자 149예 중 완전 관해를 이룬 46예 (31%)를 대상으로 단백뇨의 관해 후 재발 여부와 이에 따른 장기예후를 후향적으로 조사했다. 결과 : 특발성 막성 신염의 149예 가운데 46예 (31%)에서 완전 관해를 보였으며 이중 자연 관해 24예 (52%), 면역억제제 및 스테로이드 병합요법 14예 (30%), 경구 스테로이드 단독요법이 8예 (17%)였다. 신생검시의 임상 양상은 신증후군 26예 (57%), 급성 신염 증후군 11예 (24%), 부증상적 요이상 7예 (15%), 재발성 육안적 혈뇨 2예(4%)였다. 완전 관해에 도달하는 시기는 평균 38개월 (7-170)이었고, 단백뇨의 완전 관해 후 34예 (74%)에서 지속적인 관해를 보였으며 12예 (26%)에서는 단백뇨가 재발하였으나 관해 후 평균 89개월의 추적기간 중 신부전으로 진행한 경우는 한 예도 없었다. 완전 관해 후 5년 및 10년 관해 유지율이 각각 0.73 및 0.60였다. 단백뇨의 완전 관해 후 관해 유지군과 재발군 사이에 성별, 나이, 치료 여부, 혈청 알부민, 혈청 콜레스테롤, 혈청 creatinine치 등은 유의한 차이가 없었다. 그러나 재발군에 비해 관해 유지군에서 관해 당시 혈청 콜레스테롤치 및 혈청 creatinine치가 유의하게 낮았다 (p<0.05). 결론 : 특발성 막성 신염 환자에서 단백뇨의 완전 관해가 있을 경우 장기 예후는 매우 양호할 것으로 생각된다. Background : The natural history of idiopathic membranous nephropathy is often benign, but approximately 30% of the patients develop renal insufficiency and progress to end-stage renal failure over 5 to 15 years. Several retrospective analyses have been performed to identify favorable factors influencing the long-term prognosis of idiopathic membranous nephropathy. However, little attention has been paid to the significance of remission of proteinuria in this disease. Methods : Forty- six patients with biopsy-proven idiopathic membranous nephropathy who achieved complete remission (CR) of proteinuria between 1982 and 2003 were enrolled. We retrospectively analyzed the clinical and laboratory data between persistent remission group and relapsing group after CR of proteinuria. Results : Complete remission of proteinuria was seen in 46 patients (31%) and was obtained 7-170 months (mean 38 months) after discovery of the disease. During mean 89 months follow-up, 74% of the patients remained in remission and 26% relapsed. However, proteinuria disappeared again spontaneously in some patients so that at last follow-up 83% of the patients are in CR. The probability of remaining free of proteinuria at 5 and 10years after CR was 0.73 and 0.60, respectively. 5 and 10years renal survival in patient with CR were 100%, respectively, whereas those of patient without remission were 89% and 75%, respectively, which was significantly different (p<0.05). We could not find any specific clinical factors favored maintenance of remission, except serum level of creatinine and serum cholesterol during remission, which are significantly less in patients with persistent remission. Conclusion : In conclusion, CR of proteinuria is a strong predictor of long-term favorable outcome in patients with idiopathic membranous nephropathy. (Korean J Nephrol 2004 ;23(3):412-418)
제1형 당뇨병이 동반된 그레이브스병 치료 중 발생한 일시적 과립구 감소증 1예
박진경,성정훈,이준,주선녀,정훈,김유리,박석원,조용욱,이상종 대한내분비학회 2002 Endocrinology and metabolism Vol.17 No.2
Granulocytopenia, which can be seen in patients with Graves' disease during treatment with antithyroid agents, could be a self resolving transient episode or can imply the beginning of life threatening agranulocytosis requiring a change in treatment modality. Transient granulocytopenia could be a manifestation of hyperthyroidism itself, or a mild side effect of antithyroid drugs. Aganulocytosis is a rare, but major complications of the termination drug, propylthiouracil (PTU), requiring prompt termination of the medication, and intensive care. Therefore, differentiation of agranulocytosis and transient granulocytopenia, is important, but is not practically easy. We introduce a case of transient granulocytopenia, which was detected in a patient with Graves' Disease, accompanied by underlying type 1 diabetes mellitus, during treatment with PTU. Diagnosis of transient granulocytopenia was made by a normal granulocyte count following a single injection of G-SCF*, and the patient was treated with conservative therapy. This case confirms a diagnostic tool for differentiating transient granulocytopenia and PTU-induced agranulocytosis