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논문분류 춘계학술대회 초록집
제목 Immunoglobulin A nephropathy with superimposed podocytopathy
저자 Hee Rim Kang, Hye Jun Lee, Ju Hung Song, Seon-Ho Ahn, Jong Hwan Jung
출판정보 2019; 2019(1):
키워드 IgA nephropathy | Podocyte | Nephrotic sydnrome | Proteinuria | Hematuria
초록 "IgA nephropathy (IgAN) is the most common glomerulonephritis. IgAN represents with hematuria and mild proteinuria, but nephrotic proteinuria is not common in IgAN. The nephrotic proteinuria can be mainly due to two causes: disease progression of IgAN itself and IgAN with superimposed podocytopathy. However, clinical prognosis, presentation and responsiveness for steroid of IgAN with proteinuria of nephrotic range are significantly different. Herein, we report an interesting case of mild IgAN, presenting as nephrotic syndrome.A 62-year-old male visited the emergency department for generalized edema. His blood pressure was 120/70 mmHg, and laboratory data were followings: blood urea nitrogen, 21 mg/dL; serum creatinine, 0.85 mg/dL; serum albumin, 1.6 g/dL; C3, 135 mg/dL; C4, 33 mg/dL; urine albumin-creatinine ratio, 9.8 g/g; urine RBC, 5~10/HPF. His viral markers were negative. Chest X-ray showed pleural effusions. His symptoms was improved after use of furosemide, and we performed renal biopsy. The renal biopsy showed mildly mesangial hypercellularity with increased matrix. Tubules and interstitium revealed mild atrophy and fibrosis. On electron microscopic finding, effacement of foot process on podocytes was severely shown. On immunofluorescence microscopic finding, IgA, and kappa and lambda chains were stained on mesangium. We started high steroid therapy. His severe proteinuria and edema were completely improved after 2 months of the steroid therapy. His renal function is still preserved. If IgAN presenting as the nephrotic syndrome show following signs: sudden onset of edema, severe proteinuria, preserved renal function, and mild glomerulonephritis, steroid-responsiveness and renal prognosis of the IgAN can be usually good. Conclusively, when comparing with patients with other IgAN, patients with mild IgAN with superimposed podocytopathy may achieve a complete remission after steroid therapy. Although long-term effect of superimposed injury in podocytes in IgAN is still unclear, we carefully suggest that IgAN with superimposed podocytopathy might be a new disease."
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