Yamazaki Y, Mori A, Nomura Y, Satoh A, Park K I, Kawakatsu H, Nishida M
Department of Pediatrics, Shiga University of Medical Science, Ishibe Medical Center, Japan.
Nihon Jinzo Gakkai Shi. 1997 Oct;39(7):765-70.
We present a case of IgA nephropathy (IgAGN) which developed rapidly progressive glomerulonephritis and showed marked clinical improvement with treatment. The patient was a 7-year-old boy who initially presented with acute nephritic syndrome with hypocomplementemia. Although the renal function improved with normalization of the serum complement level, it deteriorated again progressively. The first renal biopsy revealed cellular crescents in about 70 percent of 43 glomeruli. Immunofluorescent microscopy demonstrated deposits of IgA, C3 and IgG in the mesangium; they were also deposited along the glomerular capillary walls. He was treated with plasma exchange associated with hemodialysis and methylprednisolone pulse therapy, followed by oral administration of prednisolone, cyclophosphamide and warfarin. Renal function recovered to the normal range about two months after the initiation of treatment. The second biopsy demonstrated a marked decrease in histological activity. In this case, transient hypocomplementemia at onset may indicate that acute glomerulonephritis caused exacerbation of clinically silent IgAGN. Aggressive therapy may be effective in patients with rapidly progressive IgAGN if treated at an early stage.
我们报告一例IgA肾病（IgAGN），该病例发展为快速进展性肾小球肾炎，经治疗后临床症状显著改善。患者为一名7岁男孩，最初表现为急性肾炎综合征伴低补体血症。尽管随着血清补体水平恢复正常，肾功能有所改善，但随后又逐渐恶化。首次肾活检显示，43个肾小球中约70%出现细胞性新月体。免疫荧光显微镜检查显示，系膜区有IgA、C3和IgG沉积；它们也沿肾小球毛细血管壁沉积。患者接受了血浆置换联合血液透析及甲泼尼龙冲击治疗，随后口服泼尼松龙、环磷酰胺和华法林。治疗开始约两个月后，肾功能恢复至正常范围。第二次活检显示组织学活性显著降低。在该病例中，起病时短暂的低补体血症可能表明急性肾小球肾炎导致了临床隐匿性IgAGN的加重。对于早期治疗的快速进展性IgAGN患者，积极治疗可能有效。
