关键词: 纤维连接蛋白肾小球病, , 肾活检, 蛋白尿, 高血压

Abstract:

Objective: To analyze the clinical and histopathological features of patients with fibronectin glomerulopathy (GFND). Methodology: Ten patients with renal biopsy-proven GFND were retrospectively studied. The clinical and laboratory tests, and renal biopsy data (histopathological, immunofluorescence and electron microscopy) were collected. Results: They were 6 males and 4 females with an average age of 28.8±5.8 years old (ranged from 16 to 46). Only one patient had the family history of renal disease. The initial manifestations were proteinuria and nephritic syndrome in 80%, and micro-hematuria in 40% patients. None of them showed gross hematuria. Hypertension was found in 4 and elevated serum creatinine in 5 cases at onset of the disease. All patients had abnormal lipid metabolism, and most of patients had increased urine enzymes. The light microscopy examination revealed marked hypertrophy with numerous periodic acid-Schiff (PAS) and Masson-trichrom-positive deposits in the subendothelial space of all cases and in the mesangium of 7 cases. Six patients (60.0%) presented membrane proliferative glomerulonephritis (MPGN) with endothelial and mesangial proliferation, and with mesangiolysis in 5 patients. The immunofluorescence determination showed all positive both in immunoglobulins and complements. The fibronectin was positively stained in the mesangium and capillary loop. The electron microscopy revealed numerous heterogeneous deposits included lipids in the mesangium and subendothelial space. Those deposits were fibronectin proved by the immuno-electron microscopy. Conclusion: The diagnosis of GFND must be depend on renal biopsy.