Nishimoto K, Shiiki H, Nishino T, Uyama H, Iwano M, Dohi K
First Department of Internal Medicine, Nara Medical University, Japan.
J Am Soc Nephrol. 1997 Nov;8(11):1668-78. doi: 10.1681/ASN.V8111668.
The present study was performed to assess the pathogenetic role of glomerular hypertrophy in patients with primary focal segmental glomerulosclerosis (FSGS). We studied 14 patients with FSGS by morphometry. In seven patients, minimal change nephrotic syndrome (MCNS) was diagnosed on the first renal biopsy, but FSGS was diagnosed on the second biopsy (MCNS-FSGS group). Seven other patients with FSGS on the first biopsy underwent second biopsies while in remission (FSGS-R group). Biopsy results were compared with biopsies from 10 patients with MCNS and seven control subjects. Nonsclerotic glomeruli were examined. The mean glomerular tuft area, whole glomerular area, and number of mesangial cells were significantly increased in both biopsies from the MCNS-FSGS group and in the first biopsies obtained during the nephrotic stage of the FSGS-R group, compared with control subjects and patients with MCNS. Biopsies from FSGS patients in remission showed that the mean glomerular tuft area and number of mesangial cells were significantly decreased. The fractional extracellular matrix area (extracellular matrix area/glomerular tuft area) and mesangial cell density (mesangial cell number/glomerular tuft area) in FSGS during both nephrotic and remission stages were the same as those in control subjects and patients with MCNS. The present study suggests that glomerular hypertrophy precedes the development of glomerulosclerosis in FSGS and is reversible when patients are in remission. These features support the pathogenetic importance of glomerular hypertrophy in patients with primary FSGS.
本研究旨在评估肾小球肥大在原发性局灶节段性肾小球硬化（FSGS）患者发病机制中的作用。我们通过形态学测量研究了14例FSGS患者。7例患者在首次肾活检时被诊断为微小病变肾病（MCNS），但在第二次活检时被诊断为FSGS（MCNS-FSGS组）。另外7例首次活检诊断为FSGS的患者在病情缓解时接受了第二次活检（FSGS-R组）。将活检结果与10例MCNS患者和7例对照受试者的活检结果进行比较。检查非硬化性肾小球。与对照受试者和MCNS患者相比，MCNS-FSGS组的两次活检以及FSGS-R组肾病阶段首次活检的肾小球毛细血管丛平均面积、整个肾小球面积和系膜细胞数量均显著增加。FSGS缓解期患者的活检显示，肾小球毛细血管丛平均面积和系膜细胞数量显著减少。FSGS患者在肾病期和缓解期的细胞外基质分数面积（细胞外基质面积/肾小球毛细血管丛面积）和系膜细胞密度（系膜细胞数量/肾小球毛细血管丛面积）与对照受试者和MCNS患者相同。本研究表明，肾小球肥大先于FSGS中肾小球硬化的发生，且在患者缓解时是可逆的。这些特征支持了肾小球肥大在原发性FSGS患者发病机制中的重要性。
