Bhathena D B, Julian B A, McMorrow R G, Baehler R W
Am J Kidney Dis. 1985 May;5(5):226-32. doi: 10.1016/s0272-6386(85)80113-x.
Morphometric data on glomerular size are presented on three patients with solitary functioning kidneys and one with bilateral oligomeganephronic hypoplasia. Renal biopsy of each of two patients with a congenitally absent kidney (unilateral renal agenesis) and a patient with oligomeganephronie, all with proteinuria and renal insufficiency, reveal increases of mean glomerular diameters of at least 1.75X and mean glomerular volumes greater than 5X. These dimensions, which are in the range of maximal increases recorded for man, are associated in all three biopsies with focal sclerosis of the hypertrophied glomeruli. By contrast, the functionally fully-compensated solitary kidney of a patient who lost function of the contralateral kidney from acquired disease, is characterized by the absence of focal glomerulosclerosis and by glomerular enlargement of significantly lesser degree (increased mean diameter 1.24X and mean volume less than 2X). These observations correlate glomerular injury with glomerular size and suggest that in the setting of reduced nephron numbers, nephron destruction via focal glomerulosclerosis may be initiated when compensatory glomerular hypertrophy has reached its limits.
本文呈现了3例单肾有功能患者以及1例双侧肾单位过少性肾发育不全患者的肾小球大小形态学数据。对2例先天性肾缺如(单侧肾发育不全)患者以及1例肾单位过少患者进行肾活检,所有患者均有蛋白尿和肾功能不全,结果显示平均肾小球直径至少增加1.75倍,平均肾小球体积增加超过5倍。这些数值处于人类记录到的最大增加范围内,在所有3例活检中,均伴有肥大肾小球的局灶性硬化。相比之下,1例因后天疾病导致对侧肾失去功能、功能完全代偿的单肾患者,其特点是不存在局灶性肾小球硬化,肾小球增大程度明显较小(平均直径增加1.24倍,平均体积小于2倍)。这些观察结果将肾小球损伤与肾小球大小相关联,并表明在肾单位数量减少的情况下,当代偿性肾小球肥大达到极限时,可能会通过局灶性肾小球硬化引发肾单位破坏。