Kiprov D D, Colvin R B, McCluskey R T
Lab Invest. 1982 Mar;46(3):275-81.
Because reduction of renal mass (nephrectomy) can promote the development of focal glomerulosclerosis in animals, we asked whether patients with unilateral renal agenesis might have similar lesions in the solitary kidney. We describe here the clinical course and pathologic findings of eight patients who developed focal and segmental glomerulosclerosis (FGS) in their solitary kidneys. A review of 586 surgical pathology renal specimens (452 biopsies and 134 nephrectomies) revealed 29 (4.9 per cent) cases of FGS; five also had unilateral renal agenesis (p = 2.1 x 10(-7)). In 9200 autopsies, seven cases of unilateral renal agenesis were found; two (29 per cent) died of chronic renal failure with FGS lesions, and five did not have FGS. The eighth patient was identified because he was the father of a patient in this series. At the time of diagnosis the median age of the patients with unilateral renal agenesis and FGS was 25 years; seven of eight were male. All had proteinuria; four had more than 3 gm. per 25 hours (range, 1.2 to 9.0 gm. per 24 hours). Six developed chronic renal failure, and four died of their renal disease. Two of the patients were related (father and son). One patient had clinical and morphologic evidence of reflux nephropathy. The glomerular lesions were characterized by focal and segmental scarring and adhesions in glomeruli, IgM and C3 deposition by immunofluorescence, and foot process loss and capillary loop collapse by electron microscopy. Our series, although small, indicates that patients with unilateral renal agenesis are significantly more likely to develop FGS than patients with two kidneys. In contrast, FGS did not develop after adult nephrectomy in 10 patients who died 8 to 46 years after adult unilateral nephrectomy. The reason for this association was not established; however, these findings are in accord with experimental studies in which subtotal nephrectomy in young animals promotes FGS. In that setting and in these patients, glomerular damage may result from glomerular overload.
由于减少肾脏质量（肾切除术）可促进动物局灶性肾小球硬化的发展，我们探讨了单侧肾缺如患者的孤立肾是否会出现类似病变。在此，我们描述了8例孤立肾发生局灶节段性肾小球硬化（FGS）患者的临床病程及病理表现。回顾586份手术病理肾脏标本（452份活检及134份肾切除术标本）发现29例（4.9%）FGS病例；其中5例同时伴有单侧肾缺如（p = 2.1×10⁻⁷）。在9200例尸检中，发现7例单侧肾缺如；2例（29%）死于伴有FGS病变的慢性肾衰竭，5例未发生FGS。第8例患者因是本系列中1例患者的父亲而被发现。在诊断时，单侧肾缺如并患有FGS患者的中位年龄为25岁；8例中有7例为男性。所有患者均有蛋白尿；4例每25小时蛋白尿超过3g（范围为每24小时1.2至9.0g）。6例发展为慢性肾衰竭，4例死于肾脏疾病。2例患者有亲属关系（父子）。1例患者有反流性肾病的临床及形态学证据。肾小球病变的特征为肾小球局灶节段性瘢痕形成及粘连，免疫荧光显示IgM和C3沉积，电镜显示足突消失及毛细血管袢塌陷。我们的系列病例虽少，但表明单侧肾缺如患者发生FGS的可能性显著高于有双侧肾脏的患者。相比之下，10例成年单侧肾切除术后8至46年死亡的患者未发生FGS。这种关联的原因尚未明确；然而，这些发现与幼龄动物次全肾切除促进FGS的实验研究结果一致。在这种情况下以及这些患者中，肾小球损伤可能是由肾小球超负荷导致的。
