Caletti M G, Gallo G, Gianantonio C A
Nephrology Unit, Hospital Garrahan, University of Buenos Aires, Argentina.
Pediatr Nephrol. 1996 Dec;10(6):687-92. doi: 10.1007/s004670050189.
Renal biopsies from 19 boys and 11 girls, most with moderate or severe forms of hemolytic uremic syndrome (HUS) of the classic diarrhea-associated type, were analyzed as part of their long-term follow-up. Patients were biopsied because of late or persistent proteinuria, hypertension, and prolonged renal failure. The median length of follow-up was 11.2 years (range 0.9-22.0 years). Four histological groups were identified: focal segmental glomerulosclerosis and hyalinosis (FSGSH) (17 patients), diffuse mesangial proliferative glomerulonephritis (DMPGN) (9 patients), diffuse glomerulosclerosis (2 patients), and minimal glomerular changes (2 patients). The median interval between the onset of disease and renal biopsy was significantly shorter in DMPGN than in FSGSH (P < 0.001). The pathological findings may be the expression of two different stages of the same dynamic process: a regular sequence of glomerular lesions consisting of early DMPGN, followed by FSGSH. This lesion would ultimately lead to the final stage of global glomerulosclerosis. At the last examination, only a quarter of the patients had normal renal function. These observations also confirm that prolonged oligoanuria during the acute stage of HUS frequently results in an unfavorable long-term prognosis.
对19名男孩和11名女孩的肾活检组织进行了分析，这些患儿大多患有典型腹泻相关性溶血尿毒综合征（HUS）的中度或重度形式，这是他们长期随访的一部分。患者因晚期或持续性蛋白尿、高血压和长期肾衰竭而接受活检。随访的中位时长为11.2年（范围0.9 - 22.0年）。确定了四个组织学组：局灶节段性肾小球硬化和玻璃样变性（FSGSH）（17例患者）、弥漫性系膜增生性肾小球肾炎（DMPGN）（9例患者）、弥漫性肾小球硬化（2例患者）和轻微肾小球病变（2例患者）。DMPGN组疾病发作至肾活检的中位间隔时间显著短于FSGSH组（P < 0.001）。病理结果可能是同一动态过程两个不同阶段的表现：肾小球病变的规律序列，由早期DMPGN开始，随后是FSGSH。这种病变最终会导致全球肾小球硬化的最终阶段。在最后一次检查时，只有四分之一的患者肾功能正常。这些观察结果也证实，HUS急性期的长期少尿常导致不良的长期预后。
