Moghal N E, Ferreira M A, Howie A J, Milford D V, Raafat E, Taylor C M
Department of Pathology, Children's Hospital, University of Birmingham, United Kingdom.
J Pediatr. 1998 Aug;133(2):220-3. doi: 10.1016/s0022-3476(98)70223-9.
Kidney biopsies were undertaken for persisting proteinuria 3.3 to 7 years (mean 5.4 years) from the onset of diarrhea-associated hemolytic uremic syndrome (D + HUS) in 5 boys and 2 girls (age at presentation mean 3.2 years, range 1.0 to 9.7 years). At 1 year the mean early morning urine protein/creatinine ratio was 100 mg/mmol, and the mean glomerular filtration rate was 65 mL/min/1.73 m2. At 5 years the mean early morning urine protein/creatinine ratio was 81 mg/mmol, and the mean glomerular filtration rate was 73 mL/min/1.73 m2. The biopsy specimens were compared with those of 7 age- and sex-matched children who were investigated for isolated persistent microscopic hematuria but in whom no abnormality was detected. Global glomerulosclerosis was noted in 6 patients with D + HUS, and 2 of these had segmental sclerosing lesions. Tubular atrophy and interstitial scarring were seen in all but 1 patients. The glomeruli in the D + HUS group were significantly larger than in the control group (P < .01). These findings are typically found in kidneys with reduced nephron numbers and are compatible with changes of hyperperfusion and hyperfiltration in surviving nephrons. Long-term follow-up of patients with D + HUS and proteinuria is advisable.
对5名男孩和2名女孩（就诊时年龄平均3.2岁，范围1.0至9.7岁）进行了肾活检，这些患儿自腹泻相关性溶血尿毒综合征（D + HUS）起病后持续蛋白尿3.3至7年（平均5.4年）。1岁时，清晨尿蛋白/肌酐比值平均为100 mg/mmol，肾小球滤过率平均为65 mL/min/1.73 m²。5岁时，清晨尿蛋白/肌酐比值平均为81 mg/mmol，肾小球滤过率平均为73 mL/min/1.73 m²。将活检标本与7名年龄和性别匹配的儿童的标本进行比较，这些儿童因孤立性持续性镜下血尿接受检查，但未发现异常。6例D + HUS患者出现全球肾小球硬化，其中2例有节段性硬化性病变。除1例患者外，其余患者均可见肾小管萎缩和间质瘢痕形成。D + HUS组的肾小球明显大于对照组（P <.01）。这些发现通常见于肾单位数量减少的肾脏，与存活肾单位的高灌注和高滤过变化相符。建议对D + HUS和蛋白尿患者进行长期随访。
