Proteinuria and focal segmental glomerulosclerosis in severely obese adolescents

Proteinuria and focal segmental glomerulosclerosis in severely obese adolescents
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DOI:
10.1067/mpd.2001.113006
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发表时间:
2001-04-01
影响因子:
5.1
通讯作者:
Blowey, DL
Blowey, DL
中科院分区:
医学2区
文献类型:
--
作者:
Adelman, RD;Restaino, IG;Blowey, DL

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目的:描述肥胖相关蛋白尿和局灶节段性肾小球硬化的临床和实验室特征。研究设计:患者在两家大型儿童医院就诊超过12年。肾活检是为诊断不明原因的大量蛋白尿而进行的,并准备进行光镜、免疫荧光和电子显微镜检查,由两名儿科病理学家独立阅读。结果:7名非洲裔美国青少年确诊为肥胖相关蛋白尿,其特征为重度肥胖(120+/-30公斤),体重指数显著升高(46+/-11),轻度高血压(134/74+/-10/18毫米汞),血清白蛋白水平略低至正常(3.6+/-0.2g/dL),血清胆固醇水平中度升高(196+/-60 mg/dL),24小时蛋白排泄量增加(3.1+/-1.3g/dL)。计算的肌酐清除量正常的有6例,降低的有1例。典型的肾脏组织学特征包括肾小球肥大,局灶性节段性肾小球硬化,系膜基质和细胞增多,相对保存的足突形态,缺乏炎症或免疫介导的发病机制的证据。一名患者在体重减轻后蛋白尿显著减少。3例接受血管紧张素转换酶抑制剂治疗的患者尿蛋白丢失从2.9g/d降至0.7g/d,1例发展为终末期肾病。结论:肥胖青少年应监测蛋白尿,这种蛋白尿具有明显的临床和病理特征,可能与显著的肾脏损害有关。这种蛋白尿可能对体重减轻和/或血管紧张素转换酶抑制剂治疗有反应。
Objective: To describe the clinical and laboratory features of obesity associated proteinuria and focal segmental glomerulosclerosis.Study design: The patients were seen over a 12-year period at two large children's hospitals. Renal biopsies, performed for the diagnosis of unexplained heavy proteinuria and prepared for light, immunofluorescent, and electron microscopy, were read independently by two pediatric pathologists. Blood pressure, body mass index, serum levels of creatinine, albumin, and cholesterol, and 24-hour urinary protein were measured.Results: Seven African American adolescents were identified with obesity-associated proteinuria, which was characterized by severe obesity (120 +/- 30 kg), markedly elevated body mass index (46 +/- 11), mild hypertension (134/74 +/- 10/18 mm Hg), slightly low to normal serum albumin levels (3.6 +/- 0.2 g/dL), moderately elevated serum cholesterol levels (196 +/- 60 mg/dL), and elevated 24-hour protein excretion (3.1 +/- 1.3 g/dL). Calculated creatinine clearance was normal in 6 patients and decreased in one. Typical renal histologic features included glomerular hypertrophy, focal segmental glomerulosclerosis, increased mesangial matrix and cellularity, relative preservation of foot process morphology, and absence of evidence of inflammatory or immune-mediated pathogenesis. One patient showed a dramatic reduction in proteinuria in response to weight reduction. Three patients who were given angiotensin-converting enzyme inhibitors had reduced urinary protein losses from 2.9 g to 0.7 g per day One patient developed end-stage renal disease.Conclusion: Obese adolescents should be monitored for proteinuria, which has distinct clinical and pathologic features and may be associated with significant renal seguelae. Such proteinuria may respond to weight reduction and/or treatment with angiotensin-converting enzyme inhibitors.