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大量蛋白尿和肾病综合征的概述

Authors
Ellie Kelepouris, MD, FAHA
Brad H Rovin, MD
Section Editor
Richard J Glassock, MD, MACP
Deputy Editor
John P Forman, MD, MSc
Translators
张晓良, 主任医师，副教授

引言和术语

肾小球疾病可导致3种不同的泌尿和临床类型：局灶性肾炎型、弥漫性肾炎型以及肾病型。 (参见“肾小球疾病的鉴别诊断与评估”)

  • 局灶性肾炎型–可导致局灶性肾炎性沉积的疾病通常在光学显微镜下观察到低于50%的肾小球发生炎性病变。尿液分析显示有红细胞(常为畸形红细胞)、红细胞管型(偶见)，以及轻度蛋白尿(通常<1.5g/d)。通常没有更晚期疾病的表现，如大量蛋白尿、水肿、高血压和肾功能不全。这些患者通常在常规体检时发现无症状血尿和蛋白尿，或偶尔出现肉眼血尿。
  • 弥漫性肾炎型–弥漫性肾小球肾炎的尿液分析结果与局灶性肾炎型的相似，但也可能观察到大量蛋白尿(可能在肾病范围内)、水肿、高血压和/或肾功能不全。弥漫性肾小球肾炎累及大部分或所有肾小球。
  • 肾病型–肾病性沉渣伴有大量蛋白尿和脂肪尿，但少有细胞或管型。“肾病综合征”这一术语是指肾脏疾病的独特的一系列临床特征和实验室特征。其被明确定义为出现大量蛋白尿(尿蛋白排泄>3.5g/24h)、低白蛋白血症(<3.0g/dL)和外周水肿。也常观察到高脂血症和血栓性疾病。

单纯性大量蛋白尿而不伴水肿或肾病综合征的其他特征提示有肾小球病(与肾病综合征的病因相同)，但这一表现并不一定伴有肾病综合征特有的多种临床特征和治疗问题。这是一项重要的临床区别，因为不伴水肿或低白蛋白血症的大量蛋白尿患者更可能是由继发性局灶节段性肾小球硬化(focal segmental glomerulosclerosis, FSGS)所致，例如由糖尿病所致[1]。

本专题将对大量蛋白尿和肾病综合征进行概述，重点在于具有肾病表现的疾病，即尿沉渣镜检有少量阳性发现(bland urine sediment)而不是尿沉渣镜检有大量阳性发现(active urine sediment)。与肾病综合征并发症相关的更具体的内容参见其他专题。 (参见“肾病综合征患者水肿的病理生理和治疗”和“肾病综合征的肾静脉血栓形成和高凝状态”和“肾病综合征的内分泌功能异常”和“肾病综合征患者的血脂异常”和“微小病变性肾病及其他类型肾病综合征中的急性肾损伤”)

引起肾病综合征的各疾病将在其他专题中详细讨论。将在文中适当的地方向读者提到这些单独的专题。

                            

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Literature review current through: 2017-06 . | This topic last updated: 2016-07-05.
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References
Top
  1. Praga M, Borstein B, Andres A, et al. Nephrotic proteinuria without hypoalbuminemia: clinical characteristics and response to angiotensin-converting enzyme inhibition. Am J Kidney Dis 1991; 17:330.
  2. Rivera F, López-Gómez JM, Pérez-García R, Spanish Registry of Glomerulonephritis. Clinicopathologic correlations of renal pathology in Spain. Kidney Int 2004; 66:898.
  3. Haas M, Meehan SM, Karrison TG, Spargo BH. Changing etiologies of unexplained adult nephrotic syndrome: a comparison of renal biopsy findings from 1976-1979 and 1995-1997. Am J Kidney Dis 1997; 30:621.
  4. Braden GL, Mulhern JG, O'Shea MH, et al. Changing incidence of glomerular diseases in adults. Am J Kidney Dis 2000; 35:878.
  5. Simon P, Ramee MP, Boulahrouz R, et al. Epidemiologic data of primary glomerular diseases in western France. Kidney Int 2004; 66:905.
  6. Malafronte P, Mastroianni-Kirsztajn G, Betônico GN, et al. Paulista Registry of glomerulonephritis: 5-year data report. Nephrol Dial Transplant 2006; 21:3098.
  7. Bahiense-Oliveira M, Saldanha LB, Mota EL, et al. Primary glomerular diseases in Brazil (1979-1999): is the frequency of focal and segmental glomerulosclerosis increasing? Clin Nephrol 2004; 61:90.
  8. Gesualdo L, Di Palma AM, Morrone LF, et al. The Italian experience of the national registry of renal biopsies. Kidney Int 2004; 66:890.
  9. Heaf J. The Danish Renal Biopsy Register. Kidney Int 2004; 66:895.
  10. Swaminathan S, Leung N, Lager DJ, et al. Changing incidence of glomerular disease in Olmsted County, Minnesota: a 30-year renal biopsy study. Clin J Am Soc Nephrol 2006; 1:483.
  11. D'Agati V. The many masks of focal segmental glomerulosclerosis. Kidney Int 1994; 46:1223.
  12. Hausmann R, Kuppe C, Egger H, et al. Electrical forces determine glomerular permeability. J Am Soc Nephrol 2010; 21:2053.
  13. Reiser J, von Gersdorff G, Loos M, et al. Induction of B7-1 in podocytes is associated with nephrotic syndrome. J Clin Invest 2004; 113:1390.
  14. Schönenberger E, Ehrich JH, Haller H, Schiffer M. The podocyte as a direct target of immunosuppressive agents. Nephrol Dial Transplant 2011; 26:18.
  15. Gbadegesin R, Lavin P, Foreman J, Winn M. Pathogenesis and therapy of focal segmental glomerulosclerosis: an update. Pediatr Nephrol 2011; 26:1001.
  16. Kaysen GA, Gambertoglio J, Jimenez I, et al. Effect of dietary protein intake on albumin homeostasis in nephrotic patients. Kidney Int 1986; 29:572.
  17. Kaysen GA, Kirkpatrick WG, Couser WG. Albumin homeostasis in the nephrotic rat: nutritional considerations. Am J Physiol 1984; 247:F192.
  18. Sun X, Martin V, Weiss RH, Kaysen GA. Selective transcriptional augmentation of hepatic gene expression in the rat with Heymann nephritis. Am J Physiol 1993; 264:F441.
  19. Pietrangelo A, Panduro A, Chowdhury JR, Shafritz DA. Albumin gene expression is down-regulated by albumin or macromolecule infusion in the rat. J Clin Invest 1992; 89:1755.
  20. Sun X, Kaysen GA. Albumin and transferrin synthesis are increased in H4 cells by serum from analbuminemic or nephrotic rats. Kidney Int 1994; 45:1381.
  21. Moshage HJ, Janssen JA, Franssen JH, et al. Study of the molecular mechanism of decreased liver synthesis of albumin in inflammation. J Clin Invest 1987; 79:1635.
  22. Zacchia M, Trepiccione F, Morelli F, et al. Nephrotic syndrome: new concepts in the pathophysiology of sodium retention. J Nephrol 2008; 21:836.
  23. Crew RJ, Radhakrishnan J, Appel G. Complications of the nephrotic syndrome and their treatment. Clin Nephrol 2004; 62:245.
  24. Chen T, Lv Y, Lin F, Zhu J. Acute kidney injury in adult idiopathic nephrotic syndrome. Ren Fail 2011; 33:144.
  25. Vaziri ND, Kaupke CJ, Barton CH, Gonzales E. Plasma concentration and urinary excretion of erythropoietin in adult nephrotic syndrome. Am J Med 1992; 92:35.
  26. Vaziri ND. Endocrinological consequences of the nephrotic syndrome. Am J Nephrol 1993; 13:360.
  27. Mähr N, Neyer U, Prischl F, et al. Proteinuria and hemoglobin levels in patients with primary glomerular disease. Am J Kidney Dis 2005; 46:424.
  28. Ginsberg JM, Chang BS, Matarese RA, Garella S. Use of single voided urine samples to estimate quantitative proteinuria. N Engl J Med 1983; 309:1543.
  29. Howard AD, Moore J Jr, Gouge SF, et al. Routine serologic tests in the differential diagnosis of the adult nephrotic syndrome. Am J Kidney Dis 1990; 15:24.