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http://dx.doi.org/10.3339/jkspn.2012.16.1.21

Pathology and Classification of Focal Segmental Glomerulosclerosis  

Kim, Yong-Jin (Department of Pathology, Yeungnam University College of Medicine)
Publication Information
Childhood Kidney Diseases / v.16, no.1, 2012 , pp. 21-31 More about this Journal
Abstract
Focal segmental glomerulosclerosis (FSGS) is the name of the primary glomerular disease as well as the terminology to describe the secondary phenomena of any other glomerular diseases. It is characterized by sclerosis, hyalinosis, foam cell infiltration, vacuolar change of podocytes, and halo formation in the glomerulus. Throughout the interstitium, lymphocytes infiltration, tubular atrophy and vascular changes are accompanied. Occasionally, IgM and/or C3 depositions are noted in the sclerotic areas. Electron microscopically, diffuse effacement of foot processes are seen in non-sclerotic area like minimal change disease. Podocyte injury patterns including vacuolar changes are frequently examined. Recently, Columbia group has suggested morphologic classification of FSGS and they demonstrated very good prognosis of tip lesion and poor prognosis of both collapsing and cellular types. However, the pathogenetic classification has been suggested by others; hyperfilteration, podocyte injury, genetic lesions etc. Further studies are necessary to understand and treat this disease.
Keywords
Focal segmental glomerulosclerosis; Histopathology; Classification;
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1 Hyman LR, Burkholder PM. Focal sclerosing glomerulonephropathy with segmental hyalinosis. A clinicopathologic analysis. Lab Invest 1973;28:533-44.
2 Howie AJ, Brewer DB. The glomerular tip lesion: a previously undescribed type of segmental glomerular abnormality. J Pathol 1984;142:205-20.   DOI
3 Beaman M, Howie AJ, Hardwicke J, Michael J, Adu D. The glomerular tip lesion: a steroid responsive nephrotic syndrome. Clin Nephrol 1987;27:217-21.
4 Fogo A, Hawkins EP, Berry PL, Glick AD, Chiang ML, MacDonell RC Jr, et al. Glomerular hypertrophy in minimal change disease predicts subsequent progression to focal glomerular sclerosis. Kidney Int 1990;38:115-23   DOI   ScienceOn
5 Suzuki J, Yoshikawa N, Nakamura H. A quantitative analysis of the glomeruli in focal segmental glomerulosclerosis. Pediatr Nephrol 1994;8:416-9.   DOI   ScienceOn
6 Schwartz MM, Lewis EJ. Focal segmental glomerular sclerosis: the cellular lesion. Kidney Int 1985;28:968-74.   DOI   ScienceOn
7 Weiss MA, Daquioag E, Margolin EG, Pollak VE. Nephrotic syndrome, progressive irreversible renal failure, and glomerular "collapse": a new clinicopathologic entity? Am J Kidney Dis 1986;7:20-8.
8 Meyrier AY. Collapsing glomerulopathy: expanding interest in a shrinking tuft. Am J Kidney Dis 1999;33:801-3.   DOI   ScienceOn
9 Schwartz MM. Focal segmental glomerulosclerosis. In: Jennette JC, Olson JL, Schwartz MM, editors. Heptinstall's Pathology of the Kidney. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2007, p 171-2.
10 D'Agati VD, Fogo AB, Bruijn JA, Jennette JC. Pathologic classification of focal segmental glomerulosclerosis: a working proposal. Am J Kidney Dis 2004;43:368-82.   DOI   ScienceOn
11 Schwartz MM. Focal segmental glomerulosclerosis. In: Jennette JC, Olson JL, Schwartz MM, editors. Heptinstall's Pathology of the Kidney. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2007, p 156-8.
12 Rich AR. A hitherto undescribed vulnerability of the juxtamedullary glomeruli in lipoid nephrosis. Bull Johns Hopkins Hosp 1957;100:173-86.
13 Churg J, HabibR, White R. Pathology of the nephrotic syndrome in children: A report for the Internation Study of Kidney Disease in Children. Lancet 1970;1:1299-302.
14 White R, Glasgow EF, Mills RJ. Clinicopathologic study of nephrotoc syndrome in childhood. Lancet 1970;1:1353-9.
15 Newbold KM, Sandison A, Howie AJ. Comparison of size of juxtmedullary and outer cortical glomeruli in normal adult kidney. Virchows Arch A Pathol Anat Histopathol 1992;420:127-9.   DOI   ScienceOn
16 Skov K, Nyengaard JR, Partwardan A, Mulvany MJ. Large juxtmedullary glomeruli and afferent arterioles in health primates. Kidney Int 1999;55:1462-69.   DOI   ScienceOn
17 Kim MJ, Kim D, Lim BJ, Jung HJ. An analysis of focal segmental glomerulosclerosis according to morphologic subtypes. Korean J Pathol 2010;44:589-96.   DOI
18 Bonsib SM. Focal segmental glomerulosclerosis. The relationship between tubular atrophy and segmental sclerosis. Am J Clin Pathol 1999;111:343-8.
19 Thomas DB, Franceschini N, Hogan SL, Ten Holder S, Jennette CE, Falk RJ, et al. Clinical and pathologic characteristics of focal segmental glomerulo- sclerosis pathologic variants. Kidney Int 2006;69:920-6.   DOI   ScienceOn
20 Stokes MB, Valeri AM, Markowitz GS, D'Agati VD. Cellular focal segmental glomerulosclerosis: clinical and pathologic features. Kidney Int 2006;70:1783-92.   DOI   ScienceOn
21 Barisoni L, Schnaper HW, Kopp JB. A proposed taxonomy for the podocytopathies: A reassessment of the primary nephrotic diseases. Clin J Am Soc Nephrol 2007;2:529-42.   DOI   ScienceOn
22 Albaqumi M, Barisoni L. Current views on collapsing glomerulopathy. J Am Soc Nephrol 2008;19:1276-81.   DOI   ScienceOn
23 Thomas DB. Focal segmental glomerulosclerosis. A morphologic diagnosis in evolution. Arch Pathol Lab Med 2009;133:217-23.
24 Howie JA. Problems with 'focal segmental glomerulosclerosis'. Pediatr Nephrol 2011;26:1197-205.   DOI   ScienceOn
25 Taneda S, Honda K, Uchida K, Nitta K, Yumura W, Oda H, et al. Histological heterogeneity of glomerular segmental lesions in focal segmental glomerulosclerosis. Int Urol Nephrol 2012; 44:183-96.   DOI   ScienceOn