Focal segmental glomerulosclerosis pathophysiology: Difference between revisions

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__NOTOC__
__NOTOC__
{{Focal segmental glomerulosclerosis}}
{{Focal segmental glomerulosclerosis}}
* {{CMG}};{{APM}}; '''Associate Editor-In-Chief:’’’ {{CZ}}; {{OO}}'''
{{CMG}};{{APM}}; {{AE}} {{MKA}}, {{MKK}}, {{CZ}}, {{OO}}


==Overview==
==Overview==
The [[pathophysiology]] of focal segmental glomerulosclerosis (FSGS) is based on two types of FSGS. Primary FSGS is also known as [[idiopathic]] FSGS, there is a [[hypothesis]] that suggests it occurs as a result of circulating [[immune]] activating factors interacting with the [[glomerular]] [[epithelium]]. The underlying [[pathogenesis]] of FSGS is fusion or [[effacement]] of the foot processes ([[podocytes]]) of the [[glomeruli]] and sclerosing of some parts of the [[glomeruli]]. These changes result in [[apoptosis]] and detachment of the [[Glomerular basement membrane|glomerular basement membrane (GBM)]] resulting in subsequent loss of negative charge on [[podocytes]] and podocytopenia. Secondary FSGS is based on [[glomerular]] [[hypertrophy]] and hyperfiltration and over expression of [[inflammatory]] mediators such as, [[TGF-beta]], [[PDGF]] and [[VEGF]]. The underlying [[pathogenesis]] can be based on multiple [[genetic]] [[mutations]] in NPHS1, NEPH1, [[NPHS2]], [[WT1]] and [[INF2]] [[genes]]. Conditions associated with [[FSGS]] include, [[diabetes]], [[HIV]], [[sickle cell disease]], [[nephrotic syndrome]] and [[minimal change disease]]. On [[microscopic]] [[histopathological]] analysis progressive changes seen are, foot process [[effacement]], [[podocyte]] [[apoptosis]], exposed [[GBM]], [[capillary]] expansion and mesangial [[matrix]] [[proliferation]]. 


==Pathophysiology==
==Pathophysiology==
There are two types of FSGS, primary FSGS and secondary FSGS,  pathophysiology is discussed below:
There are two types of FSGS, primary FSGS and secondary FSGS,  [[pathophysiology]] is discussed below:


=== Pathogenesis of primary FSGS ===
=== Pathogenesis of primary FSGS ===
The pathogenesis of primary or Idiopathic FSGS is not so clear. Many studies had theorized that FSGS occurs as a consequence of effects of circulating immune activating factors on the glomerular epithelium. Indeed, the damaging role of circulating factors like the soluble urokinase plasminogen activating receptor (suPAR) on the glomerular podocytes had been postulated. The underlying pathogenesis of FSGS is fusion or effacement of the foot processes ([[podocytes]]) of the [[glomeruli]], with sclerosing of some part of the [[glomeruli]] (hence its name as focal segmental).   As such, the involvement of the permselective filtration barrier and effacement of podocyte foot processes are inevitable. The four major causes that lead to the reaction of podocyte foot processes. These changes result in [[apoptosis]], detachment from the glomerular basement membrane (GBM), and subsequent podocytopenia:<ref name="pmidPMID 25168830">{{cite journal| author=Reiser J, Nast CC, Alachkar N| title=Permeability factors in focal and segmental glomerulosclerosis. | journal=Adv Chronic Kidney Dis | year= 2014 | volume= 21 | issue= 5 | pages= 417-21 | pmid=PMID 25168830 | doi=10.1053/j.ackd.2014.05.010 | pmc=4149759 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25168830 }} </ref><ref name="pmid14712353">{{cite journal| author=Asanuma K, Mundel P| title=The role of podocytes in glomerular pathobiology. | journal=Clin Exp Nephrol | year= 2003 | volume= 7 | issue= 4 | pages= 255-9 | pmid=14712353 | doi=10.1007/s10157-003-0259-6 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14712353  }} </ref><ref name="pmid12704576">{{cite journal| author=Fogo AB| title=Animal models of FSGS: lessons for pathogenesis and treatment. | journal=Semin Nephrol | year= 2003 | volume= 23 | issue= 2 | pages= 161-71 | pmid=12704576 | doi=10.1053/snep.2003.50015 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12704576 }} </ref><ref name="pmid23138488">{{cite journal| author=Wei C, Trachtman H, Li J, Dong C, Friedman AL, Gassman JJ et al.| title=Circulating suPAR in two cohorts of primary FSGS. | journal=J Am Soc Nephrol | year= 2012 | volume= 23 | issue= 12 | pages= 2051-9 | pmid=23138488 | doi=10.1681/ASN.2012030302 | pmc=3507361 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23138488  }} </ref>
*The [[pathogenesis]] of primary or [[Idiopathic]] FSGS is not so clear.<ref name="pmidPMID 25168830">{{cite journal| author=Reiser J, Nast CC, Alachkar N| title=Permeability factors in focal and segmental glomerulosclerosis. | journal=Adv Chronic Kidney Dis | year= 2014 | volume= 21 | issue= 5 | pages= 417-21 | pmid=PMID 25168830 | doi=10.1053/j.ackd.2014.05.010 | pmc=4149759 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25168830  }} </ref>
* Interference  with slit diaphragm and its corresponding [[lipid raft]]
*Many studies had theorized that FSGS occurs as a consequence of effects of circulating [[immune]] activating factors on the [[glomerular]] [[epithelium]].<ref name="pmidPMID 25168830">{{cite journal| author=Reiser J, Nast CC, Alachkar N| title=Permeability factors in focal and segmental glomerulosclerosis. | journal=Adv Chronic Kidney Dis | year= 2014 | volume= 21 | issue= 5 | pages= 417-21 | pmid=PMID 25168830 | doi=10.1053/j.ackd.2014.05.010 | pmc=4149759 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25168830  }} </ref>
* Interference with [[actin]] cytoskeleton
*The damaging role of circulating factors like the [[soluble]] [[Urokinase receptor|urokinase]] [[plasminogen]] activating [[receptor]] (suPAR) on the [[glomerular]] [[podocytes]] had been suggested.<ref name="pmidPMID 25168830">{{cite journal| author=Reiser J, Nast CC, Alachkar N| title=Permeability factors in focal and segmental glomerulosclerosis. | journal=Adv Chronic Kidney Dis | year= 2014 | volume= 21 | issue= 5 | pages= 417-21 | pmid=PMID 25168830 | doi=10.1053/j.ackd.2014.05.010 | pmc=4149759 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25168830 }} </ref>  
*Interference with the [[glomerular basement membrane|GBM]] or with the interaction of the [[glomerular basement membrane|GBM]] and the [[podocytes]]
*The underlying [[pathogenesis]] of FSGS is fusion or [[effacement]] of the foot processes ([[podocytes]]) of the [[glomeruli]], with sclerosing of some parts of the [[glomeruli]] (hence the name focal segmental). 
*Interference with the negative charge of [[podocytes]]
*The involvement of the permselective [[filtration]] barrier and [[effacement]] of [[podocyte]] foot processes are inevitable.  
There are various factors, which play important in the pathogenesis of FSGS:
*These changes result in:<ref name="pmid14712353">{{cite journal| author=Asanuma K, Mundel P| title=The role of podocytes in glomerular pathobiology. | journal=Clin Exp Nephrol | year= 2003 | volume= 7 | issue= 4 | pages= 255-9 | pmid=14712353 | doi=10.1007/s10157-003-0259-6 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14712353  }} </ref><ref name="pmid12704576">{{cite journal| author=Fogo AB| title=Animal models of FSGS: lessons for pathogenesis and treatment. | journal=Semin Nephrol | year= 2003 | volume= 23 | issue= 2 | pages= 161-71 | pmid=12704576 | doi=10.1053/snep.2003.50015 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12704576  }} </ref><ref name="pmid23138488">{{cite journal| author=Wei C, Trachtman H, Li J, Dong C, Friedman AL, Gassman JJ et al.| title=Circulating suPAR in two cohorts of primary FSGS. | journal=J Am Soc Nephrol | year= 2012 | volume= 23 | issue= 12 | pages= 2051-9 | pmid=23138488 | doi=10.1681/ASN.2012030302 | pmc=3507361 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23138488  }} </ref>
 
**[[Apoptosis]]
===='''Role of circulating permeability Factor'''====
**Detachment from the [[glomerular basement membrane]] ([[GBM]])
Circulating factors implicated in the [[pathogenesis]] of Primary FSGS include:
**Subsequent podocytopenia
* Soluble Urokinase Plasminogen Activating Receptor (suPAR) and [[MicroRNAs]].<ref name="pmidPMID 25168830">{{cite journal| author=Reiser J, Nast CC, Alachkar N| title=Permeability factors in focal and segmental glomerulosclerosis. | journal=Adv Chronic Kidney Dis | year= 2014 | volume= 21 | issue= 5 | pages= 417-21 | pmid=PMID 25168830 | doi=10.1053/j.ackd.2014.05.010 | pmc=4149759 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25168830  }} </ref>
**Interference  with slit diaphragm and its corresponding [[lipid raft]]
 
**Interference with [[actin]] [[cytoskeleton]]
* suPAR is a heavily glycosylated protein that can be found in several places.<ref name="pmid23138488">{{cite journal| author=Wei C, Trachtman H, Li J, Dong C, Friedman AL, Gassman JJ et al.| title=Circulating suPAR in two cohorts of primary FSGS. | journal=J Am Soc Nephrol | year= 2012 | volume= 23 | issue= 12 | pages= 2051-9 | pmid=23138488 | doi=10.1681/ASN.2012030302 | pmc=3507361 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23138488  }} </ref><ref name="pmid11158426">{{cite journal| author=Rea R, Smith C, Sandhu K, Kwan J, Tomson C| title=Successful transplant of a kidney with focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 2001 | volume= 16 | issue= 2 | pages= 416-7 | pmid=11158426 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11158426  }} </ref><ref name="pmid11328888">{{cite journal| author=Ghiggeri GM, Artero M, Carraro M, Perfumo F| title=Permeability plasma factors in nephrotic syndrome: more than one factor, more than one inhibitor. | journal=Nephrol Dial Transplant | year= 2001 | volume= 16 | issue= 5 | pages= 882-5 | pmid=11328888 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11328888  }} </ref><ref name="pmid11195803">{{cite journal| author=Kemper MJ, Wolf G, Müller-Wiefel DE| title=Transmission of glomerular permeability factor from a mother to her child. | journal=N Engl J Med | year= 2001 | volume= 344 | issue= 5 | pages= 386-7 | pmid=11195803 | doi=10.1056/NEJM200102013440517 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11195803  }} </ref>
**Interference with the [[glomerular basement membrane|GBM]] or with the interaction of the [[glomerular basement membrane|GBM]] and the [[podocytes]]
* Cardiotrophin-like cytokine factor-1 (CLCF1)
**Interference with the negative charge of [[podocytes]]
*Circulating factors implicated in the [[pathogenesis]] of primary FSGS include:
**[[Soluble]] [[Urokinase receptor|urokinase plasminogen activating receptor]] (suPAR) and [[MicroRNAs]].<ref name="pmidPMID 25168830">{{cite journal| author=Reiser J, Nast CC, Alachkar N| title=Permeability factors in focal and segmental glomerulosclerosis. | journal=Adv Chronic Kidney Dis | year= 2014 | volume= 21 | issue= 5 | pages= 417-21 | pmid=PMID 25168830 | doi=10.1053/j.ackd.2014.05.010 | pmc=4149759 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25168830 }} </ref>
**suPAR is a heavily [[glycosylated]] [[protein]] that can be found in several places.<ref name="pmid23138488">{{cite journal| author=Wei C, Trachtman H, Li J, Dong C, Friedman AL, Gassman JJ et al.| title=Circulating suPAR in two cohorts of primary FSGS. | journal=J Am Soc Nephrol | year= 2012 | volume= 23 | issue= 12 | pages= 2051-9 | pmid=23138488 | doi=10.1681/ASN.2012030302 | pmc=3507361 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23138488 }} </ref><ref name="pmid11158426">{{cite journal| author=Rea R, Smith C, Sandhu K, Kwan J, Tomson C| title=Successful transplant of a kidney with focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 2001 | volume= 16 | issue= 2 | pages= 416-7 | pmid=11158426 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11158426 }} </ref><ref name="pmid11328888">{{cite journal| author=Ghiggeri GM, Artero M, Carraro M, Perfumo F| title=Permeability plasma factors in nephrotic syndrome: more than one factor, more than one inhibitor. | journal=Nephrol Dial Transplant | year= 2001 | volume= 16 | issue= 5 | pages= 882-5 | pmid=11328888 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11328888 }} </ref><ref name="pmid11195803">{{cite journal| author=Kemper MJ, Wolf G, Müller-Wiefel DE| title=Transmission of glomerular permeability factor from a mother to her child. | journal=N Engl J Med | year= 2001 | volume= 344 | issue= 5 | pages= 386-7 | pmid=11195803 | doi=10.1056/NEJM200102013440517 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11195803 }} </ref>
**[[Cardiotrophin-1|Cardiotrophin]]-like [[cytokine]] factor-1 ([[CLCF1]])


=== '''Pathogenesis of secondary FSGS''' ===
=== '''Pathogenesis of secondary FSGS''' ===
The pathogenesis of secondary focal segmental glomerulosclerosis (FSGS) occurs due to the following factors :
The [[pathogenesis]] of secondary focal segmental glomerulosclerosis (FSGS) occurs due to the following factors:
* Glomerular hypertrophy and hyperfiltration, which is due to the following :<ref name="pmid16409155">{{cite journal| author=Harris RC, Neilson EG| title=Toward a unified theory of renal progression. | journal=Annu Rev Med | year= 2006 | volume= 57 | issue=  | pages= 365-80 | pmid=16409155 | doi=10.1146/annurev.med.57.121304.131342 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16409155  }} </ref><ref name="pmid11423572">{{cite journal| author=Kang DH, Joly AH, Oh SW, Hugo C, Kerjaschki D, Gordon KL et al.| title=Impaired angiogenesis in the remnant kidney model: I. Potential role of vascular endothelial growth factor and thrombospondin-1. | journal=J Am Soc Nephrol | year= 2001 | volume= 12 | issue= 7 | pages= 1434-47 | pmid=11423572 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11423572  }} </ref>
*[[Glomerular]] [[hypertrophy]] and hyperfiltration, which is due to the following:<ref name="pmid16409155">{{cite journal| author=Harris RC, Neilson EG| title=Toward a unified theory of renal progression. | journal=Annu Rev Med | year= 2006 | volume= 57 | issue=  | pages= 365-80 | pmid=16409155 | doi=10.1146/annurev.med.57.121304.131342 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16409155  }} </ref><ref name="pmid11423572">{{cite journal| author=Kang DH, Joly AH, Oh SW, Hugo C, Kerjaschki D, Gordon KL et al.| title=Impaired angiogenesis in the remnant kidney model: I. Potential role of vascular endothelial growth factor and thrombospondin-1. | journal=J Am Soc Nephrol | year= 2001 | volume= 12 | issue= 7 | pages= 1434-47 | pmid=11423572 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11423572  }} </ref>
**Scarring due to the previous injury  
**[[Scarring]] due to the previous [[injury]]
**Glomerular abnormality  
**[[Glomerular]] abnormality  
**Direct toxic injury to podocytes
**Direct [[toxic]] [[injury]] to [[podocytes]]
*Various inflammatory mediators include
*Various [[inflammatory]] mediators include over-expression of:<ref name="pmid12704576">{{cite journal| author=Fogo AB| title=Animal models of FSGS: lessons for pathogenesis and treatment. | journal=Semin Nephrol | year= 2003 | volume= 23 | issue= 2 |pages= 161-71 | pmid=12704576 | doi=10.1053/snep.2003.50015 | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12704576  }} </ref><ref name="pmid18039119">{{cite journal| author=Kwoh C, Shannon MB, Miner JH, Shaw A| title=Pathogenesis of nonimmune glomerulopathies. | journal=Annu Rev Pathol | year= 2006 | volume= 1 | issue=  | pages= 349-74 | pmid=18039119 | doi=10.1146/annurev.pathol.1.110304.100119 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18039119  }} </ref><ref name="pmid12704579">{{cite journal| author=Hostetter TH| title=Hyperfiltration and glomerulosclerosis. | journal=Semin Nephrol | year= 2003 | volume= 23 | issue= 2 | pages= 194-9 | pmid=12704579 | doi=10.1053/anep.2003.50017 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12704579  }} </ref>
**Overexpression of tumor growth factor-beta ([[TGF-beta]]), [[platelet-derived growth factor]] (PDGF), and [[vascular endothelial growth factor]] (VEGF)<ref name="pmid12704576">{{cite journal| author=Fogo AB| title=Animal models of FSGS: lessons for pathogenesis and treatment. | journal=Semin Nephrol | year= 2003 | volume= 23 | issue= 2 |pages= 161-71 | pmid=12704576 | doi=10.1053/snep.2003.50015 | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12704576  }} </ref><ref name="pmid18039119">{{cite journal| author=Kwoh C, Shannon MB, Miner JH, Shaw A| title=Pathogenesis of nonimmune glomerulopathies. | journal=Annu Rev Pathol | year= 2006 | volume= 1 | issue=  | pages= 349-74 | pmid=18039119 | doi=10.1146/annurev.pathol.1.110304.100119 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18039119  }} </ref><ref name="pmid12704579">{{cite journal| author=Hostetter TH| title=Hyperfiltration and glomerulosclerosis. | journal=Semin Nephrol | year= 2003 | volume= 23 | issue= 2 | pages= 194-9 | pmid=12704579 | doi=10.1053/anep.2003.50017 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12704579  }} </ref>
**[[TGF-beta|Tumor growth factor-beta]] ([[TGF-beta]])
**[[Platelet-derived growth factor]] ([[PDGF]])
**[[Vascular endothelial growth factor]] ([[VEGF]])


===Genetics===
==Genetics==
The development of focal segmental glomerulosclerosis is the result of multiple genetic mutations such as:<ref name="pmid9660941">{{cite journal| author=Kestilä M, Lenkkeri U, Männikkö M, Lamerdin J, McCready P, Putaala H et al.| title=Positionally cloned gene for a novel glomerular protein--nephrin--is mutated in congenital nephrotic syndrome. | journal=Mol Cell | year= 1998 | volume= 1 | issue= 4 | pages= 575-82 | pmid=9660941 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9660941  }} </ref><ref name="pmid18039119" /><ref name="pmid16571882">{{cite journal| author=Tryggvason K, Patrakka J, Wartiovaara J| title=Hereditary proteinuria syndromes and mechanisms of proteinuria. | journal=N Engl J Med | year= 2006 | volume= 354 | issue= 13 | pages= 1387-401 | pmid=16571882 | doi=10.1056/NEJMra052131 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16571882  }} </ref><ref name="pmid12764198">{{cite journal| author=Kim JM, Wu H, Green G, Winkler CA, Kopp JB, Miner JH et al.| title=CD2-associated protein haploinsufficiency is linked to glomerular disease susceptibility. | journal=Science | year= 2003 | volume= 300 | issue= 5623 | pages= 1298-300 | pmid=12764198 | doi=10.1126/science.1081068 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12764198  }} </ref><ref name="pmid10514378">{{cite journal| author=Shih NY, Li J, Karpitskii V, Nguyen A, Dustin ML, Kanagawa O et al.| title=Congenital nephrotic syndrome in mice lacking CD2-associated protein. | journal=Science | year= 1999 | volume= 286 | issue= 5438 | pages= 312-5 | pmid=10514378 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10514378  }} </ref><ref name="pmid10700177">{{cite journal| author=Kaplan JM, Kim SH, North KN, Rennke H, Correia LA, Tong HQ et al.| title=Mutations in ACTN4, encoding alpha-actinin-4, cause familial focal segmental glomerulosclerosis. | journal=Nat Genet | year= 2000 | volume= 24 | issue= 3 | pages= 251-6 | pmid=10700177 | doi=10.1038/73456 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10700177  }} </ref><ref name="pmid12953036">{{cite journal| author=Winn MP| title=Approach to the evaluation of heritable diseases and update on familial focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 2003 | volume= 18 Suppl 6 | issue=  | pages= vi14-20 | pmid=12953036 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12953036  }} </ref><ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408  }} </ref>
The development of focal segmental glomerulosclerosis is the result of multiple [[genetic mutations]] such as:<ref name="pmid9660941">{{cite journal| author=Kestilä M, Lenkkeri U, Männikkö M, Lamerdin J, McCready P, Putaala H et al.| title=Positionally cloned gene for a novel glomerular protein--nephrin--is mutated in congenital nephrotic syndrome. | journal=Mol Cell | year= 1998 | volume= 1 | issue= 4 | pages= 575-82 | pmid=9660941 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9660941  }} </ref><ref name="pmid18039119" /><ref name="pmid16571882">{{cite journal| author=Tryggvason K, Patrakka J, Wartiovaara J| title=Hereditary proteinuria syndromes and mechanisms of proteinuria. | journal=N Engl J Med | year= 2006 | volume= 354 | issue= 13 | pages= 1387-401 | pmid=16571882 | doi=10.1056/NEJMra052131 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16571882  }} </ref><ref name="pmid12764198">{{cite journal| author=Kim JM, Wu H, Green G, Winkler CA, Kopp JB, Miner JH et al.| title=CD2-associated protein haploinsufficiency is linked to glomerular disease susceptibility. | journal=Science | year= 2003 | volume= 300 | issue= 5623 | pages= 1298-300 | pmid=12764198 | doi=10.1126/science.1081068 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12764198  }} </ref><ref name="pmid10514378">{{cite journal| author=Shih NY, Li J, Karpitskii V, Nguyen A, Dustin ML, Kanagawa O et al.| title=Congenital nephrotic syndrome in mice lacking CD2-associated protein. | journal=Science | year= 1999 | volume= 286 | issue= 5438 | pages= 312-5 | pmid=10514378 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10514378  }} </ref><ref name="pmid10700177">{{cite journal| author=Kaplan JM, Kim SH, North KN, Rennke H, Correia LA, Tong HQ et al.| title=Mutations in ACTN4, encoding alpha-actinin-4, cause familial focal segmental glomerulosclerosis. | journal=Nat Genet | year= 2000 | volume= 24 | issue= 3 | pages= 251-6 | pmid=10700177 | doi=10.1038/73456 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10700177  }} </ref><ref name="pmid12953036">{{cite journal| author=Winn MP| title=Approach to the evaluation of heritable diseases and update on familial focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 2003 | volume= 18 Suppl 6 | issue=  | pages= vi14-20 | pmid=12953036 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12953036  }} </ref><ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408  }} </ref>
*Nephrin gene in congenital Finnish-type nephrotic syndrome - ''NPHS1''
*[[Nephrin]] [[gene]] in [[congenital]] Finnish-type [[nephrotic syndrome]] - ''NPHS1''
*Nephrin-like transmembrane gene  - ''NEPH1''
*[[Nephrin]]-like [[transmembrane]] [[gene]] - ''NEPH1''
*Podocin gene - ''NPHS2''
*[[Podocin]] [[gene]] - ''[[NPHS2]]''
*CD2-associated protein (CD2AP)
*[[CD2]]-associated [[protein]] ([[CD2AP]])
*Alpha-actinin-4 gene  
*Alpha-[[actinin]]-4 [[gene]]
*Transient receptor potential cation channel - TRPC6
*Transient [[receptor]] [[potential]] [[cation]] channel - [[TRPC6]]
*Mutation in wilms tumor gene - ''WT1''
*[[Mutation]] in [[wilms tumor]] [[gene]] - ''[[WT1]]''


*Mutation in SCARB2 (LIMP2) gene
*[[Mutation]] in [[SCARB2]] (LIMP2) [[gene]]


*Mutation in formin gene - ''INF2''
*[[Mutation]] in formin [[gene]] - ''[[INF2]]''


*Mitochondrial cytopathies
*[[Mitochondrial]] cytopathies


==Associated Conditions==
==Associated Conditions==
 
Conditions associated with focal segmental glomerulosclerosis (FSGS):<ref name="pmid23431071">{{cite journal |vauthors=Hogan J, Radhakrishnan J |title=The treatment of minimal change disease in adults |journal=J. Am. Soc. Nephrol. |volume=24 |issue=5 |pages=702–11 |date=April 2013 |pmid=23431071 |doi=10.1681/ASN.2012070734 |url=}}</ref><ref name="pmid21184928">{{cite journal |vauthors=Collins AJ, Foley RN, Herzog C, Chavers B, Gilbertson D, Ishani A, Kasiske B, Liu J, Mau LW, McBean M, Murray A, St Peter W, Guo H, Gustafson S, Li Q, Li S, Li S, Peng Y, Qiu Y, Roberts T, Skeans M, Snyder J, Solid C, Wang C, Weinhandl E, Zaun D, Arko C, Chen SC, Dalleska F, Daniels F, Dunning S, Ebben J, Frazier E, Hanzlik C, Johnson R, Sheets D, Wang X, Forrest B, Constantini E, Everson S, Eggers P, Agodoa L |title=US Renal Data System 2010 Annual Data Report |journal=Am. J. Kidney Dis. |volume=57 |issue=1 Suppl 1 |pages=A8, e1–526 |date=January 2011 |pmid=21184928 |doi=10.1053/j.ajkd.2010.10.007 |url=}}</ref><ref name="pmid3070550">{{cite journal |vauthors=Cohen AH, Nast CC |title=HIV-associated nephropathy. A unique combined glomerular, tubular, and interstitial lesion |journal=Mod. Pathol. |volume=1 |issue=2 |pages=87–97 |date=March 1988 |pmid=3070550 |doi= |url=}}</ref><ref name="pmid24840607">{{cite journal |vauthors=Ataga KI, Derebail VK, Archer DR |title=The glomerulopathy of sickle cell disease |journal=Am. J. Hematol. |volume=89 |issue=9 |pages=907–14 |date=September 2014 |pmid=24840607 |pmc=4320776 |doi=10.1002/ajh.23762 |url=}}</ref><ref name="pmid21074826">{{cite journal |vauthors=Gopalakrishnan I, Iskandar SS, Daeihagh P, Divers J, Langefeld CD, Bowden DW, Hicks PJ, Rocco MV, Freedman BI |title=Coincident idiopathic focal segmental glomerulosclerosis collapsing variant and diabetic nephropathy in an African American homozygous for MYH9 risk variants |journal=Hum. Pathol. |volume=42 |issue=2 |pages=291–4 |date=February 2011 |pmid=21074826 |pmc=3022108 |doi=10.1016/j.humpath.2010.07.016 |url=}}</ref><ref name="pmid28509272">{{cite journal |vauthors=Hanaoka H, Hashiguchi A, Konishi K, Kuwana M, Takeuchi T |title=An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy? |journal=CEN Case Rep |volume=4 |issue=1 |pages=70–75 |date=May 2015 |pmid=28509272 |pmc=5411626 |doi=10.1007/s13730-014-0142-1 |url=}}</ref><ref name="pmid24599252">{{cite journal |vauthors=Brown EJ, Pollak MR, Barua M |title=Genetic testing for nephrotic syndrome and FSGS in the era of next-generation sequencing |journal=Kidney Int. |volume=85 |issue=5 |pages=1030–8 |date=May 2014 |pmid=24599252 |pmc=4118212 |doi=10.1038/ki.2014.48 |url=}}</ref>
==Gross Pathology==
*[[Diabetes]]
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
*[[Human Immunodeficiency Virus (HIV)|Human immunodeficiency virus (HIV)]]
*[[Sickle-cell disease|Sickle cell disease]]
*[[Systemic lupus erythematosus|Systemic lupus erythematosus (SLE)]]
*[[Nephrotic syndrome]]
*[[End stage renal disease|End stage renal disease (ESRD)]]
*[[Minimal change disease]]


==Microscopic Pathology==
==Microscopic Pathology==
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
On [[microscopic]] [[histopathological]] analysis progressive changes seen are:<ref name="pmid18039119">{{cite journal |vauthors=Kwoh C, Shannon MB, Miner JH, Shaw A |title=Pathogenesis of nonimmune glomerulopathies |journal=Annu Rev Pathol |volume=1 |issue= |pages=349–74 |date=2006 |pmid=18039119 |doi=10.1146/annurev.pathol.1.110304.100119 |url=}}</ref><ref name="pmid17216262">{{cite journal |vauthors=Reidy K, Kaskel FJ |title=Pathophysiology of focal segmental glomerulosclerosis |journal=Pediatr. Nephrol. |volume=22 |issue=3 |pages=350–4 |date=March 2007 |pmid=17216262 |pmc=1794138 |doi=10.1007/s00467-006-0357-2 |url=}}</ref>
*Foot process [[effacement]]
*[[Podocyte]] [[apoptosis]]
*Exposed [[glomerular basement membrane]]
*[[Capillary]] expansion
*Formation of synechiae
*Mesangial [[matrix]] [[proliferation]]


==References==
==References==
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[[Category:Up-To-Date]]
[[Category:Medicine]]
[[Category:Nephrology]]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [3], Manpreet Kaur, MD [4], Cafer Zorkun, M.D., Ph.D. [5], Olufunmilola Olubukola M.D.[6]

Overview

The pathophysiology of focal segmental glomerulosclerosis (FSGS) is based on two types of FSGS. Primary FSGS is also known as idiopathic FSGS, there is a hypothesis that suggests it occurs as a result of circulating immune activating factors interacting with the glomerular epithelium. The underlying pathogenesis of FSGS is fusion or effacement of the foot processes (podocytes) of the glomeruli and sclerosing of some parts of the glomeruli. These changes result in apoptosis and detachment of the glomerular basement membrane (GBM) resulting in subsequent loss of negative charge on podocytes and podocytopenia. Secondary FSGS is based on glomerular hypertrophy and hyperfiltration and over expression of inflammatory mediators such as, TGF-beta, PDGF and VEGF. The underlying pathogenesis can be based on multiple genetic mutations in NPHS1, NEPH1, NPHS2, WT1 and INF2 genes. Conditions associated with FSGS include, diabetes, HIV, sickle cell disease, nephrotic syndrome and minimal change disease. On microscopic histopathological analysis progressive changes seen are, foot process effacement, podocyte apoptosis, exposed GBM, capillary expansion and mesangial matrix proliferation.

Pathophysiology

There are two types of FSGS, primary FSGS and secondary FSGS, pathophysiology is discussed below:

Pathogenesis of primary FSGS

Pathogenesis of secondary FSGS

The pathogenesis of secondary focal segmental glomerulosclerosis (FSGS) occurs due to the following factors:

Genetics

The development of focal segmental glomerulosclerosis is the result of multiple genetic mutations such as:[12][10][13][14][15][16][17][18]

Associated Conditions

Conditions associated with focal segmental glomerulosclerosis (FSGS):[19][20][21][22][23][24][25]

Microscopic Pathology

On microscopic histopathological analysis progressive changes seen are:[10][26]

References

  1. ↑ 1.0 1.1 1.2 1.3 Reiser J, Nast CC, Alachkar N (2014). "Permeability factors in focal and segmental glomerulosclerosis". Adv Chronic Kidney Dis. 21 (5): 417–21. doi:10.1053/j.ackd.2014.05.010. PMC 4149759. PMID 25168830 PMID 25168830 Check |pmid= value (help).
  2. ↑ Asanuma K, Mundel P (2003). "The role of podocytes in glomerular pathobiology". Clin Exp Nephrol. 7 (4): 255–9. doi:10.1007/s10157-003-0259-6. PMID 14712353.
  3. ↑ 3.0 3.1 Fogo AB (2003). "Animal models of FSGS: lessons for pathogenesis and treatment". Semin Nephrol. 23 (2): 161–71. doi:10.1053/snep.2003.50015. PMID 12704576.
  4. ↑ 4.0 4.1 Wei C, Trachtman H, Li J, Dong C, Friedman AL, Gassman JJ; et al. (2012). "Circulating suPAR in two cohorts of primary FSGS". J Am Soc Nephrol. 23 (12): 2051–9. doi:10.1681/ASN.2012030302. PMC 3507361. PMID 23138488.
  5. ↑ Rea R, Smith C, Sandhu K, Kwan J, Tomson C (2001). "Successful transplant of a kidney with focal segmental glomerulosclerosis". Nephrol Dial Transplant. 16 (2): 416–7. PMID 11158426.
  6. ↑ Ghiggeri GM, Artero M, Carraro M, Perfumo F (2001). "Permeability plasma factors in nephrotic syndrome: more than one factor, more than one inhibitor". Nephrol Dial Transplant. 16 (5): 882–5. PMID 11328888.
  7. ↑ Kemper MJ, Wolf G, Müller-Wiefel DE (2001). "Transmission of glomerular permeability factor from a mother to her child". N Engl J Med. 344 (5): 386–7. doi:10.1056/NEJM200102013440517. PMID 11195803.
  8. ↑ Harris RC, Neilson EG (2006). "Toward a unified theory of renal progression". Annu Rev Med. 57: 365–80. doi:10.1146/annurev.med.57.121304.131342. PMID 16409155.
  9. ↑ Kang DH, Joly AH, Oh SW, Hugo C, Kerjaschki D, Gordon KL; et al. (2001). "Impaired angiogenesis in the remnant kidney model: I. Potential role of vascular endothelial growth factor and thrombospondin-1". J Am Soc Nephrol. 12 (7): 1434–47. PMID 11423572.
  10. ↑ 10.0 10.1 10.2 Kwoh C, Shannon MB, Miner JH, Shaw A (2006). "Pathogenesis of nonimmune glomerulopathies". Annu Rev Pathol. 1: 349–74. doi:10.1146/annurev.pathol.1.110304.100119. PMID 18039119.
  11. ↑ Hostetter TH (2003). "Hyperfiltration and glomerulosclerosis". Semin Nephrol. 23 (2): 194–9. doi:10.1053/anep.2003.50017. PMID 12704579.
  12. ↑ Kestilä M, Lenkkeri U, Männikkö M, Lamerdin J, McCready P, Putaala H; et al. (1998). "Positionally cloned gene for a novel glomerular protein--nephrin--is mutated in congenital nephrotic syndrome". Mol Cell. 1 (4): 575–82. PMID 9660941.
  13. ↑ Tryggvason K, Patrakka J, Wartiovaara J (2006). "Hereditary proteinuria syndromes and mechanisms of proteinuria". N Engl J Med. 354 (13): 1387–401. doi:10.1056/NEJMra052131. PMID 16571882.
  14. ↑ Kim JM, Wu H, Green G, Winkler CA, Kopp JB, Miner JH; et al. (2003). "CD2-associated protein haploinsufficiency is linked to glomerular disease susceptibility". Science. 300 (5623): 1298–300. doi:10.1126/science.1081068. PMID 12764198.
  15. ↑ Shih NY, Li J, Karpitskii V, Nguyen A, Dustin ML, Kanagawa O; et al. (1999). "Congenital nephrotic syndrome in mice lacking CD2-associated protein". Science. 286 (5438): 312–5. PMID 10514378.
  16. ↑ Kaplan JM, Kim SH, North KN, Rennke H, Correia LA, Tong HQ; et al. (2000). "Mutations in ACTN4, encoding alpha-actinin-4, cause familial focal segmental glomerulosclerosis". Nat Genet. 24 (3): 251–6. doi:10.1038/73456. PMID 10700177.
  17. ↑ Winn MP (2003). "Approach to the evaluation of heritable diseases and update on familial focal segmental glomerulosclerosis". Nephrol Dial Transplant. 18 Suppl 6: vi14–20. PMID 12953036.
  18. ↑ Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH; et al. (2013). "KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis". Am J Kidney Dis. 62 (3): 403–41. doi:10.1053/j.ajkd.2013.06.002. PMID 23871408.
  19. ↑ Hogan J, Radhakrishnan J (April 2013). "The treatment of minimal change disease in adults". J. Am. Soc. Nephrol. 24 (5): 702–11. doi:10.1681/ASN.2012070734. PMID 23431071.
  20. ↑ Collins AJ, Foley RN, Herzog C, Chavers B, Gilbertson D, Ishani A, Kasiske B, Liu J, Mau LW, McBean M, Murray A, St Peter W, Guo H, Gustafson S, Li Q, Li S, Li S, Peng Y, Qiu Y, Roberts T, Skeans M, Snyder J, Solid C, Wang C, Weinhandl E, Zaun D, Arko C, Chen SC, Dalleska F, Daniels F, Dunning S, Ebben J, Frazier E, Hanzlik C, Johnson R, Sheets D, Wang X, Forrest B, Constantini E, Everson S, Eggers P, Agodoa L (January 2011). "US Renal Data System 2010 Annual Data Report". Am. J. Kidney Dis. 57 (1 Suppl 1): A8, e1–526. doi:10.1053/j.ajkd.2010.10.007. PMID 21184928.
  21. ↑ Cohen AH, Nast CC (March 1988). "HIV-associated nephropathy. A unique combined glomerular, tubular, and interstitial lesion". Mod. Pathol. 1 (2): 87–97. PMID 3070550.
  22. ↑ Ataga KI, Derebail VK, Archer DR (September 2014). "The glomerulopathy of sickle cell disease". Am. J. Hematol. 89 (9): 907–14. doi:10.1002/ajh.23762. PMC 4320776. PMID 24840607.
  23. ↑ Gopalakrishnan I, Iskandar SS, Daeihagh P, Divers J, Langefeld CD, Bowden DW, Hicks PJ, Rocco MV, Freedman BI (February 2011). "Coincident idiopathic focal segmental glomerulosclerosis collapsing variant and diabetic nephropathy in an African American homozygous for MYH9 risk variants". Hum. Pathol. 42 (2): 291–4. doi:10.1016/j.humpath.2010.07.016. PMC 3022108. PMID 21074826.
  24. ↑ Hanaoka H, Hashiguchi A, Konishi K, Kuwana M, Takeuchi T (May 2015). "An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy?". CEN Case Rep. 4 (1): 70–75. doi:10.1007/s13730-014-0142-1. PMC 5411626. PMID 28509272.
  25. ↑ Brown EJ, Pollak MR, Barua M (May 2014). "Genetic testing for nephrotic syndrome and FSGS in the era of next-generation sequencing". Kidney Int. 85 (5): 1030–8. doi:10.1038/ki.2014.48. PMC 4118212. PMID 24599252.
  26. ↑ Reidy K, Kaskel FJ (March 2007). "Pathophysiology of focal segmental glomerulosclerosis". Pediatr. Nephrol. 22 (3): 350–4. doi:10.1007/s00467-006-0357-2. PMC 1794138. PMID 17216262.


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