Shrunken pore syndrome: Difference between revisions
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[[File:Inner view of fenestrae in capillary of glomerulus in Scanning Electron Microscope, magnification 100,000x.GIF|thumb|Pores in one of the structures of the glomerular filtration barrier]] |
[[File:Inner view of fenestrae in capillary of glomerulus in Scanning Electron Microscope, magnification 100,000x.GIF|thumb|Pores in one of the structures of the glomerular filtration barrier]] |
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'''Shrunken pore syndrome''' (SPS) is a [[kidney disorder]] described in 2015 in which the pores in the [[Glomerulus_(kidney)#Filtration|glomerular filtration barrier]] are hypothesized to have shrunken so that the [[glomerular filtration rate]] (GFR) of 5–30 [[kDa]] [[proteins]], for example [[cystatin C]], is selectively reduced compared to that of small [[molecule]]s (less than 5 kDa) such as water and [[creatinine]].<ref name= Grubb2020/> The [[syndrome]] is associated with premature death.<ref name= Grubb2020/><ref name= Zhou2019/> SPS has been identified in children.<ref name= Grubb2020/> |
'''Shrunken pore syndrome''' (SPS) is a [[kidney disorder]] described in 2015 in which the pores in the [[Glomerulus_(kidney)#Filtration|glomerular filtration barrier]] are hypothesized to have shrunken so that the [[glomerular filtration rate]] (GFR) of 5–30 [[kDa]] [[proteins]], for example [[cystatin C]], is selectively reduced compared to that of small [[molecule]]s (less than 5 kDa) such as water and [[creatinine]].<ref name= Grubb2020/> The [[syndrome]] is associated with premature death.<ref name= Grubb2020/><ref name= Zhou2019/><ref name= Grubb2021/> SPS has been identified in children.<ref name= Grubb2020/><ref name= Grubb2021/> |
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==Mechanism== |
==Mechanism== |
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[[File:Bowman's capsule and glomerulus.svg|thumb|Glomerulus and Bowman's capsule. The blood filtered through the glomerular filtration barrier corresponds to the primary urine collected in the Bowman's capsule.]][[File:Shrunken Pore Syndrome. English.gif|thumb|The figure compares the glomerular barrier in kidneys with and without shrunken pore syndrome]] |
[[File:Bowman's capsule and glomerulus.svg|thumb|Glomerulus and Bowman's capsule. The blood filtered through the glomerular filtration barrier corresponds to the primary urine collected in the Bowman's capsule.]][[File:Shrunken Pore Syndrome. English.gif|thumb|The figure compares the glomerular barrier in kidneys with and without shrunken pore syndrome]] |
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It has been speculated that SPS is both caused by, and exacerbates, [[cardiovascular disease]].<ref name= Grubb2020/> It was stated in 2014 that the kidneys have a role in maintaining the equilibrium between production and [[catabolism]] of most proteins between about 5 and 30 kDa in [[molecular mass]] and that failure to do so results in serious disease and strongly increased mortality, when the kidney disorder shrunken pore syndrome was identified.<ref name= Grubb2020/><ref name=Zhou2019>{{cite journal | vauthors = Zhou H, Yang M, He X, Xu N | title = eGFR, cystatin C and creatinine in shrunken pore syndrome | journal = Clinica Chimica Acta | volume = 498 | pages = 1–5 | date =Nov 2019 | pmid = 31398310 | doi = 10.1016/j.cca.2019.08.001 |type= Review}}</ref> Proteins less than 30 kDa comprise about 36% of the total human [[proteome]].<ref name=Grubb2020/> A [[hypothesis]] concerning the [[pathophysiology]] of SPS is that several 5–30 kDa proteins with [[Cell signaling|signalling]] functions, for example [[cytokines]], are increased in concentration and promote development of serious disorders like cancer and cardiovascular disorders.<ref name=Grubb2020/> |
It has been speculated that SPS is both caused by, and exacerbates, [[cardiovascular disease]].<ref name= Grubb2020/><ref name= Grubb2021/> It was stated in 2014 that the kidneys have a role in maintaining the equilibrium between production and [[catabolism]] of most proteins between about 5 and 30 kDa in [[molecular mass]] and that failure to do so results in serious disease and strongly increased mortality, when the kidney disorder shrunken pore syndrome was identified.<ref name= Grubb2020/><ref name=Zhou2019>{{cite journal | vauthors = Zhou H, Yang M, He X, Xu N | title = eGFR, cystatin C and creatinine in shrunken pore syndrome | journal = Clinica Chimica Acta | volume = 498 | pages = 1–5 | date =Nov 2019 | pmid = 31398310 | doi = 10.1016/j.cca.2019.08.001 |type= Review}}</ref><ref name= Grubb2021/> Proteins less than 30 kDa comprise about 36% of the total human [[proteome]].<ref name=Grubb2020/> A [[hypothesis]] concerning the [[pathophysiology]] of SPS is that several 5–30 kDa proteins with [[Cell signaling|signalling]] functions, for example [[cytokines]], are increased in concentration and promote development of serious disorders like cancer and cardiovascular disorders.<ref name=Grubb2020/><ref name= Grubb2021/> |
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==Diagnosis== |
==Diagnosis== |
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==Prognosis== |
==Prognosis== |
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The mortality of SPS is higher than that of [[cancer]], [[diabetes mellitus]], [[cardiovascular disease]] or [[chronic kidney disease]].<ref name= Grubb2020>{{cite journal | vauthors = Grubb A | title = Shrunken pore syndrome – a common kidney disorder with high mortality. Diagnosis, prevalence, pathophysiology and treatment options | journal = Clinical Biochemistry | volume = 83 | pages = 12–20 | date = Sep 2020 | pmid = 32544475 | doi = 10.1016/j.clinbiochem.2020.06.002 |type= Review | doi-access = free }}</ref> |
The mortality of SPS is higher than that of [[cancer]], [[diabetes mellitus]], [[cardiovascular disease]] or [[chronic kidney disease]].<ref name= Grubb2020>{{cite journal | vauthors = Grubb A | title = Shrunken pore syndrome – a common kidney disorder with high mortality. Diagnosis, prevalence, pathophysiology and treatment options | journal = Clinical Biochemistry | volume = 83 | pages = 12–20 | date = Sep 2020 | pmid = 32544475 | doi = 10.1016/j.clinbiochem.2020.06.002 |type= Review | doi-access = free }}</ref> <ref name= Grubb2021>{{cite journal | vauthors = Grubb A | title = Glomerular filtration and shrunken pore syndrome in children and adults | journal = Acta Paediatrica | date = Mar 2021 | pmid = 33742469 | doi = 10.1111/apa.15846 |type= Review | doi-access = free }}</ref> |
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== References == |
== References == |
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Revision as of 11:32, 7 April 2021
Shrunken pore syndrome (SPS) is a kidney disorder described in 2015 in which the pores in the glomerular filtration barrier are hypothesized to have shrunken so that the glomerular filtration rate (GFR) of 5–30 kDa proteins, for example cystatin C, is selectively reduced compared to that of small molecules (less than 5 kDa) such as water and creatinine.[1] The syndrome is associated with premature death.[1][2][3] SPS has been identified in children.[1][3]
Mechanism
It has been speculated that SPS is both caused by, and exacerbates, cardiovascular disease.[1][3] It was stated in 2014 that the kidneys have a role in maintaining the equilibrium between production and catabolism of most proteins between about 5 and 30 kDa in molecular mass and that failure to do so results in serious disease and strongly increased mortality, when the kidney disorder shrunken pore syndrome was identified.[1][2][3] Proteins less than 30 kDa comprise about 36% of the total human proteome.[1] A hypothesis concerning the pathophysiology of SPS is that several 5–30 kDa proteins with signalling functions, for example cytokines, are increased in concentration and promote development of serious disorders like cancer and cardiovascular disorders.[1][3]
Diagnosis
An eGFRcystatin C/eGFRcreatinine-ratio <0.60, or <0.70, in the absence of non-renal influences on eGFRcystatin C or eGFRcreatinine, identifies a condition as SPS.[1] Optimal classification and stratification of chronic kidney disease requires not only analysis of GFR (estimated or measured) and albuminuria, but also determination of the eGFRcystatin C/eGFRcreatinine-ratio to assess the presence of SPS.[1]
Prognosis
The mortality of SPS is higher than that of cancer, diabetes mellitus, cardiovascular disease or chronic kidney disease.[1] [3]
References
- ^ a b c d e f g h i j Grubb A (Sep 2020). "Shrunken pore syndrome – a common kidney disorder with high mortality. Diagnosis, prevalence, pathophysiology and treatment options". Clinical Biochemistry (Review). 83: 12–20. doi:10.1016/j.clinbiochem.2020.06.002. PMID 32544475.
- ^ a b Zhou H, Yang M, He X, Xu N (Nov 2019). "eGFR, cystatin C and creatinine in shrunken pore syndrome". Clinica Chimica Acta (Review). 498: 1–5. doi:10.1016/j.cca.2019.08.001. PMID 31398310.
- ^ a b c d e f Grubb A (Mar 2021). "Glomerular filtration and shrunken pore syndrome in children and adults". Acta Paediatrica (Review). doi:10.1111/apa.15846. PMID 33742469.