Focal and segmental glomerulosclerosis (FSGS) is a disease characterized de la Nieta MD, Arias LF, et al., Glomeruloesclerosis focal y segmentaria familiar. Download scientific diagram | Esclerosis Focal y Segmentaria con proliferación mesangial from publication: Nephrotoxicity after recreational drug use. N Engl J Med. Dec 22;(25) doi: /NEJMra Focal segmental glomerulosclerosis. D’Agati VD(1), Kaskel FJ, Falk RJ.
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Focal segmental glomerulosclerosis FSGS is a cause of nephrotic syndrome in children and adolescents, as well as a leading cause of kidney failure in adults. The individual components of the name refer to the appearance of the kidney tissue on biopsy: Five mutually exclusive variants of focal segmental glomerulosclerosis may be distinguished by the pathologic findings seen on renal biopsy: Recognition of these variants may have prognostic value in individuals with primary focal segmental glomerulosclerosis i.
The collapsing variant is associated with higher rate of progression to end-stage renal diseasewhereas glomerular tip lesion variant has a low rate of progression to end-stage renal disease in most patients.
Cellular variant shows similar clinical presentation to collapsing and glomerular tip variant but has intermediate outcomes between these two variants.
However, because collapsing and glomerular tip variant show overlapping pathologic features with cellular variant, this intermediate difference in clinical outcomes may reflect a sampling bias in cases of cellular focal segmental glomerulosclerosis i.
The prognostic significance of perihilar and NOS variants has not yet been determined. The NOS variant is the most common subtype. Collapsing variant is the most common type of glomerulopathy caused by HIV infection. Focal segmental glomerulosclerosis may develop following acquired loss of nephrons from reflux nephropathy. Another gene that has been associated with this syndrome is the COL4A5 gene. In children and some adults, FSGS presents as a nephrotic syndromewhich is characterized by edema associated with weight gainhypoalbuminemia low serum albumina protein in the bloodhyperlipidemia and hypertension high blood pressure.
In adults, it may also present as kidney failure essclerosis proteinuriawithout a full-blown nephrotic syndrome. From Wikipedia, the free encyclopedia. Focal segmental glomerulosclerosis Light micrograph of focal segmental glomerulosclerosis, hilar variant. This section needs additional citations for verification.
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Focal segmental glomerulosclerosis.
RQ is a common disease-associated allele”. The New York Times. Retrieved 17 May The Last Hours of Natalie Segentaria. Ed Taylor and Michael Kelpie.
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While it is unclear how these effects might lead to FSGS there are a number of theories. Firstly, protein aggregation may have a toxic effect on the podocyte. Secondly, decreased protein half-life or increased affinity for actin binding may alter actin polymerization and thereby affect the podocytes cytoskeletal architecture.
Focal segmental glomerulosclerosis.
This family of ion channels conduct cations in a largely non-selective manner. There are at least 6 mutations in this channel, located throughout the channel. At least one of esclerksis mutations, PQ, leads to increased intracellular calcium influx.
It is unclear how this might lead to FSGS, though it has been proposed that it may result in alteration of podocyte dynamics or podocytopenia. The protein expressed by this gene is expressed in podocytes where it interacts with fyn and synaptopodin.
Focal Segmental Glomerulosclerosis
There is a report that a splicing mutation in this gene was found in two patients with HIV associated FSGS and this led to altered protein translation.
This has been theorized to result in altered actin binding and, thus, alteration of the cytoskeletal podocyte architecture. Esclreosis is believed that these variants arose as a defensive mechanism against Trypanosoma brucei rhodesiense or some other sub-Saharan parasite despite conferring high susceptibility to FSGS when inherited from both parents.
The observation that alterations in this podocyte-expressed formin cause FSGS emphasizes the importance of fine regulation of actin polymerization in podocyte function. Mutations in the NPHS2 gene, which codes for the protein called podocin,  can cause focal segmental glomerulosclerosis. D ICD – Ureter Ureteritis Ureterocele Megaureter.