Many diseases and conditions can affect your kidney function by attacking and damaging the glomeruli, the tiny filtering units inside your kidney where blood is cleaned. These diseases and conditions are called glomerular diseases and can have many different causes. Focal Segmental glomerulosclerosis is a type of glomerular disease and describes scarring sclerosis in your kidney. The scarring of FSGS only takes place in small sections of each glomerulus filter , and only a limited number of glomeruli are damaged at first. Focal Segmental Glomerulosclerosis affects both children and adults.
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This protein crosslinks bundles of actin filaments and is present in the podocyte. Mutations in this protein associated with FSGS result in increased affinity for actin binding, formation of intracellular aggregates, and decreased protein half-life. 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.
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 these 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. 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.
It 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.
In adults, it may also present as kidney failure and proteinuria , without a full-blown nephrotic syndrome.
Complement Activation in Patients with Focal Segmental Glomerulosclerosis
All relevant data are within the paper. Abstract Background Recent pre-clinical studies have shown that complement activation contributes to glomerular and tubular injury in experimental FSGS. Although complement proteins are detected in the glomeruli of some patients with FSGS, it is not known whether this is due to complement activation or whether the proteins are simply trapped in sclerotic glomeruli. We measured complement activation fragments in the plasma and urine of patients with primary FSGS to determine whether complement activation is part of the disease process. Setting and Participants We identified 19 patients for whom samples were available from weeks 0, 26, 52 and The results for these FSGS patients were compared to results in samples from 10 healthy controls, 10 patients with chronic kidney disease CKD , 20 patients with vasculitis, and 23 patients with lupus nephritis.
Focal Segmental Glomerulosclerosis (FSGS)
Las razones que justificarian este aumento son desconocidas en la actualidad. WT-1 pueden adoptar ambos patrones. Por otra parte, polimorfismos en ApoL1 se asocian a mayor riesgo de GFS en afroamericanos pero no en pacientes de otras etnias   . Rq, y no restringir el estudio WT1 a mujeres. En tinciones de hematoxilina y PAS, se aprecian como zonas de contenido amorfo y desestructurado con colapso de las luces capilares. En una misma biopsia pueden apreciarse lesiones de ambos tipos pero se ignora si la patogenia de las lesiones hialinas es o no la misma que la que causa las lesiones de esclerosis.
Glomeruloesclerosis focal segmentaria
GN extracapilar. GN membranosa o extramembranosa. Un aspecto controvertido es la pauta descendente de prednisona tras la respuesta inicial. No hay diferencias en el sexo. La poca evidencia existente no hace recomendable iniciar el tratamiento asociando otro inmunosupresor.