Focal Segmental Glomerulosclerosis Flashcards
Definition
Neither a disease nor a Syndrome, but rather a set of clinicopathologic syndromes
Segmental glomerular scars
Often global glomerular tubulointerstitial scarring
Symptoms
Proteinuria (albumin, immunoglobulins (lesser degree))
Edema
Hypoalbuminemia
Hyperlipidemia
Hypertension
Gfr decrease 1/3
Possible microscopic hematuria
Forms of FSGS
primary
Postadaptive
Apol 1
High penetrance Genetik
Virus associated
Mediacation associated
Pathophysiology primary FSGS
Due to circulating factors
Soluable plasminogen activator urokinase type receptor
Cardiotrophin like cytokine 1
Pathophysiology postadaptive FSGS
Post-adaptive FSGS arises from an imbalance between glomerular load (i.e., increased glomerular blood flow, arising from diverse factors) and glomerular capacity (i.e., the maximal effective glomerular capillary surface area, resulting in increased glomerular capillary pressures and, thus, placing podocytes under mechanical stress. Consequent to this maladaptation, glomerular hemody-namic alterations can arise through (1) a reduction in the number of functioning nephrons (such as after unilateral renal agenesis, surgical ablation, oligomeganephronia, or any advanced primary kidney disease) or (2) mechanisms that place hemodynamic stress on an initially normal nephron population (as in morbid obe-sity, cyanotic congenital heart disease, and sickle cell anemia). In postadaptive FSGS, proteinuria may be nephrotic range but is more typically subnephrotic. Plasma albumin concentration may be normal, even in the presence of nephrotic range proteinuria.
Edema may be absent. Renin-angiotensin-aldosterone system (RAAS) antagonism, particularly when coupled with a diuretic and dietary sodium restriction (ideally to 1 g sodium per day), may have a particularly dramatic effect in reducing proteinuria in postadaptive FSGS, and such a response may help confirm the diagnosis of the form of FSGS.
Therapy
Controversial
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