Nephrotic Sndrome Flashcards
What is nephrotic syndrome? What is it characterized by?
Glomerulal disorder characterized by proteinuria (>3.5 g/day) resulting in 1. Hypoalbuminemia (pitting edema) 2. Hypogammaglobulinemia (increased risk of infection) 3. Hypercoagulable state (due to loss of antithrombin III) 4. Hyperlipidemia and hyperchoesterolemia (fatty casts in the urine).
What is the most common cause of nephrotic syndrome in Hispanics and African Americans? What is it associated with? What structures are affected? What does it look like on IF? How does it respond to steroids? What does it progress to?
Focal Segmental Glomerulosclerosis (FSGS). May be associated with HIV, heroin use and sickle cell disease. Focal (some glomeruli) and segmental (only part of glomerulus) sclerosis on H & E stain. Effacement of foot processes on EM. No immune complex deposits so negative immunofluoresence. Poor response to steroids. Progresses to chronic renal failure.
What is the most common cause of nephrotic syndrome in Hispanics and African Americans? What is it associated with? What structures are affected? What does it look like on IF? How does it respond to steroids? What does it progress to?
Focal Segmental Glomerulosclerosis (FSGS). May be associated with HIV, heroin use and sickle cell disease. Focal (some glomeruli) and segmental (only part of glomerulus) sclerosis on H & E stain. Effacement of foot processes on EM. No immune complex deposits so negative immunofluoresence. Poor response to steroids. Progresses to chronic renal failure.
What is the most common cause of nephrotic syndrome in Caucaisan adults? What is it associated with? What structures are affected? What does it look like on EM and immunofluorescence (IF)? How does it respond to steroids? What does it progress to?
Membranous Nephropathy. usually idiopathic. May be associated with hepatitis B or C, solid tumors, SLE or drugs. Thich glomerular basement membrane on H & E Due to immun complex deposition (granular IF). Subepithelial cells with ‘spike and dome’ appearance on EM. Poor response to steroids. Progresses to chronic renal failure.
What is the role of C3 nephritic factor? What is it seen in?
Autoantibody that stabilizes C3 convertase, leading to overactivation of complement, inflammation and low levels of circulating C3. Associated with Type II membranoproliferative glomerulonephritis.
How does Diabetes Melitus cause nephrotic syndrome? WHich arteriole is more affected? What does this cause? What can it progress to? What is seen on histology? How is it treated?
High serum glucose leads to nonenzymatic glycosylation of the vascular basemet membrane resulting in hyaline arteriolosclerosis. Glomerular efferent arteriole is more affected than the afferent arteriole, leading to high glomerular filtration pressure. Hyperfiltration injuty leads to micoalbuminuria. Eventually progresses to nephrotic syndrome. Characterized by sclerosis of the mesangium with formation of Kimelstiel-Wilson nodules. ACE inhibitors slow progression of hyperfiltration-induced damage.
How does Diabetes Melitus cause nephrotic syndrome? WHich arteriole is more affected? What does this cause? What can it progress to? What is seen on histology? How is it treated?
High serum glucose leads to nonenzymatic glycosylation of the vascular basemet membrane resulting in hyaline arteriolosclerosis. Glomerular efferent arteriole is more affected than the afferent arteriole, leading to high glomerular filtration pressure. Hyperfiltration injuty leads to micoalbuminuria. Eventually progresses to nephrotic syndrome. Characterized by sclerosis of the mesangium with formation of Kimelstiel-Wilson nodules. ACE inhibitors slow progression of hyperfiltration-induced damage.
What is the most common organ involved in systemic amyloidosis? Where are the deposits? What is it characterized by?
Kidney. Amyloid deposits in the mesangium, resulting in nephrotic syndrome. Characterized by apple-green birefringence under polarized light after staining with congo red.
