Nephrotic Syndromes

Question 1

What is the Pathopysiology of Nephrotic Syndromes?

Answer 1

Question 2

What are the 5 clincial characterisitcs of Nephrotic Syndrome?

Answer 2

• Heavy proteinuria (>3.5gm/day)
• Hypoalbuminemia
• Edema
• Hyperlipidemia & lipiduria
• Normal complement levels

Question 3

what is noticed in the serum and urine labs of someone with Minimal Change Disease ?

Answer 3

• Serum: low albumin, normal Creatinine
• Urine: proteinuria, bland urine sediment

Question 4

What is Minimal change diseases main Pathogenesis?

Answer 4

• The primary target is the glomerular epithelial cells (podocyte)
• Injury results in increased glomerular permeability & subsequent massive proteinuria
• No immune-complex deposition
• Non –inflammatory injury
• may involve circulating “ glomerular permeability factors”

Question 5

what the maajor difference between Minimal Change Disease and FSGS?

Answer 5

>90% of children who have Minimal Change Disease have complete remission of proteinuria within 8 weeks of STEROIDS

Question 6

What is seen in the LM, IF, and EM of somone with Minimal change disease ?

Answer 6

• LM: normal ( glomeruli, tubules, vessels)
• IF: no immunoglobulin deposits
• EM:**** Fusion of foot processes & effacement, detachment of basement membrane

Question 7

FSGS presentation?

Answer 7

• Present with insidious onset of asymptomatic proteinuria
• Progression to nephrotic syndrome with massive proteinuria & microscopic hematuria
• Many are hypertensive & have renal insufficiency
• Most patients will have persistent proteinuria & progressive decline in renal function
• ESRD by 5-20 years
• 50% recurrence post transplant

Question 8

FSGS Causes Map

Answer 8

Question 9

What causes Secondary FSGS?

Answer 9

• Anything causing a reduction in renal mass will result in compensatory
hyperfiltration in remaining glomeruli leading injury pattern {FSGS}

*Renal ablation nephropathy (partial nephrectomy)

** Glomerulonephritis

** Congenital unilateral renal agenesis or aplasia

Question 10

What is seen in LM,IF, and EM of someone with FSGS?

Answer 10

• LM: FSGS

*IF: negative/ or non specific granular deposits of IgM

* EM: patchy fusion of the foot processes & effacement &C3

Question 11

What is the clincal presentation of someone with Membranous nephropathy?

Answer 11

• Present with nephrotic syndrome, microscopic hematuria (50%), HTN, renal insufficiency (late), renal vein thrombosis

• 20 years follow up: 25% of patients have spontaneous remission, 50% persistent proteinuria and stable or only loss of renal
function, 25% develop ESRD

• Poor prognosis: male, > 50 age, >10 gm proteinuria

Question 12

Where are immune complexes deposited in Membranous nephropathy?

Answer 12

• Localization of ICs in sub-epithelial zone as result of IC formation in situ or the deposition of circulating ICs

Question 13

Pathogenesis map of Membranous nephropathy

Answer 13

Question 14

What are the LM, IF , and EM findings in Membranous nephropathy ?

Answer 14

• LM: diffuse thickening of the glomerular basement membrane with little increase of cellularity
• IF: Fine granular deposits of IgG, C3 along the basement membrane – subepithelial
• EM: subepithelial immune complex deposits and proliferation & growth of new GBM “ spikes” formation

Question 15

What is the Pathogenesis map of Diabetic nephropathy ?

Answer 15

Question 16

What are the clincal presentation of Diabetic nephropathy Pathology?

Answer 16

• Earliest lesions: expansion of mesangial matrix & thickening of GBM
• Later lesions: diffuse global glomerulosclerosis with: - Diffuse increase in mesangial matrix & diffuse thickening of GBM
• **Kimmelstiel-Wilson nodules{nodular glomerulosclerosis}
  nodules contain lipids & fibrin
• Fibrin Cap & capsular drop ( plasma proteins )
• Ischemia: causes tubular atrophy, interstitial fibrosis
• Hyaline arteriolosclerosis

Question 17

what is seen in the LM, IF, aand EM of Amyloidosis?

Answer 17

• LM: nodular, amorphous hyaline material in the mesangium & capillary loops, with resultant narrowing or closing of capillary
lumens

• IF: Congo Red Stain positive with polarizable apple green birefringence
• EM: subendothelial & mesangial fibrils