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Flashcards in Nephrotic Syndrome Deck (27)
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1
Q

Five features of nephrotic syndrome

A
Heavy proteinuria
Hypoalbuminemia
Hyperlipidemia
Lipiduria (oval fat bodies + maltese cross)
Edema
2
Q

Five major clinical causes of nephrotic syndrome

A
Minimal Change Disease
Focal segmental glomerulosclerosis
Membranous glomerulopathy
Diabetic glomerulosclerosis
Amyloidosis
3
Q

MCD: Pathogenesis

A

90% of nephrotic syndrome cases in kids 2-6 years. Disorder of the podocyte; leads to defect in glomerular permeability barrier. Increase in size of slit filters regulated by podocyte. (Circulating “permeability factors,” immune related)

4
Q

MCD: Etiology

A

Unclear but mainly suspected immunologic mechanism: we see remissions w/ steroids or cytotoxic agents; T cell abnormalities; disease onset also associated w/ lymphoma in some patients.

Occasionally MCD can occur as drug rxn in pts w/ NSAIDS or lithium.

5
Q

Nephrotic Syndrome Rx

A

Treat both primary disease (immune modulating) and symptoms!
Symptoms: Low sodium diet, loop diuretics
Statins for hyperlipidemia
ARBs/ACE i’s to induce remission of proteinuria + decrease BP
Anticoagulation in some for ^ clotting factors

6
Q

MCD Gross pathology

A

Pale, lipid rich kidneys

due to nephrotic syndrome

7
Q

MCD micropathology

A

“Nil” disease because minimal changes seen on light microscopy. Glomeruli look normal. No immune deposits. But can see proximal tubules stuffed w/ lipid materials

8
Q

MCD treatment

A

HIGHLY responsive to steroids. 50% will never get recurrence.

9
Q

Fogal Segmental Glomerulosclerosis (FSGS) cause

A

Podocyte disease; uncertain whether it represents a progressed MCD or if it’s a separate entity.
Note: Can be unresponsive to corticosteroid therapy

10
Q

FSGS morphology/histology

A

It’s FOCAL, involves a specific subset of glomeruli. The uninvolved glomeruli demonstrate changes of MCD (normal by light microscopy w/ diffuse foot processes)

  • Hyaline deposits (entrapped plasma proteins in areas of sclerosis)
  • involves only a PORTION OF THE TUFT
  • -> lesions can progress to global glomerulosclerosis
  • IgM and C3 in sclerotic lesions
11
Q

FSGS etiology

A
  • Primary/idiopathic FSGS: Unclear, but we see foot process effacement, altered adhesion to GBM and increased permeability of the glomerulus that may be caused by circulating permeability factors
  • Genetic: podocyte mutations
  • Viral: HIV can actually infect these cells (give HAART. See tubular microcysts on microscopy)
  • Drug induced: eg Heroine
  • Adaptive FSGS: result of increased hemodynamic stress on glomerulus; ex: in obesity, reflux nephropathy, renal agenesis, any chronic renal disease. Common consequence of reduction in functioning nephrons during course of any renal disease. Hypertrophy of glomerulus can be adaptive initially but over time becomes maladaptive –> sclerosing
12
Q

Membranous Glomerulopathy (MG) morphology

A

Not glomerulonephritis because there is no proliferation of inflammatory cell infiltration of the glomerulus.
Light microscopy: diffuse and global uniform thickening of GBMs
- ALL GLOMERULI affected uniformly!
- Spikes on GBM (silver stain)
- Bumps on GBM (trichrome red)
- Fluorescence: IgG and C3 dposits on peripheral GBM

13
Q

MG clinical features

A
  • Most common cause of NS in white adults
  • ## insidious onset
14
Q

MG etiology

A
  • 60% of cases are primary, mediated by antibody to a specific podocyte antigen Phospholipase A2 receptor.
  • 40% are secondary
15
Q

4 causes of 2ndary MG

A
  • Infections (hep B, C, syphilis, schistosomiasis, malaria)
  • Autoimmune diseases (SLE, RA)
  • Neoplasm (lung/breast/colon/stomach carcinoma)
  • Meds (gold, Hg, D-penicillin)
  • usually remit after fixing the underlying problem_
16
Q

MG pathogenesis

A

Primary: AUTOIMMUNE. Binding of circulating antibody to podocyte antigen through in site immune complex formation in subepithelium.
HEYMANN NEPHRITIS is prototype.
–> antigen-antibody complex shed off clathrin-coated pits of podocyte into subepithelial space, forming deposits.
Secondary has similar process, but it’s likely that antigen is non-glomerular. Ex; viral antigens in hep B and C.

17
Q

Renal vein thrombosis

A

Dangerous complication of nephrotic syndrome. Seen almost exclusively in pts w/ MG. Due to hypercoagulable state from increased hepatic synth of fibrinogen and coag factors.

18
Q

Diabetic Nephropathy pathogenesis

A

Most common renal disease leading to dialysis and transplantation.

  • Progressive thickening of capilary and arteriolar basement membranes (microangiopathy) which is present in every organ but reaches greatest severity in kidneys and retina.
  • Excessive synthesis of collagenous proteins and glycoproteins from abnormal glucose metab.
  • Leads to glycosylation of BM proteins and the formation of advanced glycation end products (AGE - can see w/ immunostain).
19
Q

Diabetic nephropathy morphology

A
  • thickening of basement membranes
  • Can be diffuse or nodular
  • nodular from exuberant increase in mesangial matrix, forms large nodules.
  • basement membrane thickening and expansion. individual glomeruli are seen w/ increase in mesangial matrix (KW nodules), it’s uniform. you also have thickening of tubule basement membrane
  • hyalinosis in glomerulus and arterioles
  • can see glomerular microaneurysms
20
Q

RX of membranous nephropathy

A
Conservative therapy
Corticosteroids
Alternating steroids/cytotoxics
cyclosporine
mycophenolate
ACTH
Rituximab
21
Q

DM neph gross pathology

A

Enlarged kidney due to increase in all renal compartments

22
Q

Stages of Diabetic renal disease

A

1: hyperfiltration
2: clinically silent
3. incipient nephropathy
4. overt nephropathy
5. ESRD

23
Q

Hyalinosis

A

Insudation of plasma proteins into thickened basement membranes. Appear as waxy, homogenous, eosinophilic material containing focal lipid dropletx. Common in DM.

Can also see in intima or media or arterioles

24
Q

Rx for proteinuria

A

ARB’s and ACE I’s

25
Q

Renal Amyloidosis pathogenesis

A

Deposition of amorphous eosinophilic hyaline substance in tissues. Can be local or systemic in body.
- Two major forms are primary (AL) and secondary (AA);
Cause:
1) dysproteinemias (myeloma, B CEll lymphomas, macroglobulinemia, occult plasma cell dyscrasia): increased light chains (lambda tends to be particularly common myeloma)
2) chronic inflammatory or infectious states (TB, chronic bronchiectasis, osteomyelitis, RA, ankylosing spondylitis, IBS)

26
Q

Amyloidosis Morphology and Clinical Features

A

May deposit in all four renal compartments (glomeruli, vessels, tubular basement membranes, interstitium)
- As glomeruli incrreasingly replaced by amyloid deposits, there is a reduction in glomerular filtration rate, leading to renal insufficiency and CRF.

27
Q

Amyloid gross path

A

large kidneys, pale and waxy