Nephrotic Syndromes- MJ Flashcards

1
Q

Nephrotic syndrome has significantly increased _____ ______ permeability

A

Basement membrane

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2
Q

What are the essential components of diagnosis of Nephrotic syndrome? (5)

A
  • Urine protein excretion ≥ 3.5 g per 24 hours (LOTS of protein)
  • Hypoalbuminemia (serum albumin < 3 g/dL)
  • Bland urinary sediment
  • Oval fat bodies may be seen
  • Peripheral edema (significant, “tree trunk legs”)
  • Hyperlipidemia
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3
Q

What is a hallmark finding of nephrotic syndrome?

A

peripheral edema

  • initially presents in dependent areas of body- LE
  • occurs when serum albumin is <2g
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4
Q

What are 2 signs/symptoms of nephrotic syndrome?

A
  • Peripheral edema (hallmark finding)
  • Dyspnea (pulmonary edema, pleural effusions, diaphragmatic compromise from ascites)
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5
Q

What is seen on urinalysis in nephrotic syndrome?

A
  • Proteinuria (from alteration of GBM)
  • Oval fat bodies (associated w/ hyperlipidemia- lipid deposits in sloughed renal tubular epithelial cells)
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6
Q

What is seen on microscopic exam of urinalisis, particularly relating to the proteinuria?

A

•sediment has few cellular elements or casts

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7
Q

What are 2 characteristic lab findings of nephrotic syndrome?

A

1. Decreased serum albumin (< 3g)

2. Total serum protein < 6 g

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8
Q

Blood chemistries in nephrotic syndrome:

Hyperlipidemia occurs in > ____% of those w/ early nephrotic syndrome

Why is this?

A

50%

inc. protein excretion–> oncotic pressure falls–> liver produces more lipids–> decreased clearance of VLDL–> hypertriglyceridemia

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9
Q

What 3 things can be deficient in nephrotic syndrome due to loss of binding proteins in the urine?

A

Vitamin D, zinc and copper levels

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10
Q

What should be considered in a patient with nephrotic syndrome regarding protein loss?

A
  • Protein malnutrition (occurs w/ urinary protein loss >10g/d)
  • Daily total dietary protein intake should replace losses through urinary excretion in order to avoid negative nitrogen balance
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11
Q

How would you treat edema in nephrotic syndrome

A
  • Dietary salt restriction
  • Thiazide and loop diuretics used frequently (combo therapy and high doses often required)
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12
Q

How do you treat hyperlipidemia in nephrotic syndrome? (2 ways)

A
  • Dietary modifications and exercise should be advocated
  • Aggressive pharmacologic therapy is often required (Statins)
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13
Q

Nephrotic syndrome:

When does a patient become hypercoagulable? Why does this happen?

A
  • Hypercoagulable when serum albumin < 2g
    • Urinary loss of antithrombin III, protein C, protein S
    • Increased platelet activation
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14
Q

T/F: patients with nephrotic syndrome are prone to renal vein thrombosis and other venous thromboemboli because they can become hypercoagulable?

A

True

•Those with renal vein thrombosis, pulmonary embolus, or recurrent thromboemboli require anticoagulation indefinitely

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15
Q

What are the 3 types of nephrotic syndromes?

A

1. Minimal change Disease (MC kids)

2. Membranous nephropathy (MC adults)

3. Focal Segmental glomerulosclerosis (FSGS)

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16
Q

More common in kids or adults:

  • Minimal change disease?
  • Memranous nephropathy?
A
  • Minimal change disease- Kids
  • Memranous nephropathy- Adults
17
Q

Pathogenesis of which disease?

  • Exact pathogenesis unclear
  • Increased glomerular permeability
  • Foot process effacement (of podocytes)
A

Minimal Change Disease

18
Q

20-25% of adults w/ nephrotic syndrome has which disease?

A

Minimal Change Disease

19
Q

Is Minimal Change Disease MC in men or women?

A
  • M=F in adults
  • Boys > girls in children
20
Q

What is the MC cause of primary nephrotic syndrome in adults?

A

Membranous Nephropathy

21
Q

Which nephrotic syndrome?

  • Idiopathic immune mediated glomerulopathy
  • Immune complex deposition in glomerular capillary walls result in increased permeability
A

Membranous Nephropathy

22
Q

Although the clinical presentation of Membranous Nephropathy is variable and often asymptomatic, what 2 things are classic for this?

A

Edema w/ frothy urine

23
Q

People with Membranous Nephropathy have a high incidence of what?

A

Venous thromboembolism

(b/c they are hypercoagulable)

24
Q
A
25
Q

In Membranous Nephropathy, labs range from _________ syndrome (30%) to classic nephrotic syndrome

A

In Membranous Nephropathy, labs range from Subnephrotic syndrome (30%) to classic nephrotic syndrome

(note: Subnephrotic= <3-3.5g pro in urine,

nephrotic = >3.5g pro in urine)

26
Q

How do you treat Membranous Nephropathy?

A

ACE or ARB (if BP >125/75)–> this targets reduction of urine protein

27
Q

Disease course of Membranous Nephropathy:

Subnephrotic responds ______ (poor or well?)

A

Well

28
Q

Disease course of Membranous Nephropathy:

  • Nephrotic (>3.5g protein loss in urine):
    • ____% spontaneous recovery
    • What do you give patients who fail to improve w/ 6 months of conservative care?
A
  • Nephrotic level proteinuria(>3.5g protein loss in urine):
    • 30% spontaneous recovery
    • Corticosteroid therapy in pts who fail to improve
29
Q

Which Nephrotic Syndrome has increased permeability due to podocyte injury?

A

Focal segmental Glomerulosclerosis (FSGS)

30
Q

The following is the pathogenesis behing Focal Segmental Glomerulosclerosis (FSGS) in kids or adults?

Primary renal disease → largely idiopathic but some demonstrate genetic alterations leading to altered podocyte formation (especially those of African descent)

A

FSGS in Kids

31
Q

The following is the pathogenesis behing Focal Segmental Glomerulosclerosis (FSGS) in kids or adults?

Secondary → obesity, hypertension, chronic urinary reflux, HIV infection, analgesic or bisphosphonate exposure

A

Adults

32
Q
  • What is the initial presentation of Focal Segmental Glomerulosclerosis (FSGS)?
  • 80% of children and 50% of adults present with what?
A
  • Proteinuria is initial presentation
  • 80% of kids and 50% of adults present w/ overt nephrotic syndrome
33
Q

What 4 meds are used to treat Focal Segmental Glomerulosclerosis (FSGS)? What is each medication used for?

A

•Diuretics for edema

  • ACE-I or ARB to reduce proteinuria & HTN
  • Statins for hyperlipidemia
  • Corticosteroids for those w/ primary cause & overt nephrotic syndrome
  • •High dose for up to 16 weeks followed by slow taper
34
Q

Treatment of FSGS:

Other than Diuretics, ACE/ARBs and statins, what other medication do you give to patients with primary cause and overt nephrotic syndrome

A

High dose corticosteroids (methylprednisolone) for up to 16 weeks, followed by taper