Nephrotic Syndromes- MJ Flashcards
Nephrotic syndrome has significantly increased _____ ______ permeability
Basement membrane
What are the essential components of diagnosis of Nephrotic syndrome? (5)
- 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
What is a hallmark finding of nephrotic syndrome?
peripheral edema
- initially presents in dependent areas of body- LE
- occurs when serum albumin is <2g
What are 2 signs/symptoms of nephrotic syndrome?
- Peripheral edema (hallmark finding)
- Dyspnea (pulmonary edema, pleural effusions, diaphragmatic compromise from ascites)
What is seen on urinalysis in nephrotic syndrome?
- Proteinuria (from alteration of GBM)
- Oval fat bodies (associated w/ hyperlipidemia- lipid deposits in sloughed renal tubular epithelial cells)
What is seen on microscopic exam of urinalisis, particularly relating to the proteinuria?
•sediment has few cellular elements or casts
What are 2 characteristic lab findings of nephrotic syndrome?
1. Decreased serum albumin (< 3g)
2. Total serum protein < 6 g
Blood chemistries in nephrotic syndrome:
Hyperlipidemia occurs in > ____% of those w/ early nephrotic syndrome
Why is this?
50%
inc. protein excretion–> oncotic pressure falls–> liver produces more lipids–> decreased clearance of VLDL–> hypertriglyceridemia
What 3 things can be deficient in nephrotic syndrome due to loss of binding proteins in the urine?
Vitamin D, zinc and copper levels
What should be considered in a patient with nephrotic syndrome regarding protein loss?
- 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
How would you treat edema in nephrotic syndrome
- Dietary salt restriction
- Thiazide and loop diuretics used frequently (combo therapy and high doses often required)
How do you treat hyperlipidemia in nephrotic syndrome? (2 ways)
- Dietary modifications and exercise should be advocated
- Aggressive pharmacologic therapy is often required (Statins)
Nephrotic syndrome:
When does a patient become hypercoagulable? Why does this happen?
- Hypercoagulable when serum albumin < 2g
- Urinary loss of antithrombin III, protein C, protein S
- Increased platelet activation
T/F: patients with nephrotic syndrome are prone to renal vein thrombosis and other venous thromboemboli because they can become hypercoagulable?
True
•Those with renal vein thrombosis, pulmonary embolus, or recurrent thromboemboli require anticoagulation indefinitely
What are the 3 types of nephrotic syndromes?
1. Minimal change Disease (MC kids)
2. Membranous nephropathy (MC adults)
3. Focal Segmental glomerulosclerosis (FSGS)
More common in kids or adults:
- Minimal change disease?
- Memranous nephropathy?
- Minimal change disease- Kids
- Memranous nephropathy- Adults
Pathogenesis of which disease?
- Exact pathogenesis unclear
- Increased glomerular permeability
- Foot process effacement (of podocytes)
Minimal Change Disease
20-25% of adults w/ nephrotic syndrome has which disease?
Minimal Change Disease
Is Minimal Change Disease MC in men or women?
- M=F in adults
- Boys > girls in children
What is the MC cause of primary nephrotic syndrome in adults?
Membranous Nephropathy
Which nephrotic syndrome?
- Idiopathic immune mediated glomerulopathy
- Immune complex deposition in glomerular capillary walls result in increased permeability
Membranous Nephropathy
Although the clinical presentation of Membranous Nephropathy is variable and often asymptomatic, what 2 things are classic for this?
Edema w/ frothy urine
People with Membranous Nephropathy have a high incidence of what?
Venous thromboembolism
(b/c they are hypercoagulable)
In Membranous Nephropathy, labs range from _________ syndrome (30%) to classic nephrotic syndrome
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)
How do you treat Membranous Nephropathy?
ACE or ARB (if BP >125/75)–> this targets reduction of urine protein
Disease course of Membranous Nephropathy:
Subnephrotic responds ______ (poor or well?)
Well
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?
- Nephrotic level proteinuria(>3.5g protein loss in urine):
- 30% spontaneous recovery
- Corticosteroid therapy in pts who fail to improve
Which Nephrotic Syndrome has increased permeability due to podocyte injury?
Focal segmental Glomerulosclerosis (FSGS)
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)
FSGS in Kids
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
Adults
- What is the initial presentation of Focal Segmental Glomerulosclerosis (FSGS)?
- 80% of children and 50% of adults present with what?
- Proteinuria is initial presentation
- 80% of kids and 50% of adults present w/ overt nephrotic syndrome
What 4 meds are used to treat Focal Segmental Glomerulosclerosis (FSGS)? What is each medication used for?
•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
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
High dose corticosteroids (methylprednisolone) for up to 16 weeks, followed by taper
