NEPHROTIC SYNDROME Flashcards
What are the key features of Nephrotic Syndrome?
- Edema
- Massive proteinuria
- Hypoalbuminemia
- Hypercholesterolemia
- Hypercoagulable state
What is the definition of massive proteinuria in adults?
> 3.5 g/24h
What is the definition of massive proteinuria in children?
Urine protein:creatinine ratio >2
What is the underfill hypothesis in nephrotic syndrome?
Hypoalbuminemia → reduced plasma oncotic pressure → activation of renin-angiotensin axis → sodium retention
What causes edema formation in nephrotic syndrome according to the overfill hypothesis?
Primary renal sodium retention due to corin-mediated activation of epithelial sodium channel
What are the primary causes of Nephrotic Syndrome?
- Minimal Change Nephrotic Syndrome (MCNS)
- Focal Segmental Glomerulosclerosis (FSGS)
- Membranous Nephropathy (MN)
- Membranoproliferative Glomerulonephritis (MPGN)
What are some secondary causes of Nephrotic Syndrome?
Infections, systemic diseases, medications, malignancies, amyloidosis
What is a common clinical presentation of edema in adults with Nephrotic Syndrome?
Lower extremities
What is a common clinical presentation of edema in children with Nephrotic Syndrome?
Periorbital edema and ascites
What are the diagnostic criteria for Nephrotic Syndrome?
- Urine protein:creatinine ratio >2 in children or >3.5 g/24h in adults
- Blood tests showing hypoalbuminemia and hypercholesterolemia
When is a kidney biopsy deferred in children with Nephrotic Syndrome?
Until steroid resistance
What is the prognosis for Minimal Change Nephrotic Syndrome (MCNS)?
Excellent prognosis, most patients outgrow the disease
What percentage of patients with Focal Segmental Glomerulosclerosis (FSGS) progress to end-stage kidney disease (ESKD) in 5-10 years?
50%
What is the first-line treatment for Nephrotic Syndrome?
Corticosteroids
What are some second-line treatments for Nephrotic Syndrome?
- Calcineurin inhibitors
- Mycophenolate mofetil
- Cyclophosphamide
- Rituximab (under investigation)
What supportive care measures are recommended for Nephrotic Syndrome?
- Dietary sodium restriction
- Diuretics for edema
- ACE inhibitors/ARBs to reduce proteinuria and blood pressure
- Statins for hypercholesterolemia
What genetic mutations are associated with Focal Segmental Glomerulosclerosis (FSGS)?
Mutations in podocyte proteins (e.g., podocin, α-actinin-4)
What is the risk of recurrence for FSGS and MPGN after kidney transplantation?
20-40%
What novel therapies are under investigation for Nephrotic Syndrome?
- Rituximab
- Abatacept
- Anti-TNF antibodies
Fill in the blank: Nephrotic syndrome is characterized by _______ + massive proteinuria + hypoalbuminemia + hypercholesterolemia.
edema
True or False: Corticosteroids have a high response rate in Focal Segmental Glomerulosclerosis (FSGS).
False
What is the definition of massive proteinuria in adults?
> 3.5 g/24h
What is the definition of massive proteinuria in children?
Urine protein:creatinine ratio >2
What is the underfill hypothesis in nephrotic syndrome?
Hypoalbuminemia → reduced plasma oncotic pressure → activation of renin-angiotensin axis → sodium retention
