Nephrotic Syndrome Flashcards
Def of Nephrotic Syndrome
Incidence of Idiopathic Nephrotic Syndrome
- It is the most common form of childhood NS.
- Representing more than 90 percent of cases between 1 and 10 years of age and 50 percent after 10 years of age.
Age in Idiopathic Nephrotic Syndrome
2:8 years
Sex in Idiopathic Nephrotic Syndrome
Male 2 : Female 1
Pathogenesis of Idiopathic Nephrotic Syndrome
- Mechanism of Glomerular Injury
- Circulating non-immune Factors
- Circulating immune Factors
- Mutations in podocyte or slit diaphragm proteins
Mechanism of Glomerular Injury in Nephrotic Syndrome
- Circulating non-immune factors:
Mechanism of Glomerular Injury in Nephrotic Syndrome
- Circulating immune factors:
Mechanism of Glomerular Injury in Nephrotic Syndrome
- Mutations in podocyte or slit diaphragm proteins
Pathogenesis of Idiopathic Nephrotic Syndrome
- Mechanism of Edema
Mechanism of Edema in Nephrotic Syndrome
- Arterial Underfiling
- A reduction in plasma oncotic pressure induced by hypoalbuminemia would seem to favor the movement of fluid out of the vascular space into the interstitium and produce arterial underfilling.
Mechanism of Edema in Nephrotic Syndrome
- Sodium retention
- Sodium retention is due to increased reabsorption in the collecting tubules mainly due to increased activity of the Na-K-ATPase pump in the cortical collecting tubule and relative resistance to atrial natriuretic peptide.
Pathogenesis of Nephrotic Syndrome
- Mechanism of hypoalbuminemia
Pathogenesis of Nephrotic Syndrome
- Mechanism of Proteinurea
Pathogenesis of Idiopathic Nephrotic Syndrome
- Mechanism of Hyperlipidemia
CP of Nephrotic Syndrome
CP of Nephrotic Syndrome
- Edema
What is the major Presenting Symptom of Nephrotic Syndrome?
Edema
Edema in Nephrotic Syndrome
- Manifestation
It becomes clinically detectable when fluid retention exceeds 3-5% of body weight.
Edema in Nephrotic Syndrome
- Sites
- Periorbital edema frequently misdiagnosed as allergy is often the initial symptom.
- Edema is gravity dependent, localized to the lower extremities in the upright position, and to the dorsal part of the body in reclining position.
Edema in Nephrotic Syndrome
- Characters
- This edema is soft and pitting, keeping the marks of clothes or finger pressure.
Edema in Nephrotic Syndrome
- Severity
- Anasarca may develop with ascites, and pleural and pericardial effusions. (If anasarca occurred can’t be AGN)
- Edema of the scrotum and penis, or labia, may be seen.
CP of Nephrotic Syndrome
- Abdominal Pain
- It may be related to rapid formation of ascites or concomitant hypovolemia.
- It is occasionally due to a complication such as peritonitis, thrombosis or rarely, pancreatitis.
CP of Nephrotic Syndrome
- HTN
- Mild in 95% of cases.
- Blood pressure is usually normal but sometimes elevated.
CP of Nephrotic Syndrome
- Macroscopic Hematuria
It is observed in 3% of cases.
CP of Nephrotic Syndrome
- Complications
The disease may also be revealed by a complication
CP of Nephrotic Syndrome
- Asymptomatic
NS is occasionally discovered during a routine urine analysis.
Urine Analysis in Nephrotic Syndrome
- Volume
- Color
- Casts
- Specific Gravity
Investigations for Nephrotic Syndrome
- Urine Analysis
Investigations for Nephrotic Syndrome
- Urine & Blood
Urine Analysis in Nephrotic Syndrome
- Proteinuria
Qualitative Evaluation of Proteinuria in Nehrotic Syndrome
Quantitative Evaluation of Proteinuria in Nehrotic Syndrome
What is the screening test for Proteinuria in Nephrotic Syndrome?
Using urine dipstick
Urine Analysis in Nephrotic Syndrome
- Urine Protein Selectivity
Investigations for Nephrotic Syndrome
- Blood Tests
What is the Confirmatory test for Proteinuria in Nephrotic Syndrome?
- Using 24-hour urine protein
- Using Spot urine protein / creatinine ratio
Blood Tests for Nephrotic Syndrome
- CBC
- PTN
- Lipids
- C3
- Ck
Pathological Dx of Nephrotic Syndrome
When is Renal Bx Indicated in children in Nephrotic Syndrome?
Complications of Nephrotic Syndrome
- Infections
- Thromboembolism
- Hypovolemia
- Renal Insufficiency
- Corticosteroids SE
Most Common Complications of Nephrotic Syndrome
Infections
Bacterial Infections in Nephrotic Syndrome
- most Common Site
- The most common infection is peritonitis.
- Patients may also develop meningitis, pneumonia, UTI and cellulitis.
Bacterial Infections in Nephrotic Syndrome
- Organism
- Streptococcus pneumoniae “most common”
- Others: E. coli, streptococcus B, Haemophilus influenzae & other G-ve organisms
Complications in Nephrotic Syndrome
- Thromboembolism
Most Dangerous Complication in Nephrotic Syndrome
Thromboembolism
Thromboembolism in Nephrotic Syndrome
- Sites
- Both arterial and venous thromboses have been reported.
- Most common: pulmonary artery, renal vein, deep leg veins, inferior vena cava, and femoral/iliac artery.
- Others: cerebral and meningeal arteries, mesenteric and hepatic veins.
Complications in Nephrotic Syndrome
- Renal Insufficiency
TTT Aspects of Nephrotic Syndrome
- General
- Specific
General TTT of Nephrotic Syndrome
- Diet
General TTT of Nephrotic Syndrome
- Bed Rest
Noooo “cause thrombosis”
General TTT of Nephrotic Syndrome
- Edema
General TTT of Nephrotic Syndrome
- Hypovolemia
General TTT of Nephrotic Syndrome
- Prevention of Complications
Prevention of Complications in Nephrotic Syndrome
- Infections
- S pneumonia: Oral penicillin + Pneumococcal vaccine
- Varicella: Acyclovir + Varicella vaccine
Prevention of Complications in Nephrotic Syndrome
- Thromboembolism
Prevention of Complications in Nephrotic Syndrome
- Hyperlipidemia
It is reasonable to consider a lipid lowering regimen in children with a persistent NS
Specific TTT of Nephrotic Syndrome
- DOC
- Oral steroids: Corner stone
- The commonly used preparation is prednisone.
Specific TTT of Nephrotic Syndrome
- TTT of First Episode
Indications of Empiric TTT of Nephrotic Syndrome with Corticosteroids
TTT of First Episode in Nephrotic Syndrome
- Monitoring
Once a patient responds to steroid therapy, monitoring for proteinuria is required to detect relapses early.
Def of Relapse in Nephrotic Syndrome
Appearance of significant proteinuria (>40 mg/h/ m2 or >50 mg/kg/day or Albustix +++) for 3 consecutive days after having been in remission.
Patients who respond to steroid have one of the following courses:
Treatment of frequent relapsing/ steroid dependent NS:
Treatment of steroid resistant NS (SRNS)
- Due to Genetic Disorder
Treatment of steroid resistant NS (SRNS)
- Not Due to Gentic Disorder
- ACEI and ARB are used.
- Immunosuppressive therapy depends on the underlying pathology.
What are Examples of Steroid Sparing Agents?
- Levamisole
- Cyclophosphamide
- Cyclosporine
- Myco-phenolate mofetil
Compare between Nephrotic & Nephritic Syndrome in terms of
- Strept inf
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- Edema
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- Oliguria
Compare between Nephrotic & Nephritic Syndrome in terms of
- HTN
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- Hematuria
Compare between Nephrotic & Nephritic Syndrome in terms of
- Serum Albumin
Compare between Nephrotic & Nephritic Syndrome in terms of
- Serum Cholesterol
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- C3 & C4
Compare between Nephrotic & Nephritic Syndrome in terms of
- ASO
Compare between Nephrotic & Nephritic Syndrome in terms of
- Proyeinuria
Compare between Nephrotic & Nephritic Syndrome in terms of
- Casts