1. Nephrotic Syndrome
Pediatrics Presentation
Joel E. Rodriguez Ramos
Sunday, February 10, 13

2. Case
• An 11-year-old girl while undergoing tonsillectomy
presented with a urine dipstick of 1+ protein.
Numerous tests revealed normal serum creatinine,
albumin, normal complement C3 and C4 levels, and
normal metabolic chemistry, serology, and
immunology tests. Repeat day time random urine
tests continued to be positive for protein. However,
consequent first morning void urinalysis showed
negative protein by dipstick.
• What is the diagnosis?
Sunday, February 10, 13

3. Definition
• Types of Proteinuria
• Transient Proteinuria - Associated with
physical activity, fever or stress.
• Orthostatic Proteinuria - An increase of
protein glomerular filtration induced by
gravity. Most commonly seen in school
age children and adolescents.
• Fixed Proteinuria - Associated with renal
glomerular lesions or progression to end-
stage renal disease
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4. Definition
• Nephrotic Syndrome
• Proteinuria (24hr urine)
• > 3.5 g/day
• > 40 mg/m²/hr
• Hypoalbuminemia
• < 2.5 g/dL
• Edema
• Orbital
• Pitting
• Dyslipidemia
• Frothy Urine
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5. Differential Diagnosis
• Minimal Change Disease
• Diabetic Glomerulonephropathy
• Focal Segmental Gromerulosclerosis
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6. Did you know?
Minimal Change Disease
is commonly seen in
children!
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7. Minimal Change Disease
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8. Signs and Symptoms
• Age: 2 - 6 years old
• “Puffy” appearance
• Orbital Edema
• Anasarca
• Irritating, Itchy and
Burning
• Rarely Hypertension
Sunday, February 10, 13

9. Diagnostic Test
• U/A with +3 to +4
proteinuria
• 24hr urine > 3.5 g/day
• Increased Creatinine
• Albumin < 2.5 g/dL
• Increased cholesterol and
triglycerides
• Normal C3 and C4 levels
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10. When to consider Biopsy?
• Persistent microscopic or gross hematuria
• Development of hypertension
• Heart Failure
• No response to Tx after 8 weeks
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11. Next Step in Management?
• Mild Disease - Outpatient
• Oral Corticosteroids
• Na+ Restrictions
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12. Next Step in Management?
• Severe Disease - Inpatient
• Fluid and Na+ restrictions
• IV Steroids
• IV Albumin
• Diuretics
• Renal Biopsy
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13. Prognosis
• Repeat remissions with eventual resolution
• Poorer prognosis with steroid treatment resistance
• Complications
• Spontaneous Bacterial Peritonitis
• Thromboembolism
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14. Diabetic Glomerulonephropathy
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15. Signs and Symptoms
• Frothy urine
• Bilateral pitting
edema
• Nueropathy
• Retinopathy
• Hypertension
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16. Diagnostic Test
• U/A with +3 to +4 proteinuria
• 24hr urine > 3.5 g/day
• Microalbuminuria (20 !g/min)
• Increased Creatine, Decreased GFR
• Increased cholesterol and triglycerides
• Normal C3 and C4 levels
• Renal biopsy showing Kimmelstiel-Wilson lesions
Sunday, February 10, 13

17. Next Step in Management?
• Na+ restrictions
• Diuretics
• ACE inhibitors
• Lipid-lowering agents
• Glycemic control
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18. Prognosis
• Poor prognosis if proteinuria occurs earlier in life
• ESRD is the major cause of death, accounting for 2/3 of
deaths
• 1/4 of patients may develop cardiovascular disease
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19. Focal Segmental Glomerulosclerosis
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20. Signs and Symptoms
• Associated with HIV
patients
• Recent URI
• Abrupt weight gain,
about 15-20 lbs
• Anasarca
• Severe Hypertension
• Rarely Pericardial effusions
Sunday, February 10, 13

21. Diagnostic Test
• U/A with +3 to +4 proteinuria
• 24hr urine > 3.5 g/day
• Albumin < 2.5 g/dL
• Normal Creatinine
• Increased cholesterol and triglycerides
• Normal C3 and C4 levels
• Renal biopsy showing sclerosis and hyalinosis
Sunday, February 10, 13

22. Next Step in Management?
• Na+ restrictions
• Diuretics
• ACE inhibitors
• Lipid-lowering agents
• Long term Steroids
• HAART for HIV patients
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23. Prognosis
• Variable progression of disease, generally poor prognosis
• Average time from the onset of proteinuria to ESRD is 6-8
years
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24. Doubts?
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25. References
• Sebestyen J, Alon U. The teenager with asymptomatic proteinuria: think orthostatic first.
Clinical Pediatrics [serial online]. March 2011;50(3):179-182. Available from: MEDLINE with
Full Text, Ipswich, MA. Accessed February 10, 2013.
• Medscape - http://www.emedicine.medscape.com
• Medline Plus - http://www.nlm.nih.gov/medlineplus
• PubMed Health - http://www.ncbi.nlm.nih.gov
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