Nephrotic syndrome

Question 1

What is Nephrotic syndrome?

Answer 1

• Childhood nephrotic syndrome is not a disease itself, it’s a group of symptoms that indicate kidney damage. ( particularly damage of the glomeruli, the tiny units within the kidney filter blood)

Question 2

What are the two types of childhood nephrotic syndrome?

Answer 2

• Primary: which is the most common type, which begins in kidneys and affects only the kidneys.
• Secondary: the syndrome is caused by other diseases

Question 3

Nephrotic syndrome causes?

Answer 3

• Glumeroloscrelosis (inside the kidney because scarred)
• General damage to the glomerulus
• Glumerolonephritis
• Diabetes
• Infection

Question 4

Nephrotic syndrome symptoms?

Answer 4

• In children swelling face, followed with swelling of the entire body
• In adults, can present with dependent edema
• Foamy urine
• Thrombotic complications
• Fatigue
• Loss of appetite

Question 5

Differential diagnosis?

Answer 5

• Renal amyloidosis
• Chronic glumerolonephritis
• Myeloma nephropathy
• Diabetic nephrophaty
• Hepatitis B
• Systemic Lupus Erythematous (SLE)

Question 6

Work up (Lab tests)?

Answer 6

• Urine microscopy
• Urinary protein test
• BUN (blood urea nitrogen)
• Kidney biopsy
• Renal imaging
• Creatinine
• C3 and C4 level
• ANA (antinuclear antibodies, to detect autoimmune diseases)
• ANCA (anti-neutrophil cytoplasm antibodies)
• Anti-GBM (anti-glomerular basement membrane)
• Cryoglubulins test ( to see if you have a protein called cryoglubulins protein in your blood)

Question 7

Complications ?

Answer 7

• AK failure (due to hypovolemia)
• Pulmonary edema
• Hypothyroidism
• Hypocalcemia
• Microcytic hypochromic anemia
• Growth retardation
• Vitamin D deficiency

Question 8

Nephrotic syndrome criteria?

Answer 8

• Heavy proteinuria ( >3.5g/day)
• Hypoalbuminea ( < 3.5g/L )
• Edema
• Hyperlipidemia

Question 9

Treatment ?

Answer 9

• In general:
• Bed rest
• Quantity protein diet ( 1g/kg/day )
• Low salt diet ( 3g/day )
• Diuretics ( Thiazide, 25mg)
• Decrease of proteinuria ( ACE, ARB, diet restriction —> anti-hypertensive)
• Main treatment:
• Glucocorticoid (prednisone)
  • Starting dose : 40-60mg/day for 8-12weeks
  • Reducing dose : 10% every 1-2weeks
  • Maintaining dose : 5-15mg for 6-2years
• Alkylating agent ( cyclophosphamide )
  • Dose 600-1000mg ( IV monthly )
  • Total dose 6-8g
• Cyclosponin
  • 5mg/kg/day for 2-3 months, reduce the dose slowly