Nephrotic Syndrome

Heavy proteinuria, Hypoalbuminemia, Edema (often frank anasarca), Hyperlipidemia

Primary idiopathic renal disorder or result from underlying systemic disease

 

Laboratory

  • Urine: Routine

  • Urine protein : creatinine ratio - First morning sample 

  • Blood urea nitrogen and creatinine

  • Serum total protein and Albumin

  • Serum Cholesterol

  • Serum Electrolytes

  • Total calcium: ionized calcium levels.

  • Complement C3

  • 24-hour urinary protein

 

Laboratory Interpretation

  • Urine: Proteinuria >3+, Hematuria ± (Microscopic/Gross), First morning urine protein : creatinine ratio >2

  • Blood urea nitrogen and creatinine: Normal/slight increase

  • Serum total protein and Albumin: Low (Alb: <2.5g/dL)

  • Serum Cholesterol: Increased

  • Serum Na: Mild hyponatremia ±

  • Total calcium: low; ionized calcium levels: usually normal.

  • Complement C3: Generally normal (low level suggests MPGN, poststreptococcal glomerulonephritis, or lupus nephritis.)

  • 24-hour urinary protein losses:

    • Nephrotic range urinary protein excretion > 40 mg/m2 per hour or 1 g/m2 per day in children

    • In incontinent children, urine protein-to-creatinine ratio > 2:1 on a random urine sample

    • Urinary protein quantification is helpful for monitoring the response to treatment of children with resistant forms NS.

A renal biopsy is not routinely performed if the patient fits the standard clinical picture of MCNS.

 

Specific Treatments

Corticosteroids (Start 4 to 6 --- Maintain 4 to 6 --- Taper 4 wk)

    Prednisone, 2 mg/kg daily or 60 mg/m2 daily (maximum, 60–80 mg/day) for 4–6 weeks

        Usually in divided doses - Can be given as a single morning dose

        Followed by single dose of 1.3 mg/kg or 40 mg/m2 for an additional 4–6 weeks given in the morning on alternate days

       Tapered off over an additional 4 weeks

     

Treatment of relapse (Start till Protein Negative - Maintain 4 - Taper 4 wk)

        Prednisone, 2 mg/kg daily or 60 mg/m2 daily until urine is free of protein for 3 consecutive days

        Dose then changed to 1.3 mg/kg or 40 mg/m2 on alternate days for 4 weeks

        Tapered off over an additional 4 weeks

     

Treatment Approach

    • Long-term outcome depends upon Clinical response to corticosteroids - 85% respond to a trial of prednisone -75% within 2 weeks - 94% by 4 weeks.

    • Biopsy findings do not predict corticosteroid resistant or responsive NS.

    • Who achieve remission on steroids and remain responsive to corticosteroid do not progress to renal failure.

    • Age 1–6 years (most likely to have MCNS) with new onset of typical, pure nephrotic syndrome should be given a trial of corticosteroids.

     

Relapse:

    • Urine Protein ≥ 2+ on 3 consecutive days

    • Usually triggered by intercurrent illnesses or allergies.

    • Parents can be taught to use albumin test sticks or sulfosalicylic acid at home to monitor urinary protein excretion.

Children who have relapse while corticosteroids are being tapered or within 2 weeks of completing a course of corticosteroids are considered corticosteroid dependent and may develop toxicity.

 

When to Refer

    • Complicated nephrotic syndrome

    • Outside the expected age range (< 1 year or > 10 years of age)

    • Accompanied by signs of glomerulonephritis (renal insufficiency, hypertension, hematuria, hypocomplementemia)

    • Refractory edema

    • Frequently relapsing nephrotic syndrome

    • Corticosteroid-dependent nephrotic syndrome

    • Corticosteroid-resistant nephrotic syndrome

 

 

When to Admit

    • Initial episode for teaching of parents, especially if complications +

    • Anasarca interfering with ambulation or compromising ventilation

    • Pleural effusions or ascites interfering with ventilation

    • Signs of volume overload (congestive heart failure)

    • Infection (eg, severe cellulitis, peritonitis)

    • Significant hypertension

    • Significant electrolyte abnormalities

    • Compromised renal function - May require dialysis to manage edema, electrolyte disturbances, and uremia

 

  

Epidemiology

    • Incidence: 2–7 cases per 100,000 children annually

    • Sex: Male : Female Children (2:1);  adolescents and adults (1:1)

    • Children 3 months to 16 years: 76% Minimal Change Nephrotic syndrome (MCNS).

      • Peaks between 2 and 5 years of age.

      • 7–25% - focal segmental glomerulosclerosis (FSGS).

      • 2–5% - diffuse mesangial hypercellularity or mesangial proliferation.

      • 7% - membranoproliferative glomerulonephritis (MPGN).

      • 1% - membranous nephropathy.

         Adolescents are more likely than younger children to have a more aggressive cause, such as FSGS, MPGN, or membranous nephropathy.

 

Major causes include:

    • Membranous glomerulonephritis (MGN, also called membranous nephropathy)

    • Minimal change disease (MCD)

    • Focal and segmental glomerulosclerosis (FSGS)

    • Diabetic nephropathy

    • Glomerular deposition diseases

      •  Amyloidosis

      •  Light chain deposition disease

      •  Fibrillary glomerulonephritis and immunotactoid glomerulonephritis

    • Fabry disease

 

Complications of corticosteroid therapy

    • Development of cushingoid features

    • Cataract formation

    • Glaucoma

    • Gastritis

    • Peptic ulcer disease

    • Pancreatitis

    • Hypokalemia

    • Hypertension

    • Increased risk of infection

    • Behavioral changes

    • Growth delay if treatment is prolonged

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