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9. Clinical Features
Minimal Change Nephrotic Syndrome >90%
Main manifestation:
• Local edema (periorbital, face, in lower extremities)
• General edema (Anasarca)
• Hydrocele
• Ascites
• hydrothorax
10. Clinical Features
Non-specific symptoms:
• Fatigue and lethargy
• Loss of appetite
• Nausea and vomiting
• abdominal pain and diarrhea
• Body weight increase
• Urine output decrease
• Pleural effusion (Respiratory distress)
11. Complication
Edema
Infection
•Loss of immunoglobulins in urine, use immunosuppressive agents,
malnutrition
•Common infections: URI, Peritonitis, Cellulitis, meningitis, UTI
•Organisms: Pneumococci, H.influenza, Gram negative organisms (E.coli)
•Varicella: Oral acyclovir, I.V acyclovir, after 4 weeks (steroid) V. vaccine
12. Complication
Thrombotic Complication
• Hypercoagulability in NS:
• Loss of anti-thrombin III decrease fibrinolysis
• Higher concentration of I,II,V,VII,X factors and fibrinogen
• higher blood viscosity
• thrombosis (Renal, pulmonary, cerebral)
Acute Renal Failure
• Pre-renal, renal
13. Complication
Hypovolemia
• Low oral intake, vomiting, diarrhea
• Abdominal discomfort, lethargy, dizziness, leg cramps, tachycardia,
hypotension, delayed capillary refill time, low volume pulses and
clammy distal extremities.
• Elevated ratio of blood urea to creatinine, high hematocrit, urine
Na<20mEq/L, Fractional excretion of Na 0.2 – 0.4 % and urinary K
index [urine K+/(urine K+ + urine Na+ )]>0.6
14. Complication
Cardiovascular disease
• Hyperlipidemia may be a risk factor for cardiovascular disease.
Steroid Toxicity
• Cushingoid features, short stature, hypertension, osteoporosis and
sub-capsular cataract.
Others
• Growth retardation, cortical insufficiency
15. Differential
Diagnosis
D.D of generalize edema (Anasarca)
Protein losing enteropathy
Hepatic failure
Heart failure
Protein energy malnutrition [SAM(Kwashiorkor type)]
Acute and chronic glomerulo nephritis
16. Laboratory Data
Urine analysis
• Heavy proteinuria (3 – 4+), selective or non- selective
• Urine collection for protein >40mg/m2/ hour for children
• Oliguria (during stage of edema formation)
• Microscopic hematuria 20%, large number of hyaline cast.
17. Laboratory Data
Blood
• Low serum albumin < 1gr/dl
• Hypercholesterolemia > 220mg/dl, may impart a milky appearance to the plasma.
•
• Normal C3 level, low IgG and high IgM
Renal function
• Blood urea and creatinine (normal range) except when there is hypovolemia and
fall in renal perfusion.
Tuberculin test, urine culture, X-ray (additionally)
19. Management
Definitions regarding course of nephrotic syndrome
Remission: Urine albumin nil or trace for 3 con. Days
Relapse: Urine albumin 3+ or 4+ for 3 consecutive days
Frequent relapses: 4 or more relapses/year
Infrequent relapses: 3 or less relapses/year
20. Management
Definitions regarding course of nephrotic syndrome
Steroid dependence: 2 consecutive relapses when on alternate
day steroids or within 14 days of its discontinuation.
steroid resistance: Absence of remission despite therapy with
daily prednisolone at a dose of 60mg/m2/day 4 week and
alternate day for next 4 weeks.
21. Steroid – Sensitive
NS
General therapy (Non-specific)
• The child should receive a high protein diet.
• Salt is restricted to the amount in usual cooking
(no extra salt).
• Any associated infection is treated.
• Patient should screened for tuberculosis.
• If significant edema restrict fluid intake and diuretics
(Furosemide 1 – 4mg/kg/day in 2 divided doses) alone or with
spironolactone (2-3mg/kg/day in 2 divided doses)
22. Steroid – Sensitive
NS
Drug therapy (Specific)
Management of initial episode:
• Prednisolone or Prednisone 60mg/m2/day (Max 60mg) in
single or divided doses for 6 weeks, followed by 40mg/m2
(Max 40mg) as a single morning dose on alternate days for the
next 6 weeks.
• Initial therapy beyond 12 weeks Corticosteroid Toxicity
Parent Education:
23. Steroid – Sensitive NS (cont…)
Management of Relapses:
• Relapses are often triggered by minor infections.
• Infrequent relapsers (3 or less relapses/year)
Prednisolone 60mg/m2/day protein trace for 3
consecutive days and then on alternate days at a dose
of 40mg/m2 for 4 weeks. (5 – 6 weeks)
24. Steroid – Sensitive NS (cont…)
•Frequent relapsers (4 or more relapses/year) and
steroid dependence
•Long-term alternate day prednisolone
small dose is given on alternate days for 9-18 months.
25. Steroid – Sensitive NS (cont…)
•Patient with repeated relapses while on long-term therapy
• Steroid sparing agents.
Livamisole 2 – 2.5 mg/kg alt. days 1-2year +
Taper prednisolone alt. days (until 0.3-0.5mg/kg) 3-6m
Cyclophosphamide* 2 – 2.5mg/kg/day +
Alternated day prednisolone
Rituximab* 375mg/m2 I.V once a week
remission lasting 6 – 18 months
26. Steroid – Resistant NS
Patient with steroid resistant nephrotic syndrome
The best results are obtained with regimens combining
Calcineurin inhibitors and tapering dose of corticosteroids
+ ACE inhibitors
28. I.V albumin Indication
The use of intravenous albumin is indicated in cases with:
• Symptomatic hypovolemia
10 – 20 ml /kg of 4.5 – 5% albumin should be infused.
• Symptomatic edema + Marked ascites (respiratory compromise)
0.75 – 1 g/kg of 20% albumin, infused over 2 hours
In order to expand the circulating volume followed by furosemide
1mg/kg.
Close monitoring to avoid overload/pulmonary edema