Nephrotic syndrome made easy, it can help you a lot to learn the basics about Nephrotic Syndrome and for more information I recommend the Dr. Najeeb videos about nephrotic and nephritic syndrome.

1. Nephrotic Syndrome
Prepared By: Dr. Merwais Azizyar
Supervisor: Assistant Clinical Professor
Dr. Zabehullah Fazli
2019

2. Introduction
 Definition of NS
 Classification of NS
 Pathophysiology of NS
 Clinical Manifestation of NS
 Complication of NS
 Differential Diagnosis (Edema)
 Laboratory Data
 Management

3. Nephrotic Syndrome
 Definition:
• Heavy proteinuria  hypo-proteinemia
(>1gr/m2/24hour or >3.5gr/24hour) 3 – 4 +
• Hypo- albumineimia
Serum albumin < 2.5gr/dl
• Hyperlipidemia  Hyper cholesterolemia
• Edema
• * HTN
• * Hematuria

4.  Idiopathic Nephrotic Syndrome (MCNS)
• 90%
• Unknown cause
• Respond to steroid therapy, remission 95%
• 10 Years researches suggests
Primary disorder of T- cell functioning
Classification

5.  Secondary Nephrotic Syndrome (NS with significant lesions)<10%
• SLE
• Vasculitis
• Amyloidosis
• Post infection glomerulonephritis
• Hepatitis B nephropathy
Classification

6.  Congenital Nephrotic Syndrome
• 1st three months
• Anasarca, Hypoalbuminemia, Oliguria
Classification

7. Pathophysiology
Minimal change NS > 90%
Nephrotic syndrome with significant lesions
<10%
1- + Selective proteinuria
2- ++ Selective/non-selective proteinuria
3- +++..+ Nephrotic Range
(heavy Proteinuria, Hypoproteinemia, Edema,
hyperlipidemia, hyperlipiduria)
(Edema Oncotic pressure, hydrostatic
pressure, hypovolemia  renal perfusion
RAAA, Renin angiotensin angiotensin 1
angiotensin converting enzyme angiotensin
2 constriction and adrenal cortex
(aldosterone) Ed
Hypothalamus osmo receptor
(osmolality) trigger of ADH Ed

8. Pathophysiology

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)

18. Management
Steroid – Sensitive Nephrotic Syndrome
• General therapy (non- specific)
• Steroid therapy (Specific)
Steroid – Resistant Nephrotic Syndrome

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

27. Steroid – Resistant NS
Cyclosporine 4-5mg/kg/day  12 – 36 months +
Prednisolone tapering doses 18 months +
Enalapril 0.3 – 0.6 mg/kg/day (is associated with
decrease in proteinuria, control hypertension)

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

29. Kidney biopsy
 Renal biopsy considered in:
• Secondary nephrotic syndrome
• Frequent relapsing nephrotic syndrome
• Steroid resistant nephrotic syndrome
• Hematuria
• Hypertension
• Low GFR

30. References
 Ghai
 Nelson

31. Thank you…