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1. NEPHROTIC
SYNDROME
BY: NIK MOHD FAUZAN

2. NEPHROTIC
SYNDROME
Oedema
Hypoalbuminaemia
<25g/l
Hypercholesterolaemia
Proteinuria .40mg/m2/hour
(>1g/m2/day) or early
morning urine protein
creatinine index
>200mg/mmol

3. AETIOLOGY
Primary or Idiopathic Secondary causes
70-90% of cases 10% of cases
Minimal change disease Membranous nephropathy
- SLE
- Diabetes mellitus
- Hepatitis B
- Sjogren’s disease
- Drugs
- Malignany
Focal segmental glomerulosclerosis
- Diabetes mellitus
- Obesity
- Kidney loss
- HIV
- Hypertensive nephrosclerosis

4. History
◦ AGE : < 6 years old (peak of age)
◦ Symptoms : Tiredness, Edema, Breathless, Oliguria, Frothy urine
◦ Signs : Xantelasma, Periorbital edema, Ascites, Pedal Edema
◦ Family history : Family that having similar presentation
◦ Any clues for complications : Generalize anasarca, Peritonitis, Thrombosis

5. Examinations ??
◦ Height & Weight : Gross edematous can cause increase weight
◦ Blood Pressure : High in AGN & normal in NS
◦ Edema : Periorbital, sacral, genital (scrotum,labium), lower limb
◦ Oral : Injected pharynx and tonsil enlargement  post infectious or recurrent infection
◦ Neck : Cervical lymphadenopathy & JVP for fluid overload
◦ Lung : Reduce A/E ?, Crepitation
◦ Abdomen : Distended ? Soft ? Ascites ? (shiffting dullnes / Fluid trill)

6. Full Blood Count TWC – infection , Hb - Anemia
Renal profile : Urea, Electrolyte,
Creatinine
Renal Impairment (AKI) or Electrolyte
Imbalance
Serum Cholesterol Hyperlipidemia
Liver Function Test : Albumin Hypoalbunimia
Urinalysis, Urine Culture Why?? – Haematuria, Proteinuria
Quantitative urinary protein excretion Why?? – for diagnosis
What to expect ? More > 200mg/mmol
If urine PCI sent, still need to send 24H urine
protein collection? Yes
Issues with 24H urine collection? – Observe
proteinuria (worsening or improving)

7. Serum complement (C3, C4) level What to expect?
C3 level – low at onset symptom, normalizes
by 6 weeks
C4 level – usually normal limit in post
streptococcal AGN
ASOT titres Why?? ( ASOT > 200 IU/ml)
What are significant titre – Post streptococcal
infection
Antinuclear factor / anti-dsDNA What to expect? Positive to exclude SLE

8. Management
Non- pharmacological
Normal protein
diet
Fluid restriction
Restriction of
dietary sodium
Pharmacological

9. Management
Non- pharmacological Pharmacological
Penicillin V
(Prophylaxis/
treatment)
125mg BD (1-
5years), 250mg BD
(6-12years), 500mg
BD (>12years)
recommended
Human albumin
(20-25%)
0.5-1g/kg can be use in
symptomatic, grossly
oedematous case,
together with
IV Frusemide at 1-
2mg/kg
Diuretic Steroid therapy
Steroid-sparing
agents

10. Prednisolone 60mg/m2/day for 4 weeks
(max dose 60mg/day)
Followed by alternate-day prednisolone 40mg/m2/day for 4 weeks (max dose 40mg/day), the taper
over 4 weeks and stop

11. Referral to
Paediatric
Nephrologist
Failure to
achieve
response to
initial 4 weeks
treatment
Urine dipstix
trace or nil
for 3
consecutive
days
Achieve
remission
within 28
days (80%)

12. Infrequent
Relapse
< 2 relapse within
6 month or < 4
relapse within 1
years
Induction with
prednisolone at
dose 60mg/m2/day
(max dose
60mg/day) until
remission
THEN
40mg/m2/EOD
(max dose
40mg/day) for 4
weeks then stop
Frequent
Relapse
≥2 relapses within
6 month or ≥4
relapses within 1
years
Induction with
prednisolone at
dose 60mg/m2/day
(max dose
60mg/day) until
remission,
THEN
40mg/m2/EOD
(max dose
40mg/day) for 4
weeks only.
THEN taper dose
every 2 weeks and
keep on as low on
alternate day dose
as possible for 6
month
Relapse Nephrotic
Syndrome
Urine albumin
excretion
40mg/m2/hour or urine
dipstick > 2+ for 3
consecutive days
No need admission
unless having grossly
edematous or
complication

13. Short stature
Striae
Cataract
Glaucoma
severe cushingoid features
≥2 consecutive relapses
occurring during steroid taper
or within 14 days of cessation
of steroids.
Steroid Dependent
Nephrotic Syndrome
Steroid Non-toxic
Re-induce with steroid
and maintain on as low a
dose alternate day
prednisolone as possible
Steroid Toxic
Consider for steroid-
sparing agent

14. Steroids-Sparing agents
Cyclophosphamide Therapy
Dose : 2-3mg/kg/day for 8-12
weeks (cumulative dose
168mg/kg)
Side effect leucopenia, alopecia,
hemorrhagic cystitis
Counseled about effectiveness
Need to monitor FBC and
urinalysis by 2 weekly
Relapse post
Cyclophosphamide
Treated as for relapse following
the initial diagnosis of nephrotic
syndrome, IF child does not have
sign of steroid toxicity.
Levamisole
Dose 2.5mg/kg on alternate days
for at least 12 month
Calcineurin inhibitor
Cyclosporin
Tacrolimus
Rituximab

16. Complications
Thrombosis
Primary
Peritonitis
Hypovolemia
Infuse Human Albumin
0.5-1.0g/kg/dose fast.
Blood culture,
peritoneal fluid
culture
Parenteral
penicillin and
3rd generation
cephalosporin
Consult with Pediatric Nephrologist

17. REFERENCE
Paediatric protocols for Malaysian Hospital 4th Edition
Essential Medicine Concise Clinical Notes on Understanding & Managing Disease
Robbins and Cotran, Pathologic Basis of Disease, 8th Edition

23. NEPHRITIC
Characterized by inflammation in glomeruli that present with :
◦ Edema : Facial puffiness
◦ Hypertension
◦ Oliguria
◦ Azotemia : High level nitrogen-containing compound
◦ Microscopic / Macroscopic hematuria : Tea colour-urine / Cola like colour / Smoky

24. AETIOLOGY
POST STREPTOCOCCAL AGN ( Group A Beta Hemolytic Streptococci )
◦ Comment causes of acute nephritic syndrome, mainly due to post-streptococcal
Pharynx or skin infection
◦ Commonest at 6-10 years age
◦ Latent period :
◦ After sore throat : 1-2 weeks
◦ After skin infection : 2-3 weeks

25. Major Symptoms
◦ Hematuria (most frequent)
◦ Gross/microscopic >5 RBCs/HPF
◦ Urine Color : deep red-brownish, smoky, cola like color
◦ Hypertension (60-80%)
◦ Monitoring BP 4 Hourly
◦ Due to : General vasospasm, Hypervolemia or Salt water retention
◦ Oliguria (50%) <0.5mk/kg/h. Occur due to reduce GFR
◦ Edema
◦ Course : Begin with puffy eye in morning & LL edema at night

26. Investigation
◦ Urinalysis
◦ Hematuria : present in all patient
◦ Protenuria (trace to 2+, but mayb in nephrotic range)
◦ Red blood cell casts
◦ Pyuria may present
◦ Evidence Streptococcus infection
◦ Throat & Skin swab culture
◦ Raised ASOT (Anti Streptolysin O test) >200IU/ml
◦ Increased anti-DNAse B : better serological marker of preceding streptococcal skin infection
◦ Renal Function Test

27. Investigation
◦ Full Blood Count
◦ Leukocytosis may present
◦ Anaemia
◦ Complement Level
◦ C3 level : Low at onset of symptom, normalies within 6 weeks
◦ C4 level : Usually normal limits in post-streptococcal AGN

28. Management
◦ Strict monitoring : Fluid intake, Urine output, daily weight, BP (Nephrotic chart)
◦ Penicillin V x10/7 to eliminate B-haemolytic streptococcal infection
◦ Fluid restriction to control oedema and circulatory overload during oliguric phase until child diureses
and blood pressure controlled
◦ Day 1 : up to 400ml/m2/day. DO NOT administer IV or oral fluid if child has pulmonary edema
◦ Day 2 : till patient diureses – 400ml/m2/day
◦ When child diureses - allowed free fluid

29. Management
◦ Diuretics (Frusemide) should given pt that having pulmonary edema. Usually needed for treatment
hypertension
◦ Diet – NO added salt to diet. Protein restriction unnecessary
◦ Watch Out for Complication post-streptococcal AGN

30. Follow Up
◦ Atleast 1 year
◦ Monitor BP every visit
◦ Do urinalysis and renal function to evaluate recovery
◦ Repeat C3 levels 6 weeks later if not already normalized by time discharge

31. RENAL BIOPSY
Kidney biopsy is a procedure where a
doctor takes a tiny piece of kidney tissue
using a special needle. The tissue is
examined under a microscope in the
laboratory

32. Risk of Procedure
Kidney biopsies are usually low-risk procedures. The risk may increase depending on your child’s
condition, age and health.
◦ bleeding into the urine
◦ bleeding around or into the kidney
◦ infection
◦ needle injury to any other nearby organ
◦ urine leak from the kidney
◦ kidney damage

33. Procedure
1. Your child will be lying prone.
2. The interventional radiologist uses ultrasound to view the kidneys.
3. Local anaesthetic is then injected into the skin to numb the biopsy area.
4. Then, while watching the kidney using the ultrasound, the interventional radiologist passes a special
thin needle into one of the kidneys to get samples. Usually two or three samples are taken
5. The samples are about 2 to 3 centimeters (1 inch) long, and look like a piece of thread. These kidney
samples are then sent to the lab for examination.
6. You child will usually not need any stitches. A small bandage is placed over the biopsy site.
7. A kidney biopsy usually takes 45 minutes to one hour

34. Indication of Renal Biopsy in Nephrotic Syndrome

38. REFERENCE
Paediatric protocols for Malaysian Hospital 4th Edition
Essential Medicine Concise Clinical Notes on Understanding & Managing Disease
Robbins and Cotran, Pathologic Basis of Disease, 8th Edition