This 5-year-old boy presented with generalized body swelling for 1 week. Examination found him to be edematous with ascites. Laboratory tests confirmed nephrotic syndrome with hypoalbuminemia, heavy proteinuria, and hyperlipidemia. The diagnosis was made based on edema, proteinuria over 40mg/m2/hour, hyperlipidemia, and low albumin. Treatment began with prednisolone to induce remission within 4 weeks. Management involved monitoring for hypovolemia or hypervolemia, restricting salt intake, and prescribing antibiotics during relapses.

1. NEPHROTIC SYNDROME

2.  Boy, 5y.o, 25kg
 p/w:
 Generalised body swelling for the past 1/52
 Started around the eye then gradually increasing
 Mother claimed that child initially well
 Afebrile

3.  o/e: child active, alert, grossly edematous
 BP: 95/50 HR:110 RR:28 T:36.9
 CVS: DRNM
 Lung: clear
 p/a: ascites++
 Lower limb: edema+ (pitting)

4.  How do you approach this case?
 Ie: what investigation to take?

5.  1st
step in treating a child with edema: - to
establish presence of hypoproteinemia and
proteinuria
 hypoalbuminemia can occur in the absence of
proteinuria (such as from protein-losing enteropathy)
 edema can occur in the absence of hypoalbuminemia
(for example, in angioedema, capillary leak, venous
insufficiency, congestive heart failure)

6.  We take:
 UFEME/urinalysis
 Urine protein 24h
 Urine protein/creatinine ratio
 LFT
 Lipid profile

7.  2nd
step: to determine causes or nature (primary
or secondary) and signs of kidney damage
 FBC
 BUSE/Creat
 Complement (C3/C4)
 ANA/RF (selected patient)

8.  You’ve taken all the necessary investigations and
traced the results promptly.
 Results came as follows:
 FBC: Hb: 9.8 TW: 8 Plt: 205 Pcv:30
 Buse: Na: 128 K: 4.5 Ur: 3.5 Creat: 42
 LFT: TP: 40 Alb/Glo: 18/22 Alt/AST: 15/32
 UFEME: glu: neg pro:4+ RBC/WBC: 0-1

9.  Urine protein 24 hours: 4g/m2/day
 UPCR: 400 mg/mmol
 Lipid profile: TG: 3.05 HDL/LDL: 8/4

10.  What are the diagnostic criteria in this patient?

11. HOW TO DIAGNOSE NEPHROTIC
SYNDROME?
 Edema
 Proteinuria: >40mg/m2/H (or 1g/m2/day) or a
early morning UPCR of >200 mg/mmol ( >3.5
mg/mg)
 Hyperlipidemia
 Hypoalbuminemia >25 g/l

12. GENERAL MANAGEMENT
 Recommend normal protein diet
 No added salt to the diet when child has oedema.
 Penicillin V 125 mg BD (1-5 years age), 250 mg
BD (6-12 years), 500 mg BD (> 12 years) is
recommended at diagnosis and during relapses,
particularly in the presence of gross oedema.
 Careful assessment of the haemodynamic status.

13.  Check for signs and symptoms which may
indicate
 Hypovolaemia: Abdominal pain, cold
peripheries, poor capillary refill, poor pulse
volume with or without low blood pressure; OR
 Hypervolaemia: Basal lung crepitations,
rhonchi, hepatomegaly, hypertension.
 Fluid restriction is not recommended unless
chronic edematous state

14. DIURETICS?/ALBUMIN?
 Diuretics (e.g. frusemide) is not necessary in
steroid responsive nephrotic syndrome but if
required, use with caution as may precipitate
hypovolaemia.
 Human albumin (20-25%) at 0.5 - 1.0 g/kg can be
used in symptomatic grossly oedematous states
together with IV frusemide at 1-2 mg/kg to
produce a diuresis

15. HOW TO TREAT?
 Initial Diagnosis
 Start t. prednisolone 60mg/m2 (induction dose) for 4
weeks (max 80mg)
 Followed by 40mg/m2 EOD (60mg) for 4 weeks then
to taper down in 4 months (25% decrease each
month)
 Atleast 80% achive remission in 4 weeks (28 days) –
maybe extended to 6 weeks
 If unable to achieve remission – child should be
referred to nephrologist for biopsy

16. UNDERSTANDING TERMS…
 Remission: having urine dipstick protein level nil
to trace for 3 consecutive days within 28 days of
adequate steroid therapy.
 Steroid resistant nephrotic syndrome: did not
achieve remission after initial treatment of
prednisolone 60 mg/m2
 Relapse: urine albumine excretion of 40mg/m2/h
or urine dipstick of 2+ or above in 3 consecutive
days

17.  Frequent relapse: ≥ 2 relapses within 6 months of
initial diagnosis or ≥ 4 relapses within any 12
month period.
 Steroid dependent nephrotic syndrome: ≥ 2
consecutive relapses occurring during steroid
taper or within 14 days of the cessation of
steroids.

19. TREATING STEROID RESISTANT
NEPHROTIC SYNDROME
 If the child is not steroid toxic, re-induce with
steroids and maintain on as low a dose of
alternate day prednisolone as possible.
 If the child is steroid toxic (short stature, striae,
cataracts, glaucoma, severe cushingoid features)
consider cyclophosphamide therapy.

20. COMPLICATIONS
 Hypovolaemia.
 Clinical features: abdominal pain, cold peripheries,
poor pulse volume, hypotension, and
haemoconcentration.
 Treatment: infuse Human Albumin at 0.5 to 1.0
g/kg/dose fast.
 If human albumin is not available, other volume
expanders like human plasma can be used. Do not
give Frusemide.

21.  Primary Peritonitis
 Clinical features: fever, abdominal pain and
tenderness in children with newly diagnosed or
relapse nephrotic syndrome.
 Investigations: Blood culture, peritoneal fluid culture
(not usually done)
 Treatment: parenteral penicillin and a third
generation cephalosporin
 Thrombosis

22. GENERAL ADVICE
 Counsel regarding risk of relapse
 When to come to hospital?
 Urine protein
 Swelling/edematous
 Immunisation
 While the child is on corticosteroid treatment and
within 6 weeks after itscessation, only killed vaccines
may safely be administered to the child.
 Give live vaccines 6 weeks after cessation of
corticosteroid therapy.
 Pneumococcal vaccine should be administered to all
children with nephrotic syndrome. If possible, give
when the child is in remission.