NEPHROTIC SYNDROME

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

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)

How do you approach this case? Ie: what investigation to take?

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)

We take: UFEME/urinalysis Urine protein 24h Urine protein/creatinine ratio LFT Lipid profile

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)

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

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

What are the diagnostic criteria in this patient?

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

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.

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

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

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

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

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.

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.

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.

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

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.