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NEPHROTIC SYNDROME
By:
HO Arvind
Mentor: Dr Aisyah

DEFINITION
Is a clinical syndrome of massive proteinuria defined by
-oedema
-hypoalbuminemia (<25g/L)
-proteinuria (>1g/m2/day) (>40mg/m2/hour) or
early morning urine protein creatinine index of >200mg/mmol (>3.5
mg/mg)
-hypercholesterolemia

• The kidneys are made up of about a
million filtering units called nephrons.
Each nephron includes a filter, called
the glomerulus, and a tubule.
The glomerulus filters the blood, and
the tubule returns needed substances
to the blood and removes wastes and
extra water, which become urine.
Nephrotic syndrome usually happens
when the glomeruli are damaged,
allowing too much protein to leak from
the blood into the urine.

AETIOLOGY
• Primary or idiopathic (of unknown cause) nephrotic
syndrome is the commonest type of nephrotic syndrome in
children.
• Secondary causes of nephrotic syndrome include post-
streptococcal glomerulonephritis and systemic lupus
erythematosus (SLE).

Clinical Features
1. Marked edema
-periorbital  pitting edema
2. Weight gain
3. Frothy urine
4. Hypertension
5. Hypercoagulable state with increased risk of thrombosis/embolic
event
6. Increased risk of infection

Causes of nephrotic syndrome
• Primary or idiopathic
(of unknown cause) nephrotic syndrome is the commonest type
• Secondary causes of nephrotic syndrome such as
• Henoch–Schönlein purpura and SLE (systemic lupus erythematosus),
• Malignancy—lymphoma, leukaemia
• Infections (e.g. malaria, hepatitis B, CMV, infective endocarditis) or
• allergens (e.g. bee sting)
• post-streptococcal glomerulonephritis
• Congenital nephrotic syndrome in the first 3 months of life. It is rare.
• The most common kind is recessively inherited and the gene frequency is particularly high in
Finns.

Investigations at initial presentation
-Full blood count
-Renal profile: Urea, electrolyte, creatinine
-Serum cholesterol
-Liver function tests, particularly serum albumin
-Urinalysis, urine culture
-Quantitative urinary protein excretion
(spot urine protein: creatinine ratio or 24 hour urine protein)
Other investigations would depend on the age of the patient, associated
renal impairment, hematuria, hypertension or features to suggest an
underlying secondary cause for the nephrotic syndrome.
These tests include:
-Antinuclear factor / anti-dsDNA to exclude SLE.
-Serum complement (C3, C4) levels to exclude SLE, post-infectious
glomerulonephritis.
-ASOT titres to exclude Post-streptococcal glomerulonephritis.
-Other tests as indicated.

COMPLICATION
• 1 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.

• 2. 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
3. Thrombosis
• Thorough investigation and adequate treatment with anticoagulation is usually needed. Please consult a
Paediatric Nephrologist.

Genaral managemant
• A normal protein diet with adequate calories is recommended.
• 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 - not recommended except in chronic oedematous states.
• Diuretics (e.g. frusemide) are not necessary in steroid responsive nephrotic
syndrome but use with caution if required, 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.
• Caution: fluid overload and pulmonary oedema can occur with albumin infusion
especially in those with impaired renal function. Urine output and blood pressure
should be closely monitored.

Intinial treatment
• Once a diagnosis of idiopathic nephrotic syndrome has been established, oral Prednisolone should be started at:
- Initial Prednisolone therapy of 60 mg/m2 per day for 4 weeks
(maximum dose of 60 mg/day), followed by
-Alternate-day prednisolone of 40 mg/m2 per day for 4 weeks
(maximum dose of 40 mg/day), then taper over 4 weeks and stop.
• With this corticosteroid regime, 80% of children will achieve remission (defined as urine dipstix trace or nil for 3
consecutive days) within 28 days.
• Children with Steroid resistant nephrotic syndrome, defined by failure to achieve response to an initial 4 weeks
treatment with prednisolone at 60 mg/m²/ day, should be referred to a Paediatric Nephrologist

Treatment of relapses
• The majority of children with nephrotic syndrome will relapse.
• A relapse is defined by urine albumin excretion > 40 mg/m²/hour or urine dipstix of
≥ 2+ for 3 consecutive days.
• These children do not need admission unless they are grossly oedematous or have
any of the complications of nephrotic syndrome.
Treatment of Initial or Infrequent Relapse
• Induction with Prednisolone at dose of 60 mg/m2 per day (maximum dose of 60 mg/day)
until remission
• then 40mg/m2/EOD (maximum dose 40mg /day) for 4 weeks then stop.

Treatment of frequent relapses
• Defined as ≥ 2 relapses within 6 months of initial diagnosis or ≥ 4 relapses within
any 12 month period.
• Induction of relapse is with oral Prednisolone as follows:
1. 60 mg/m²/day ( maximum 60 mg/day ) until remission followed by
2. 40 mg/m²/EOD (maximum 40 mg) for 4 weeks only.
• Taper Prednisolone dose every 2 weeks and keep on as low an alternate day dose
as possible for 6 months. Should a child relapse while on low dose alternate day
Prednisolone, then re-induce with Prednisolone as for relapse.

Treatment of steroid dependent nephrotic syndrome
• Defined as ≥ 2 consecutive relapses occurring during steroid taper or
within 14 days of the cessation of steroids.
• 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 steroid-sparing agents.

NEPHROTIC SYNDROME.pptx1236ygdsdfhjjhtgedsx

Breakthrough proteinuria/ Intercurrent infections
• Most common relapse trigger is intercurrent infection.
• In patients on weaning or maintenance alternate day prednisolone: Risk of
relapse can be reduced by temporarily increasing the dose from alternate to
every day for 3-5 days.
• Usually does not require corticosteroid induction if the child has no oedema,
remains well and the proteinuria remits with resolution of the infection.
However, if proteinuria persists, treat as a relapse.

• Age <12 months or >12 years.
• Persistent hypertension +/- persistent microscopic hematuria.
• Elevated creatinine despite correction of any hypovolemia.
• C3 or C4 below normal range.
• Unclear if nephrotic versus mixed nephritic-nephrotic (e.g. macroscopic
• haematuria, intravascular fluid overload with hypertension, renal
• impairment).
• Steroid resistance.
• Needing steroid sparing agents beyond oral Cyclophosphamide/Levamisole.
A Paediatric nephrology consultation is recommended if:

Steroid resistant nephrotic syndrome
• Refer for renal biopsy. Specific treatment will depend on the histopathology. General management of the
Nephrotic state:
Control of edema:
Restriction of dietary sodium.
Diuretics e.g. Frusemide, Spironolactone.
• ACE inhibitor e.g. Captopril or Angiotensin II receptor blocker (AIIRB). e.g. Losartan, Irbesartan, to reduce
proteinuria.
• Monitor BP and renal profile 1-2 weeks after initiation of ACE inhibitor or AIIRB.

• Control of hypertension: antihypertensive of choice - ACE inhibitor/AIIRB.
• Penicillin prophylaxis.
• Monitor renal function.
• Nutrition: normal dietary protein content, salt-restricted diet.
• Evaluate calcium and phosphate metabolism.

Acute glomerulonephritis (AGN) is an abrupt onset of one or
more features of an Acute Nephritic Syndrome:
• Oedema e.g. facial puffiness
• Microscopic /macroscopic haematuria (urine: tea-coloured
or smoky)
• Decreased urine output (oliguria)
• Hypertension
• Azotemia
Definition OF ACUTE GLOMERULONEPHRITIS

CAUSES OF ACUTE NEPHRITIS
• Post streptococcal AGN
• Post-infectious acute glomerulonephritis
(other than Grp A ß-Haemolytic Streptococci )
• Subacute bacterial endocarditis
• Henoch-Schoenlein purpura
• IgA nephropathy
• Hereditary nephritis
• Systemic lupus erythematosus
• Systemic vasculitidis

POST STREPTOCOCCAL AGN
• The commonest cause of an acute nephritic syndrome is
post-infectious AGN, mainly due to post-streptococcal
pharynx or skin infection.
• Post streptococcal AGN is commonest at 6 – 10 years age.

CLINICAL FEATURES
• Develops 1-2 weeks after streptococcal infection
• Edema (periorbital, pedal, scrotal)
• Hypertension
• Oliguria
• Hematuria
• Proteinuria
• azotemia

COMPLICATION
1. CNS
-hypertensive encephalopathy: restless, drowsy, seizure, coma, visual
disturbances, nausea, vomiting
2. RS
-pulmonary edema
3. Renal
-irreversible renal failure
4. Blood
-severe uremia
-hyperkalemia

Investigation findings in Post-Streptococcal AGN
• Urinalysis and culture
Haematuria – present in all patients.
Proteinuria (trace to 2+, but may be in
the nephrotic range; usually associated
with more severe disease.)
 Red blood cell casts (pathognomonic of
acute glomerulonephritis).
Other cellular casts.
 Pyuria may also be present.
Bacteriological and serological evidence
of an antecedent streptococcal
infection:
 Raised ASOT ( > 200 IU/ml )
Increased anti-DNAse B (if available) –
a better serological marker of
preceding streptococcal skin infection
Throat swab or skin swab

Renal function test
 Blood urea, electrolytes
and serum creatinine
Full blood count
• Anaemia (mainly dilutional)
• Leucocytosis may be
present
Complement levels
C3 level – low at onset of
symptoms, normalises by 6 weeks
C4 is usually within normal limits in
post-streptococcal AGN
Ultrasound of the kidneys
• Not necessary if patient has clear cut
acute nephritic syndrome

MANAGEMENT
• Strict monitoring - fluid intake, urine output, daily weight, BP (Nephrotic chart)
• Penicillin V for 10 days to eliminate β - haemolytic streptococcal infection (give
erythromycin if penicillin is contraindicated)
• Fluid restriction to control oedema and circulatory overload during the oliguric
phase until child diureses and blood pressure is controlled
• Day 1 : up to 400 mls/m²/day. Do not administer intravenous or oral fluids
if child has pulmonary oedema.
• Day 2 : till patient diureses – 400 mls/m²/day
(as long as patient remains in circulatory overload)
• When child is in diuresis – free fluid is allowed

• Diuretics (e.g. Frusemide) should be given in children with pulmonary
oedema. It is also usually needed for treatment of hypertension.
• Diet – no added salt to diet. Protein restriction is unnecessary
• Look out for complications of post-streptococcal AGN:
• Hypertensive encephalopathy usually presenting with seizures
• Pulmonary oedema (acute left ventricular failure)
• Acute renal failure

Management of severe
complications of post-
streptococcal AGN
• HYPERTENSION
-treatment goal is
reduction of SBP and DBP
to <90th percentile and
<130/80 mmHg in
adolescents >13 years old

• Acute kidney injury
1. Metabolic acidosis
-treat if pH<7.2 or symptomatic
-bicarbonate deficit: 0.3xbody weight (kg)x base excess (BE)
-replace half the deficit with IV 8.4% sodium bicarbonate (1:1 dilution)
-ensure patient’s serum calcium is >1.8 mmol/L to prevent hypocalcemic seizure with
sodium bicarbonate therapy
2. Hypocalcemia
-treat if symptomatic (ca<1.8mmol/L) or if sodium bicarbonate is required for
hyperkalemia
-with IV 10% calcium gluconate 0.5mL/kg, given over 10-20 minutes
3. Hyperkalemia
-serum K>6 in neonate, >5.5 in children
-cardiac toxicity when K>7mmol/L
-ECG changes: tall tented T wave, prolonged PR interval, widened QRS complex,
flattened P wave, sine wave (QRS complex merges with peaked T wave), VF, asystole

Treatment of hyperkalemia
IV 10% calcium gluconate 0.5-1.0 mL/kg (1:1 dilution) over 5-15 mins
IV dextrose 0.5g/kg (2mL/kg of 25%) over 15-30 mins
IV insulin 0.1 unit/kg
IV 8.4% sodium bicarbonate 1mL/kg (1:1 dilution) over 10-30 minutes
Neb 0.5% salbutamol 2.5-5mg (0.5-1mL:3mL 0.9% saline)
Calcium polystyrene sulphonate 0.25g/kg oral or rectally 4x/day (max 10g/dose)
Neonates: per rectal 0.125-0.25g/kg 4x/day
Or
Sodium polysterene sulphonate 1g/kg oral or rectally 4x/day (max 15g/day)

4. Dialysis
-fluid overload manifesting as pulmonary oedema, congestive cardiac
failure, refractory hypertension
-electrolyte/acid-base imbalances: hyperkalemia K>7, symptomatic hypo
or hypernatremia, refractory metabolic acidosis
-symptomatic uraemia
-oliguria preventing adequate nutrition
-oliguria following recent cardiac surgery

Reference
• Paediatric protocols for Malaysian Hospitals 4th Edition
• Tom Lissauer, Will Carroll - Illustrated Textbook of Paediatrics-Elsevier
(2018)
• www.usmle.org

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