NEPHROTIC
.
SYNDROME
IE
E KE
C H
O
R
D
It is among the most common kidney disease seen in
children(peak age of 2-6 years)-
years characterised by-
A triad of –
Massive proteinuria
Hypoproteinemia/Hypoalbuminemia
Oedema
Some children may have
Hyperlipidaemia (cholesterolaemia) but it is NOT a consistent
feature in all children with nephrotic syndrome [ a response to
hypoproteinaemia]
Children with this condition often present with a sudden
development of peri-orbital oedema- with or without
generalised oedema
Could be a primary disease or secondary to a variety of
systemic illnesses
Proteinuria > 40mg/m /hr
2
Hypoalbuminemia <2.5gm/L
Protein/creatinine ratio >2
Hyperlidemia(+/-)
CLASSIFICATION
Idiopathic due to primary glomerulopathies that occur in
isolation
Minimal change disease
Focal segmental glomerulosclerosis (FSGS)
Membranous glomerulosclerosis
Mesangioproliferative GN(MPGN)
Mesangiocapillary GN or MPGN
SECONDARY
Infections
- Congenital syphilis, HIV, CMV
- Toxoplasmosis, rubella
hepatitis B & C
- Malaria(P. malariae)
Connective tissue disease
SLE, HSP
Others- Bee sting, heavy metals, SCA, Lymphoma.
DEFINITION OF TERMS
Remission – proteinuria <4gmg/m2/hr, urine dipstick of 0 to
trace for 3 consecutive days in association with resolution of
oedema
Relapse – recurrence of proteinuria >40mg/m 2/hr or albumin
dipstick ≥2+ on 3 consecutive days
Steroid resistance - failure to achieve remission after 4wks
of treatment
DEFINITION OF TERMS CONT’D
Steroid dependent- initial complete remission then relapse
either while weaning steroid or within 2wks of
discontinuation
Frequently relapsing – Patients who develop 4 or more
episodes of NS in a 12 month period.
CLINICAL FEATURES
- Peak age is 2 to 6 years
- incidence of 2-7 per 100000
children and a prevalence of 16
per 100000 or 1 in 6000.
In younger children, boys are twice as
likely to develop NS than girls but
equal ratio in adolescence
TYPICAL NS APPEARANCE
CLINICAL FEATURES
Severe proteinuria
Selective proteinuria
Normal renal function
,
Acute renal failure occur seldomly
Associated with exposure to allergens or immunisations
Associated with Hodgkins disease
PATHOPHYSIOLOGY
Traditional
Reduction in oncotic pressure due to low albumin resulting in
overriding hydrostatic pressure
Podocyte/ Nephrin(a transmembrane
protein involved in maturation of
podocytes & maitenance of glomerular
function) interactions in congenital
NS
Sodium retention unrelated to
renin-angiotensin-aldosterone
pathway.
APPROACH TO MANAGEMENT
Adequate history
Physical examination
Laboratory evaluation
Includes biopsy
MEDICAL MANAGEMENT
Managing a child with
nephrotic syndrome can
be a daunting task!
INITIAL THERAPY
Resuscitation- identifying life threatening conditions.
Shock
Infections
Hypertension
NB: Acute abdomen in a child with NS is usually due to
spontaneous bacterial peritonitis(SBP) & most
implicated organism is Strep. pneumoniae(gram +,
encapsulated)
MANAGEMENT CONT’D
The central role of steroid.
When to start: after diagnosis of NS is made
Who to start: any age group that is responsive
How much to give: 2mg/kg up to a maximum dose of 80mg/day &
a single daily dose
How long to give: -For initial therapy; minimum duration of 8 weeks
-For relapse; give until there is a 3-day absence of
proteinuria, then wean from steroid
When to consider other immunosuppressive
MANAGEMENT CONT’D
Chronic kidney disease, thus adequate information appropriate
for the child and the parents is critical to effective therapy.
Adequate counselling is central
MANAGEMENT CONT’D
Initial drug- Steroid
prednisone at a dose of 2mg/kg to a maximum dose of
80mg/day,
Or at a dose of 60mg/m2
Continue
for 8weeks
Daily
check for proteinuria
MANAGEMENT CONT’D
The International Study of Kidney Disease in Children (ISKDC)
recommends that the initial prednisone dose be given at
2mg/kg for 4wks, then reduced to 40mg/m2 3 days in a
week for the next 4weeks and taper dose subsequently in
STEROID SENSITIVE NS.
MANAGEMENT CONT’D
When to use other agents
Steroid dependent
Frequently relapsing
Steroid resistance
MANAGING DIFFICULT NEPHROTIC
Other drugs
Alkylating agents
Cyclophosphamide
chlorambucil
Calcineurin inhibitors
Cyclosporin
Tacrolimus
MANAGING DIFFICULT NEPHROTIC
Purine antagonists
Azathioprine
Mycophenolate morfetil
Plasma exchange
Plasmapharesis
MANAGING DIFFICULT NEPHROTIC
Non immunosuppressive therapy
ACE inhibitors
Vitamine E
Non steroidal anti inflammatory drugs
Other forms of treatment
Avoidance of salt
Vaccinations
Lipid inhibitory agents
Increased protein intake
NOTE ON CONGENITAL NS
Any child with NS in 1st 12 months of life
Most common in Finland
Incidence of 1.2 in 10,000
Most kids born premature
Huge placenta more than 25% of the total birth weight
Fetal distress is common at birth
Edema present at birth or shortly afterward
NOTE ON CONGENITAL NS
Profound hypoalbuminemia and hypogammaglobulinemia with
associated severe malnutrition, poor growth and high risk of
infection.
Hypothyroidism due to loss of TBP(Thyroxine Binding Protein)
Normal RF
Enlarged hyperechogenic kidneys with poor CMD
NB: Children/infants with congenital NS lose
both high & low molecular weight proteins
unlike in other cases of NS where only low
molecular weight proteins are lost
MANAGEMENT
Resistant to corticosteroid
Daily albumin infusion and gammaglobulin replacement
High protein feeding including TPN
Thyroxine replacement therapy
RRT
COMPLICATIONS
Primary disease
From therapy
