Nephrotic Syndrome

in Children
Supervised by : dr. Pulung M. Silalahi Sp.A
Presented by : Anna Listiana
INTRODUCTION
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BACKGROUND

● Nephrotic syndrome (NS) is a

kidney disease characterized by
proteinuria, hypoalbuminemia,
edema, and hypercholesterolemia
due to renal glomerular disorders.
● Complication is edema, infection
(UTI, peritonitis)
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Epidemiology
● Most common kidney disease in
children (15x), 90% idiopathic,
male:female (2:1), 14 y.o.
● America  Prevalent 12-16 cases
from 100.000 child/year, Incident
2-7 cases from 100.000 child/year
● Development country  Incident 6
Gipson DS. et al. in 223 child with NS
cases from 100.000 child/year
PROBLEM

“How to diagnose and

treat child with nephrotic
syndrome?”

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 PURPOSE

General purpose Special purpose

● To understand ● To understand what are the
how to diagnose definitions, etiologies, and
and treat NS in pathophysiology of NS in children.
children ● To understand the clinical
manifestations and examination of NS
in children.
● To understand the complications and
management of NS in children.
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BENEFIT

Academic
● Increase knowledge about Nephrotic
Syndrome in children
Community
● Provide information to the public
about Nephrotic Syndrome in children
Research
● This paper is expected to be used as a
reference for further referrals relating
to Nephrotic Syndrome in children
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Nephrotic
Syndrome
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Definition
➔ Clinical manifestation of glomerular
diseases, associated with:
◆ Massive Proteinuria >40mg/m2
/BSA /hour OR 50 mg/kg/day
OR Protein:Creatinin Ratio > 2
◆ Hipoalbuminemia < 2,5 g/dL
◆ Edema
◆ Hipercholesterolemia > 200 mg/dL
Etiology of Nephrotic Syndrome
● Primary
Idiopathic, Genetic
● Secondary
Systemic (systemic lupus erythematosus, Henoch-
Schönlein purpura), Infection (endocarditis, HIV,
hepatitis), Neoplasma(lymphoma, leukemia), Drugs
(Captopril, penicillamine, gold), etc
Etiology
Etiology
13
 1 Remission No proteinuria or trace proteinuria <4mg/m2 BSA/day for

Restriction 3 consecutive days in 1 week

2 Relaps Proteinuria ≥2+ or >40mg/m2 BSA/hour, Protein/creatinin

rasio in urine >2 for 3 consecutive days in 1 week

3 Rarely ≤ 2x relapses within 6 months after the initial therapy or ≤

Relapsing 4x in a 12-mo period.

4 Frequently ≥ 2x relapses within 6 months after the initial therapy or ≥

relapsing 4x in a 12-mo period.

5 Steroid Relapse 2x during steroid tapering or a relapse within 2

dependent weeks of the discontinuation of therapy.

6 Steroid Attainment of remission within the initial 4 wk

Sensitive corticosteroid (Prednison 2mg/kg/day) therapy

7 Steroid No remission within the initial 4 wk corticosteroid

Resistant (Prednison 2mg/kg/day) therapy
Patogenesis
● Podocyte → differentiated epithelial cell, outside of the glomerular
capillary loop, it has foot processes that connected by slit diaphragm.
● Function → fltration barrier ( by structural shape and special protein
in slit diaphragm (nephrin, podocin))
Patogenesis

● Any distrubances of this component by idiopathic,

hereditary (mutation of protein), and secondary causes
● May lead to effacement of podocyte, decreasing of
functional podocytes, and altered slit diaphgram, lead to
nephrotic syndrome
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Clinical Manifestation
Edema
● Periorbital (morning), scrotum, pleural
effusion, ascites
o Uderfill → increase permeability of
glomerular capillary wall → protein
leakage → decrease oncotic pressure →
plasma leakage, edema → intravascular
volume depression, RAAS activation →
edema more severe
o Overfill → Water and Na retention in
distal tubule → volume expansion
Clinical Manifestation
Hipoalbuminemia
o Nomally, Liver produce albumin 12-14 g/day or 130-
200mg/kg.
o Nephrotic Syndrome → liver do not produce enough
albumin even in the adequate protein supply →
Hipoalbuminemia <2,5 g/dL
Hipercoagulation
o Increase production of fbrinogen in liver → thrombus in
commonly in lung and kidney
Clinical Manifestation
Proteinuria
o Glomerullar leakage
Hipercholesterolemiaia
o Increase lipid fraction → LDL,VLDL but HDL decrease
o It stimulated as response of protein plasma leakage
Increase Rate of Infection
o Increase risk of infection → globulin (igG,igM,igA) and
complement loss
o Most Common → peritonitis, sellulitis
 Laboratory Investigation

Urinalysis
▰ First morning urine sample (protein:creatinin
ratio)
▰ 24 hours urine sample (dipstick)
Blood Analysis
▰ Complete blood count, serum albumin, lipid
profile, electrolyte, screening of secondary
causes (HIV, Hepatitis, autoimmune)
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 Laboratory Investigation

Kidney Biopsy
▰ Take sample of
patient kidney
using biopsy needle
and USG guide

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 Laboratory Investigation

KIDNEY BIOPSY INDICATION

▰ Nephrotic Syndrome in child < 1 y.o. or > 16 y.o.
▰ Macroscopic and Microscopic hematuria, C3
complement low
▰ Persistent Hipertension
▰ Decrease kidney function (w/o hipovolemia)
▰ Secondary Nephrotic Syndrome
▰ Steroid Resistant
▰ Before cyclosporine therapy
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 Complication

Spontaneous bacterial peritonitis

defcie
Invasion of gut bacteria to the mucosa and n cy
lymph node lead to peritonitis → sepsis
Streptococcus pneumonia, Haemophillus
influenza, group B streptococcus, Escherichia coli
 General Therapy

▰ Measurement of weight, length, blood pressure

▰ Control systemic and secondary infection: worm, ear,
dental, lung infection(TB,Mantoux)  eradicate first
▰ Dietary
▻ Low protein  protein malnutrition
▻ High protein  not recommended
▻ Recommendation: normal protein intake 1.5-2
gr/kg/day and low salt diet 1-2 gr/day
Primary Therapy
Furosemide 1-3 mg/kg/day
+ spironolakton 2-4
mg/kg/day
No Response
No weight loss or no diuresis in
Diuretic 48 hours
No Response

Add Hidrochlortiazid 1-2

mg/kg/day
No Response
▰ Edema
Increase furosemide dose 2
▰ Evaluation of times higher (max 4-6
electrolyte serum mg/kg/day)
No Response
after 1-2 weeks Furosemide bolus IV 1-3
mg/kg/dose, in 0,1-1
diuretic use mg/kg/ hour
No Response

Albumin 20% 1g/kg IV, then

furosemide IV 1-2 mg/kg
Primary Therapy
Immunization

▰ Children who receive corticosteroid therapy > 2

mg/kg/day or >20 mg/day for 2 weeks, are
immunocompromised
▰ Stop steroid < 6 weeks → inactivated vaccine (IPV)
▰ Stop steroid > 6 weeks → attenuated vaccine
(MMR, varicella,OPV)
Corticosteroid
Corticosteroid
 Special
Condition
 For
Complication
 PROGNOSIS

▰ Based on histology type

▰ There are risk to be end stage renal
disease in 20 years:
▻ Focal Segmental Glomerulosclerosis
58,6%
▻ Diffuse Mesangial Proliferation 50%
▻ Minimal Change Disease 4,9%
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CONCLUSION
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 CONCLUSION

▰ Nephrotic syndrome (NS) is a kidney disease characterized by

proteinuria, hypoalbuminemia, edema, and hypercholesterolemia due
to renal glomerular disorders.
▰ Nephrotic syndrome cases 15 times higher in children and 90% of
nephrotic syndrome in children is idiopathic.
▰ The most common form of idiopathic nephrotic syndrome is focal
segmental glomerulosclerosis
▰ There are glomerular disturbance occurs in NS, the podocyte posses
widening, podocyte cells decreases, and changes in the integrity of the
slit diaphgram.
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 CONCLUSION

▰ The diagnostic approach is through clinical manifestations and

investigations where massive proteinuria (> 40 mg / m2 BSA / hour or
protein / creatinine ratio in urine is> 2), hypoalbuminemia <2.5 g / dL,
edema, hypercholesterolemia> 200 mg / dL, and hyperlipidemia. As a
confrmation, kidney biopsy can be done.
▰ Treatment for nephrotic syndrome is diuretic, immunization, and
corticosteroid, also supportive therapy like dietary restrictions and
management of complications
▰ The prognosis of patients with nephrotic syndrome depends on the
type of histopathology 35
Reference
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Paediatr Indones. 2016 Aug 31;56(4):238–41.
▰ 2. Gipson DS, Troost JP, Lafayette RA, Hladunewich MA, Trachtman H, Gadegbeku CA, et al. Complete
Remission in the Nephrotic Syndrome Study Network. Clin J Am Soc Nephrol CJASN. 2016 Jan 7;11(1):81–9.
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http://www.idai.or.id/professional-resources/guideline-consensus/tata-laksana-sindrom-nefrotik-
idiopatik-pada-anak
Reference
▰ 6. Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory
characteristics at time of diagnosis. A report of the International Study of Kidney Disease in Children.
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▰ 7. Kerlin BA, Ayoob R, Smoyer WE. Epidemiology and pathophysiology of nephrotic syndrome-associated
thromboembolic disease. Clin J Am Soc Nephrol CJASN. 2012 Mar;7(3):513–20.
▰ 8. Park SJ, Shin JI. Complications of nephrotic syndrome. Korean J Pediatr. 2011 Aug;54(8):322–8.
▰ 9. Hahn D, Hodson EM, Willis NS, Craig JC. Corticosteroid therapy for nephrotic syndrome in children.
Cochrane Database Syst Rev [Internet]. 2015 [cited 2019 Nov 12];(3). Available from:
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD001533.pub5/full
▰ 10. pubmeddev, DS SC and G. Treatment of FSGS in Children. - PubMed - NCBI [Internet]. [cited 2019 Nov 3].
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THANKS!
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