What he may had?

2/6/17
Childhood Nephr
otic Syndrome
dr. Kristia Hermawan, MSc, Sp.A

Division of Pediatric Nephrology

Department of Child Health
Faculty of Medicine, Universitas Gadjah Mada
Sardjito Hospital Yogyakarta
2015
Definition

Clinical abnormality characterised by

Significant proteinuria
Hypo-albuminaemia (plasma albumin of les
s than 2,5 g/dl)
Generalized edema
Typically accompanied by dis-lipidaemia (el
evated plasma cholesterol and triglycerides;
total cholesterol > 200 mg/dL)
Classification

ETIOLOGY
Congenital
Primary/ idiopathic
Secondary

PATHOLOGY
minimal change disease 80%
Focal segmental glomerulosclerosis 7-8%
Difuse mesangial proliferative 2-5%
Glomerulonefritis membranoproliferative 4-6%
Membranous nephropathy 1,5%

Churg et al.Lancet 1970;i:1353-9

Heparan sulfate a proteoglycan (Ext1gene expression) produce
by podocytes is a major contributor of glomerular anionic charge.

molecluar
weight > 40 KD

filtration
pressure >>>
Pathogenesis
Proteinuria
due to
defective
glomerular
filtration
barrier
which
normally
impermeabl
e to proteins

Podocyte
lose their
complex
Pathophysiology
- Transferin Ig G
PROTEINURIA
- Glob. Tiroksin Ig E
- Glob. Vit D Ig A
- Coagulation factors Ig M
VII, IX, XII Fibrinogen
HIPOALBUMINEMIA
lipoprotein Hiperlipidemia
OSMOTIC PRESSURE
Lipiduria
EDEMA Fluid extravasation

HIPOVOLEMIA
Peripheral
Renal perfussion hypoperfussion

Plasma Ureum death

Renin + Vol .Packed cell
K
Viscocity
Adolsteron
Hiponatremia

Na retention Thrombosis
H20
Clinical manifestation

Edema: palpebral edema, pretibial, scrotal,

ascites, pleural efussion
Proteinuria: cloudy urine
Deminished cardiac output: tachycardi, olig
uria

Hipertension ?
Hematuria ?
Etiology
Pitting edema
Role of RAA System in NS
Routine laboratory investigation
Urinalysis
Quantitative protein test 24 h urine collec
tion

Serum albumin level

Serum cholesterol level

Ureum, creatinin
CBC
C3 complement
Differential diagnosis

Other renal cause of edema:

Acute glomerulonephritis (hypertension, oliguria, oedema)
Renal failure(abnormal plasma creatinine)

Non renal cause of edema:

Protein losing enteropathy
severe cardiac failure
chronic liver disease
angioedema
kwashiorkor
Management
Hospitalization: first episode, relaps with ed
ema, any life threathening complication
regular observations and close monitoring f
or complications such as hypovolemia, thr
ombosis or septicemia.
Strict fluid input and output should be mea
sured along with daily weights.
Children with significant edema require a fl
uid restriction (1l/m2/24h as oral fluids) an
d a salt-restricted diet
Corticosteroid
Preparation for long term therapy with ster
oid evaluate
Body weight n height
Blood pressure
Focal infection
Sign of systemic disease
Tuberculin test

5 mg Prednison 4 mg Metilprednisolon
Treatment protocol

Steroid medication (ISKDC)

Prednisone
60 mg/m2/day 4 weeks
40 mg/m2/day 4 weeks AD

1st 4 weeks 2nd 4 weeks

Predn 2/3 Initial dose

Initial
 Criteria for treatment outco
me
Remission: proteinuria negative/ trac
e for 3 consecutive days

Relapse: proteinuria >= +2 for 3 cons

ecutive days after remission
 Adverse effect of steroid
Cushingoid, Moon Face, Acne, Striae
Hipokalemia
Growth supression. Adrenal function supression
Hematology: Leukocytosis
Hipertension
Demineralization, Osteoporosis
Peptic ulcer
Immunocompromized: fungal infection, TB activ
ation
 Supportive management
Antihypertensive drugs:
Not only for hypertension proteinuria treatment
ACE inhibitor, ARB

Hyperlipidemia:
Steroid sensitive: transient diet
Steroid resistant: diet, goal: normal weight for height, medicatio
n: statin if serum cholesterol level > 350 mg/dL

Hipocalcemia:
Ca suplementation 250 500 mg/day
Vitamin D
Diuretic

Needed during flare

Decreasing edema compression symp
toms
Loop diuretik: furosemide 1- 3 mg/kg/day, c
an be combined with spironolakton 2- 4mg/
kg/hari
Refractory edema hypovolemia/ severe h
ypoalbuminemia (<1g/dL) albumin transf
ussion followed by diuretic administration
alternative: human plasma 20 cc/kg/day
Dietary management

PROTEIN
High protein diet increase glomerular load for ex
creting protein metabolite further tubular dama
ge
Low protein diet endogen breakdown malnutr
ition
Reccomendation RDA 1,5 2 g/kg/d
SALT
Restriction, tolerable flavour [no added salt] max 1-
2 g/d
Life threathening complicatio
n
Hypovolemic shock
fluid resuscitation
Albumin transfusion
g Alb = 0.8 x [Alb target (g/dL) Alb actual (g/dL)] x BW (kg)
Max dose 1g/kg daily
Fresh Plasma transfusion: 10cc/kg

Spontaneous bacterial peritonitis

Septicemia
2/6/17
 Criteria for treatment outco
me
Frequent relapse: relapse 2x or more within 6 month a
fter the first treatment response or at least 4x in a year

Steroid sensitive: remission achieved within first 4 we

eks of treatment

Steroid resistant: no remission achieved within first 4

weeks of treatment

Steroid dependent: relapse for at least 2x while altern

ating dose were given or relpase within 14 days after ste
roid discontinuation
Prognosis

Minimal change NS

100 %
Response Non response
93 % 7%
Minimal change disease response to steroid (ISKDC)

Non Relaps Relaps Respons Non

relaps (rare) (frequent) 5% Respon
36 % 36 % 3% 2%

Non response
Kidney Int. 13-43, 1974;
5% Damanik & Yoshikawa,
1997
 Thank You

dr.kryzt@gmail.com

Division of Pediatric Nephrology

Department of Child Health
Faculty of Medicine, Universitas Gadjah Mada
Sardjito Hospital Yogyakarta
2015
QUESTIONS ??