Nama: Syarifah Hidayah Fatriah

Pembimbing: dr. Elmi Ridar Sp.A

 Definition
Nephrotic syndrome(NS)
very high levels of protein in the urine, a condition
called proteinuria
low levels of protein in the blood hipoalbuminemia
swelling, especially around the eyes, feet, and hands
Oedema
high level of cholesterol in the blood
hypercholesterolemia
Epidemiology
In the US, its annual incidence among children is
reported to be 27 cases per 100,000.
Nephrotic syndrome may occur in 1 in 10,000 or 2-7 in
1.000.000 births
In children, it is diagnosed in more boys than girls,
usually between 2 and 3 years of age.
Ratio 2:1
Chances of recurrence or relapse of NS is as high as 50-
75% in a Nephrotic child.
Pathophysiology
Proteinuria occurs due to increased glomerular
permeability of proteins resulting from the loss of
fixed negative charges and inability of the proximal
tubules to reabsorb all of the filtered proteins.
Mean glomerular pore size or density may be altered
due to lack of electrostatic interaction between
glomerular capillaries and polyionic plasma proteins,
such as albumin
Edema occurs due to a decrease in intravascular oncotic
pressure secondary to urinary protein losses and the
inability to increase synthesis to compensate for such
losses, leading to reduced plasma albumin levels.
Reduced plasma albumin leads to intravascular
hypovolemia, increased aldosterone, antidiuretic
hormone secretion, and subsequent renal salt and
water retention.
 Hyperlipidemia appears due to decreased oncotic
pressure, accompanied by a reduction in fat
degradation activity due to loss of a lipase-glycoprotein
as a stimulant.
Sign
Oedema of dependent parts or generalised oedema are
the main clinical findings.
Facial oedema may be found in children.
Occasionally, severely hypoalbuminaemic cases may
have pleural effusions or ascites.
Urinalysis will reveal gross proteinuria.
Hypertension and haematuria are not usually found
but may affect a minority of cases.
Diagnosis
Diagnostic criteria for nephrotic syndrome:
1. Proteinuria greater than 3-3.5 g/24 hour or spot urine
protein
2. Serum albumin <2,5 mg/l
3. Clinical evidence of peripheral oedema
4. Severe hyperlipidaemia (total cholesterol often >10
mmol/l) is often present
Laboratory tests
The following are baseline, essential investigations:
24 hour bedside urinary total protein estimation.
Urine sample shows proteinuria (>3.5 g per 1.73 m2 per
24 hours).
Comprehensive metabolic panel (CMP) shows
hypoalbuminemia: albumin level 2.5 g/dL
(normal=3.5-5 g/dL).
High levels of cholesterol (hypercholesterolemia),
specifically elevated LDL, usually with concomitantly
elevated VLDL is typical.
Electrolytes, urea and creatinine (EUCs): to evaluate
renal function.
Further investigations are indicated if the cause is not
clear:
Biopsy of kidney (in case of adult patients only).
Auto-immune markers (ANA, ASOT, C3,
cryoglobulins, serum electrophoresis).
Ultrasound of the whole abdomen.
Treatment
Standard ISKDC regime for first episode: prednisolone
-60 mg/m2/day in 3 divided doses for 4 weeks followed
by 40 mg/m2/day in a single dose on every alternate
day for 4 weeks.

Relapses by prednisolone 2 mg/kg/day till urine

becomes negative for protein. Then, 1.5 mg/kg/day for
4 weeks.
 Frequent relapses treated by: cyclophosphamide or
nitrogen mustard or ciclosporin or levamisole.
Achieving better blood glucose level control if the
patient is diabetic.
Blood pressure control. ACE inhibitors are the drug of
choice. Independent of their blood pressure lowering
effect, they have been shown to decrease protein loss
CASE REPORT
Alloanamnesis
Patient An, male, 4 years 8 month , came to AA
hospital on July 2nd 2011

Chief complain : swelling whole body since one week

ago
Present illness history
One weeks ago patients present with swelling around
eyes in the morning in the early stage, which
subsequently spreads to legs, back, abdomen and
whole body.
Complaints swelling is not accompanied by complaints
of shortness of breath during sleep and the patient can
still sleep with a pillow, the patient never complained
of waking up as urination at night. The patient had
never had jaundice.
 Past illness history
- No history of food allergies or medications.
- Patients had renal impairment at diagnosis by a
pediatrician in RSUD AA (date 8/11/2010)
Family history
No family members of patients who had similar
complaints with the patient.
 Pregnancy History
- Pregnancy single. During pregnancy, the mother
during their pregnancy to the midwife 3 times,
irregular, injections of TT (+), ultrasound (-). No
drinking herbs, drank (-), smoking (-).
- Children born spontaneously assisted by midwives,
started to cry, pink skin color, cyanosis (-), patent
anus, 3300 grams birth weight, birth body length 49
cm.
Food and Drink History
-Breast milk is given from birth through age 19
months.
- Porridge 3-9 months of age.
- Porridge rice began to be given 9-12 months of age
- Rice be started at the age of 1 year
Immunization History
BCG(+)
Hepatitis(+)

Growth History
Accordance with age.
Physical examination
General condition
consciousness : composmentis
Vital sign :
BP : 110/80 mmHg
P : 70 x/minutes
RR : 26 x/minutes
T : 37,5 C
Nutrition status
Stature = 92 cm
Weight = 19 kg
Head Circumference = 46cm
Mid uper hand circumference= 18,5cm

HEAD

Hair :black, not easy to pull

Eyes : conjungtiva anemi (-/-), sclera ikteric (-), pupil
isochor, light refleks (+/+)
Ears : normal
Nose : normal
Mouth: lip wet, mukosa wet.
Neck: enlargement limfonodus (-)
 Thorax
Lung:
- Inspection : symmetrical, rib retraction (-)
- Palpation : fremitus R=L
- Percussion : sonor
- Auscultation : vesicular, ronkhi (-/-), wh (-/-)
Heart
- Inspection : ictus cordis is not visible
- Palpation : IC palpable in ICS V LMCS
- Percussion : L: ICS V LMCS, R: Linea
parasternal dextra
- Auscultation : regular heart rhythm,heart noise(-)
 Abdomen
- Inspection : convex, venektasi (-)
- Palpation : Liver and spleen not palpable,
abdominal circumference 54 cm.
- Percussion : timpany
- Auscultation : Bowel sound (+) N
Genitals
- Oedema Scrotum +
Extremity
- Akral warm,
- RCT <2
- edema (+)
Laboratory Examination
Blood
- Hb : 12,5 g/dl ( 10,8-15,6 g/dL)
- Ht : 38,2 % ( 33-45%)
- Leukocytes: 15.600 / UL (4500-14500/UL )
- Platelets: 310.000 /UL ( 150.000-450.000 /UL)
Urine
Protein : +3
Urobilinogen : normal
Bilirubin :-
 BJ : 1,025
Color : kuning keruh
Keton :-
Ureum : 80 mg/dl
Creatinin : 1,3 mg/dl

stool routine
Macroscopic: yellow, soft, mucous pus (-), blood (-)
Microscopic: cyst (-), eggs (-)
IMPORTANT THINGS FROM
ANAMNESIS
Patients present with swelling around eyes in the
morning in the early stage, which subsequently
spreads to legs, back, abdomen and whole body.
Patients had renal impairment at diagnosis by a
pediatrician in RSUD AA (date 8/11/2010)
IMPORTANT THINGS OF EXAMINATION SUPPORT
Genitalia: Edema scrotum (+)
Ekstremitas: pitting edem (+/+)
Albumin : 2,6 g/dl
Protein urine : +3

Diagnosis
Sindroma nefrotik relaps
Therapy
Siklopospamid 2x15gr
Methilprednisolon 1x4tab
Captopril 3x6,25gr
Losartan 1x15gr
 Follow Up

03/07/2011 S: bengkak seluruh tubuh Siklopospamid 2x15gr

O: kesadaran komposmentis, Methilprednisolon
TD = 110/80mmHg 1x4tab
BB: 19kg Captopril 3x6,25gr
Edema ekstremitas +/+ Losartan 1x15gr
Edema scrotum +
Linkar Pinggang(LP)= 54 cm
posisi berbaring
Lab urin: protein +3
A: Sindroma nefrotik Relaps
04/07/2011 S: bengkak seluruh tubuh Siklopospamid 2x15gr
O: kesadaran komposmentis, Methilprednisolon
TD = 110/70mmHg 1x4tab
BB: 19kg Captopril 3x6,25gr
Edema ekstremitas +/+ Losartan 1x15gr
Edema scrotum +
Linkar Pinggang(LP)= 55 cm
posisi berbaring
Lab urin: protein +3
A: Sindroma nefrotik Relaps

05/07/2011 S: bengkak seluruh tubuh Siklopospamid 2x15gr

O: kesadaran komposmentis, Methilprednisolon
TD = 100/70mmHg 1x4tab
BB: 19kg Captopril 3x6,25gr
Edema ekstremitas +/+ Losartan 1x15gr
Edema scrotum +
Linkar Pinggang(LP)= 53 cm
posisi berbaring
Lab urin: protein +1
A: Sindroma nefrotik Relaps
06/07/2011 S: bengkak seluruh tubuh Siklopospamid 2x15gr
O: kesadaran komposmentis, Methilprednisolon
TD = 90/70mmHg 1x4tab
BB: 18kg Captopril 3x6,25gr
Edema ekstremitas +/+ Losartan 1x15gr
Linkar Pinggang(LP)= 53 cm
posisi berbaring
Lab urin: protein +2
A: Sindroma nefrotik Relaps

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