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 -60mg/m2/day in 3 divided

doses for 4 weeks followed by 40mg/m2/day
in a single dose on every alternate day for 4
weeks.
Relapses by prednisolone 2mg/kg/day till

urine becomes negative for protein. Then,

1.5mg/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

ImmunizationHistory
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,noteasy topull
Eyes : conjungtiva anemi (-/-), sclera ikteric
isochor, light refleks (+/+)
Ears : normal
Nose : normal
Mouth: lip wet, mukosa wet.
Neck: enlargement limfonodus (-)

(-), pupil

Thorax

Lung:
- Inspection
- Palpation
- Percussion
- Auscultation
Heart
- Inspection
- Palpation
- Percussion
- Auscultation

: symmetrical, rib retraction (-)

: fremitus R=L
: sonor
: vesicular, ronkhi (-/-), wh (-/-)
: ictus cordis is not visible
: IC palpable in ICS V LMCS
: L: ICS V LMCS, R: Linea
parasternal dextra
: regular heart rhythm,heart noise(-)

Abdomen

- Inspection
- Palpation

: convex, venektasi (-)

: Liver and spleen not palpable,
abdominal circumference 54 cm.
: timpany
: Bowel sound (+) N

- Percussion
- Auscultation
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
Color
Keton
Ureum
Creatinin

:
:
:
:
:

1,025
kuning keruh
80 mg/dl
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
03/07/201 S: bengkak seluruh tubuh
O: kesadaran komposmentis,
1
TD = 110/80mmHg
BB: 19kg
Edema ekstremitas +/+
Edema scrotum +
Linkar Pinggang(LP)= 54 cm
posisi berbaring
Lab urin: protein +3
A: Sindroma nefrotik Relaps

Siklopospamid
2x15gr
Methilprednisolon
1x4tab
Captopril 3x6,25gr
Losartan 1x15gr

04/07/201 S: bengkak seluruh tubuh

O: kesadaran komposmentis,
1
TD = 110/70mmHg
BB: 19kg
Edema ekstremitas +/+
Edema scrotum +
Linkar Pinggang(LP)= 55 cm
posisi berbaring
Lab urin: protein +3
A: Sindroma nefrotik Relaps

Siklopospamid
2x15gr
Methilprednisolon
1x4tab
Captopril 3x6,25gr
Losartan 1x15gr

05/07/201 S: bengkak seluruh tubuh

O: kesadaran komposmentis,
1
TD = 100/70mmHg
BB: 19kg
Edema ekstremitas +/+
Edema scrotum +
Linkar Pinggang(LP)= 53 cm
posisi berbaring
Lab urin: protein +1
A: Sindroma nefrotik Relaps

Siklopospamid
2x15gr
Methilprednisolon
1x4tab
Captopril 3x6,25gr
Losartan 1x15gr

06/07/201 S: bengkak seluruh tubuh

O: kesadaran komposmentis,
1
TD = 90/70mmHg
BB: 18kg
Edema ekstremitas +/+
Linkar Pinggang(LP)= 53 cm
posisi berbaring
Lab urin: protein +2
A: Sindroma nefrotik Relaps
Pasien pulang

Siklopospamid
2x15gr
Methilprednisolon
1x4tab
Captopril 3x6,25gr
Losartan 1x15gr

Thank You