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Heru Pranata (090100073)
Rio Nurdiansyah Batubara
(090100173)
Supervisor : dr. Yazid Dimyati,
Sp.A(K)
Nephrotic Syndrome
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Definition
Proteinuria(>40mg/m2/h)
Hypoalbuminemia
Hypercholesterolemia
Edema
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AethiologyA. Genetic disorders Nephrotic-syndrome
typical
Proteinuira with or
without nephroticsyndrome
Multisystem syndromeswith or withoutnephrotic syndrome
Metabolic disorderswith or withoutnephrotic syndrome
Idiopathic nephrotic
syndrome
B. Secondary causes
Infections
Drugs
Immunological orallergic disorders
Associated withmalignant disease
Glomerularhyperfiltration
C. Congenital nephroticsyndrome
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Also known as lipoidnephrosis or nil
disease
It refers to a
histopathologic lesion
in the glomerulus
Disorder of T cells,
which release a
cytokine that injures
the glomerular
epithelial foot
processes.
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A viral- or toxin-mediated damage or
intrarenal
hemodynamic
changes such ashyperperfusion and
high intraglomerular
capillary pressure
1. Injury to podocytes
2. shrinkage/collapse
of glomerular
capillaries
3. scarring
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Pathophysiology
Proteinuria
Hypoalbuminemi
a
Hypercholesterole
mia
Oedem
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Pathophysiology
Glomerular filtration process in interrupted
Commonly a defect in the podocytes and/or
glomerular basement membrane.
Recent experiments have implicated T-Cells inthe damage to podocytes leading to 2 common
types of nephrotic syndrome (minimal change
disease and focal-segmental glomerulosclerosis)
Exact pathology varies depending on the specifictype of nephrotic syndrome.
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Pathophisiology
HYPERCHOLESTROLEMIA.Response toHypoalbuminemia reflex to liver-- synthesis
of generalize protein ( including lipoprotein )
and lipid in the liver ,the lipoprotein high
molecular weight no loss in urine hyperCHOLESTROLEMIA
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Pathophisiology
*Reduction plasma colloid oncoticpressure secondary to hypoalbuminemiaOEdema and hypovolemia
*Intravascular volume antidiuretic hormone(ADH ) and aldosterone(ALD) water andsodium retentionOEdema
*Intravascular volume glomerular filtrationrate
(GFR) water and sodium retentionOEdema
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CLINICAL MANIFESTATIONS
Proteinuria
Hypoalbuminemia
Generalized Oedema
Hyperlipidemia
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Diagnose
Anamnesis
Laboratory Evaluation
- Urine Test
-Albumin Level
-Renal Function test
Renal Biopsy
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Indication for Renal Biopsy
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Differential Diagnosis
Nephritic Syndrome
Non Renal Disease
-CHF
-Nutrition Imbalance-Hepatic oedema
-Acute Glomerulonephritis
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Remision Urine albumin nil or trace (or proteinuria
< 4 mg/m2/h) for 3 consecutive early
morning specimens
Relapse Urine albumin 3+ or 4+ ( or proteinuria >40mg/m2/h) for 3 consecutive early
morning specimens, having been in
remission peviously
Frequents Relapses Two or more relapses in initial six months
or more than three relapses in any twelve
months
Steroid Dependence Two consecutive relapses when or
alternate day steroids or within 14 days of
its discontinuation
Steroid Resistance Absence of remission despite therapy with
daily prednisone at dose of 2 mg/kg per
day for 4 weeks
Infrequent s Resistance relapses occurred less than 2 times in the
first 6 months after initial response or less
than 4 times per year of observation
Steroid Sensitive remission is achieved in 4 weeks or less
after full-dose steroid treatment
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Treatment Initial
60 mg/m2/day for 4 weeks (maximum 80 mg)
40 mg/m2/on alternate days for 4 weeks (maximum
60mg)
Reduce dose by 5-10mg/m2 each week for another 4
weeks then stop
If If prednisolone causes gastric irritation, start
ranitidine 2mg/kg bid for the duration of steroid
treatment
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continous
Albumin
Penicilin Prophylaxis
Salt / Fluid Restriction
Vaccination
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Treatment
Relapsing Infrequent Relapsing Frequent
Prednisone or prednisolone -
start at 60mg/ m2/day (max
80mg) until in remission
Then give alternate day
prednisone or prednisolone at
40mg/ m2/day (max 60mg) fortotal of 28 days, then stop
Low Dose Alternate
Day Prednisolone
Levamisole
Cyclophosphamide
Cyclosporin
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Nephrotic Syndrome Resistant
Steroid
Prednisone 40 mg/m2LPB/day tapering off prednisone
at a dose of 1 mg / kg / day for 1 month, followed by
0.5 mg / kg / day for 1 month (long tapering off 2
months)
cyclophosphamide 2-3 mg / kg / day single dose for 3-
6 months
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Supportive Care
Diet Edema
Adequate in protein
(1,5-2g/kg)
reduction of salt intake
(1-2 g per day) is
advised for those with
persistent edema
Patients with persisten
edema and weight gain of 7-
10 % are treated with oral
furosemide (1-3 mg/kg,
daily).
potassium sparing diuretics,e.g. spironolactone (2-4
mg/kg daily)
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Hypovolaemia
Despite odema may be intra-vascularly depleted
Infection Loss of complement components
Thrombosis
Loss of proteins and exacerbated by hypovolaemia
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Prognose
MINIMAL CHANGE PATHOLOGY :- GOOD PROGNOSIS
FOCAL SEGMENTAL
GLOMERULARSCLEROSIS :-
PROGNOSIS IS GRAVE
END STAGE IS LIKELY TO HAPPEN
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CASE REPORT
Name : JS
Age : 9 years
Sex : Male
Date of Admission : August, 21th
2013
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Main Complaint : Swelling on the eyelids
History : Swelling on the eyelids had been complained by the patient in
2 days before admitted to the hospital. Swelling on the eyelids became
worst in 2 days. Patient also complain that his urine output became
lesser in 2 days. History of waists pain was not found. Fever was notfound, Unclear urine was complained by patient since yesterday.
Painful urinating was not suffered by patient.
History of body swelling had been suffered by patient since 2 months
ago. Patient had been treated before in Sidikalang Hospital for 3 days
and was diagnosed by nephrotic syndrome by pediatrician. Patient wasunder treatment of steroid for 4 weeks, but patient didnt get remission.
Then, patient was diagnosed nephrotic syndrome resistant steroid and
treated by cyclophosphamide and prednisone. After that patient was
consuled to Adam Malik General Hospital
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History of Immunization: Not Clear
History of birth : Not Clear
History of immunization : Not Clear
History of Growth and Development : Not clear
History of Illness : Not clear
History of Medication : Not clear
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Physical Examination
BW: 30 kg ; BL: 127 cm cm
Presens status
Sensorium : Compos Mentis, Blood Pressure : 100/70 mmHg,
Body temperature: 36,8oC, Pulse: 100 bpm, Respiratory Rate:
20 bpm.
Localized status
Head : Eyes : Light reflexes(+/+), isochoric pupil, pale
conjunctiva palpebra inferior (-/-), preorbital oedem (+/+),
icteric (-/-) , Ear : Normal appereance ,Mouth : Sianosis (-),
Nose: Normal appereance.
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Neck : Lymph node enlargement (-), nuchal rigidity (-)
Thorax : Symmetrical fusiformis, retraction (-).
HR: 88 bpm, reguler, murmur (-).
RR: 26 bpm, regular, crackles (-/-)
Abdomen : Soepel, peristaltic (+) normal. Liver and spleen not palpable
Extremities : Pulse 88 bpm, regular, adequate pressure and volume warm
acral, CRT < 3, TD: 100/70 mmhg, pitting oedem(+/+).
Differential Diagnosis
Steroid Resistance Nephrotic Syndrome
Relaps Nephrotic Syndrome
Nutrition Imbalance
Working Diagnosis
Steroid Resistance Nephrotic Syndrome
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Treatment
Threeway
Normal diet low salt with 60gr protein and 1900Kcalories
Captopril 2x25 mg
Losartan 1x0,5 tab
Prednison 1x7 mg
Inj. Ceftriaxone 1 gr/12h/IV Inj. Furosemide 30 mg/8h
Aldacton 3x25 mg
Planning
Serial Urinalisis Fluid Balance per 6 hours
Renal Function Test
Renal Biopsy
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Laboratory Result (August 21th 2013)
Complete Blood
Count
Result Normal Range
Hemoglobin (HGB) g% 10.50 11.314.1
Eritrosit (RBC) 106/ mm3 3.39 4.404.48
Leukosit (WBC) 103
/ mm3
6640 4.5- 13.5
Hematokrit % 29.00 3741
Trombosit (PLT) 103/ mm3 633000 217497
MCV fL 85,50 8195
MCH Pg 31.00 2529
MCHC g% 36.20 2931
RDW % 13.70 11.614.8
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Diftel Result Normal Range
Neutrofil % 62,70 3780
Limfosit % 20,80 2040
Monosit % 14,60 28
Eosinofil % 0.50 16
Basofil % 1200 01
Neutrofil Absolut 103/L 4.17 2.4 - 7.3
Limfosit Absolut 103
/L 1,38 1.7 - 5.1
Monosit Absolut 103/L 0.97 0.2 - 0.6
Eosinofil Absolut 103/L 0.03 0.10 - 0.30
Basofil Absolut 103/L 0.09 0 - 0.1
Electrolyte
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Electrolyte
Electrolyte Result Normal Range
Natrium( Na) mEq/L 130 135-155
Kalium (K) mEq/L 5.1 3.6-5.5
Cloride (Cl) mEq/L 107 96-106
Hepar
Albumin g/dl 1,6 3,8-5,4
Kidney
Ureum mg/dL 91.10
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Urinalisis Result
Colour Kuning keruh
Glucose -
Bilirubin -
Keton -
Berat Jenis 1015
PH 5
Protein +++
Urobilinogen -
Nitrit -
Blood -
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Sedimen Result
Eritrocyte 0-2
Leukocyte 25-30
Ephitel 1-2
Casts Granular
Crystal -
August, 21th 2013 (First day)
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g , ( y)
S:Swelling on the face and foot
O: Sens: Compos Mentis , Temp: 37oC, Body weight: 30kg
Head Eye : Light reflexes(+/+), isochoric pupil, pale conjunctiva palpebra inferior(-/-).Neck : Lymph node enlargement (-), nuchal rigidity (-)Ear/Mouth/Nose: normal
Thorax Symmetrical fusiformis. Epigastrial retraction (-). HR: 88 bpm,reguler, murmur (-). RR: 28 bpm,regular,crackles (-/-)
Abdomen Soepel,Peristaltic (+) Normal. Liver and spleen not palpableExtremities Pulse 88 bpm, regular, adequate pressure and volume, warm acral, CRT 40mg/kgbb/day),
hypoalbunemia (200mg/dl) and also proof of oedema. In
this patient all of the characteristic symptomps was found. Proteinuria (+++) which mean that
the protein loss is 300mg/dL, hypoalbuminemia 1,6mg/dL (N: 3,85,4) as the consequences of
protein loss from urine. Oedem was found in preorbital and pretibial area.
Hypercholesterolemia were unknown because it was not checked.
According to anamnesis, the patient has been treated about 2 months ago and diagnosed by
nephrotic syndrome. The patient had full dose steroid for 4 weeks, but the patient still not have
remission. For now, the patient came back with oedem preorbital and still not have remission.
After that, the patient diagnosed by Steroid Resistant Nephrotic Syndrome because of the
patient was not have remission after steroid therapy.
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DISCUSSION The patient was given diuretic (furosemide and spironolactone) to treat
fluid retention that cause oedem on this patient. Then captopril and
losartan has given to this patient to avoid renal hypertension and
cardiac remodeling. Prednisone still given to wait to cyclophosphamide
ready to be administrated to this patient for steroid resistant nephrotic
syndrome therapy to prevent genetic mutation as cytostatic and
imunosupressan. Ceftriaxone injection was given as profilaxis to
nosocomial infection.
A low salt diet and high protein has been given to the patient to prevent
further fluid retention and oedem.
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SUMMARY
JS, 9 years old male diagnosed with SteroidResistent Nephrotic Syndrome and treated
byThree way,Diet MB low salt with 60 gr protein
and 1900 calories, Inj. Ceftriaxone 1gr/12 hours,
Inj. Lasix 30 mg/ 8 hours, Aldacton 3 x 25 mg,Captopril 2 x 25 mg, Losartan 1 x 0,5 tab,
Prednison 1x 7 mg AD
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Thank you