Nephrotic Syndrome

Heru Pranata (090100073) Rio Nurdiansyah Batubara (090100173) Supervisor Sp.A(K) : dr. Yazid Dimyati,

Definition
Proteinuria (>40mg/m2/h) Hypoalbumine mia

Edema

Hypercholesterole mia

Aethiology
A. Genetic disorders Nephrotic-syndrome B. Secondary causes

typical Proteinuira with or without nephrotic syndrome Multisystem syndromes with or without nephrotic syndrome Metabolic disorders with or without nephrotic syndrome C. Idiopathic nephrotic syndrome

Infections Drugs Immunological or allergic disorders Associated with malignant disease Glomerular hyperfiltration Congenital nephrotic syndrome

 Also known as lipoid

nephrosis 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.

 A viral- or toxin-

mediated damage or intrarenal hemodynamic changes such as hyperperfusion and high intraglomerular capillary pressure
Injury to podocytes 2. shrinkage/collapse of glomerular capillaries 3. scarring
1.

Pathophysiology
Hypoalb uminemi a

Proteinuria

Oede m

Hypercho lesterole mia

Pathophysiology
Glomerular filtration process in interrupted

Commonly a defect in the podocytes and/or

glomerular basement membrane. Recent experiments have implicated T-Cells in the damage to podocytes leading to 2 common types of nephrotic syndrome (minimal change disease and focal-segmental glomerulosclerosis) Exact pathology varies depending on the specific type of nephrotic syndrome.

Pathophisiology
HYPERCHOLESTROLEMIA. Response to

Hypoalbuminemia reflex to liver -- synthesis of generalize protein ( including lipoprotein ) and lipid in the liver ,the lipoprotein high molecular weight no loss in urine hyperCHOLESTROLEMIA

Pathophisiology
*Reduction plasma colloid oncotic

pressure secondary to hypoalbuminemia OEdema and hypovolemia

*Intravascular volume antidiuretic hormone

(ADH ) and aldosterone(ALD) water and sodium retention OEdema

*Intravascular volume glomerular filtration

rate (GFR) water and sodium retention OEdema

CLINICAL MANIFESTATIONS
Proteinuria

Hypoalbuminemia
Generalized Oedema Hyperlipidemia

Diagnose
Anamnesis

Laboratory Evaluation

- Urine Test -Albumin Level -Renal Function test Renal Biopsy

Indication for Renal Biopsy

Differential Diagnosis
Nephritic Syndrome

Non Renal Disease

-CHF -Nutrition Imbalance -Hepatic oedema -Acute Glomerulonephritis

Remision

Urine albumin nil or trace (or proteinuria < 4 mg/m2/h) for 3 consecutive early morning specimens Urine albumin 3+ or 4+ ( or proteinuria > 40mg/m2/h) for 3 consecutive early morning specimens, having been in remission peviously Two or more relapses in initial six months or more than three relapses in any twelve months Two consecutive relapses when or alternate day steroids or within 14 days of its discontinuation Absence of remission despite therapy with daily prednisone at dose of 2 mg/kg per day for 4 weeks relapses occurred less than 2 times in the first 6 months after initial response or less than 4 times per year of observation remission is achieved in 4 weeks or less after full-dose steroid treatment

Relapse

Frequents Relapses

Steroid Dependence

Steroid Resistance

Infrequent s Resistance

Steroid Sensitive

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

continous
Albumin

Penicilin Prophylaxis
Salt / Fluid Restriction Vaccination

Treatment
Relapsing Infrequent
Prednisone or prednisolone -

Relapsing Frequent
Low Dose Alternate

start at 60mg/ m2/day (max 80mg) until in remission

Then give alternate day

Day Prednisolone
Levamisole
Cyclophosphamide

prednisone or prednisolone at 40mg/ m2/day (max 60mg) for

total of 28 days, then stop

Cyclosporin

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

Supportive Care
Diet
Adequate in protein

Edema
Patients with persisten

(1,5-2g/kg) reduction of salt intake (1-2 g per day) is advised for those with persistent edema

edema and weight gain of 710 % are treated with oral furosemide (1-3 mg/kg, daily).
potassium sparing diuretics,

e.g. spironolactone (2-4

mg/kg daily)

 Hypovolaemia
Despite odema may be intra-vascularly depleted

Infection
Loss of complement components

Thrombosis
Loss of proteins and exacerbated by hypovolaemia

Prognose
MINIMAL CHANGE PATHOLOGY : GOOD PROGNOSIS

FOCAL SEGMENTAL

GLOMERULARSCLEROSIS : PROGNOSIS IS GRAVE END STAGE IS LIKELY TO HAPPEN

CASE REPORT
Name

Age
Sex Date of Admission

: JS : 9 years : Male : August, 21 th 2013

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 not found, 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 was under 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

 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

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.

 Neck : Lymph node enlargement (-), nuchal rigidity (-)

Thorax

: Symmetrical fusiformis, retraction (-).

HR: 88 bpm, reguler, murmur (-). RR: 26 bpm, regular, crackles (-/-)

Abdomen
Extremities

: Soepel, peristaltic (+) normal. Liver and spleen not palpable

: 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

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

Laboratory Result (August 21th 2013)

Complete Blood Result Normal Range 11.3 14.1

Count
Hemoglobin (HGB) g% 10.50

Eritrosit (RBC)
Leukosit (WBC) Hematokrit Trombosit (PLT) MCV MCH MCHC RDW

106/ mm3
103/ mm3 % 103/ mm3 fL Pg g% %

3.39
6640 29.00 633000 85,50 31.00 36.20 13.70

4.40 4.48
4.5- 13.5 37 41 217 497 81 95 25 29 29 31 11.6 14.8

Diftel
Neutrofil Limfosit
Monosit Eosinofil Basofil Neutrofil Absolut Limfosit Absolut Monosit Absolut Eosinofil Absolut Basofil Absolut

Result
% %
% % % 103/L 103/L 103/L 103/L 103/L

Normal Range
37 80 20 40
28 16 01 2.4 - 7.3 1.7 - 5.1 0.2 - 0.6 0.10 - 0.30 0 - 0.1

62,70 20,80
14,60 0.50 1200 4.17 1,38 0.97 0.03 0.09

Electrolyte

Electrolyte
Natrium( Na)
Kalium (K) Cloride (Cl) Hepar Albumin Kidney Ureum mg/dL g/dl

Result
mEq/L
mEq/L mEq/L

Normal Range
135-155
3.6-5.5 96-106

130
5.1 107

1,6

3,8-5,4

91.10

<50

Creatinine
Urid Acid

mg/dL
mg/dl

2.57
107

0.24-0.41
96-106

Urinalisis
Colour

Result
Kuning keruh
-

Glucose Bilirubin
Keton Berat Jenis PH Protein Urobilinogen Nitrit Blood

1015 5 +++
-

Sedimen Eritrocyte Leukocyte

Result 0-2 25-30

Ephitel
Casts Crystal

1-2
Granular -

August, 21th 2013 (First day) 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 Extremities Soepel,Peristaltic (+) Normal. Liver and spleen not palpable

Pulse 88 bpm, regular, adequate pressure and volume, warm acral, CRT<

3. TD: 100/70mmhg, pitting oedem(+/+).

A: Steroid Resistance Nephrotic Syndrome P: Management: - Three Way - Normal diet low salt with 60 gr protein and 1900 calories -Captopril 2 x 25 mg - Losartan 1x 0,5 tab

- Prednison 1x7 mg AD
-Inj. Cefriaxone 1 gr/12 j/IV - Inj. Furosemide 30 mg/ 8 jam
-Aldacton 3x25 mg

August, 22th 2013 (Second day) S:Swelling on the face and foot O: Sens: Compos Mentis , Temp: 37oC, Body weight: 29 kg Head Eye : Light reflexes(+/+), isochoric pupil, pale conjunctiva palpebra inferior (-/-), icteric (-/-) , oedem preorbital(+/+) .Ear : Normal appereance ,Mouth : Sianosis (-), Nose: Normal appereance Thorax Symmetrical fusiformis. Epigastrial retraction (-). HR: 90 bpm,reguler, murmur (-). RR: 28 bpm, regular,crackles (-/-) Abdomen Extremities Soepel,Peristaltic (+) Normal. Liver and spleen not palpable Pulse 90 bpm, regular, adequate pressure and volume,warm acral, CRT< 3. TD: 110/70mmhg, pitting oedem(+/+). A: Steroid Resistance Nephrotic Syndrome P: Management: - Three Way - Normal diet low salt with 60 gr protein and 1900 calories -Captopril 2 x 25 mg - Losartan 1x 0,5 tab - Prednison 1x7 mg AD -Inj. Cefriaxone 1 gr/12 j - Inj. Lasix30 mg/ 8 jam -Aldacton 3x25 mg Planning = serial urinalisis, fluid balance per 6 hours, renal function test, renal biopsy

August, 23th 2013 (Third day) S:Swelling on the face and foot O: Sens: Compos Mentis , Temp: 37oC, Body weight: 29 kg Head Eye : Light reflexes(+/+), isochoric pupil, pale conjunctiva palpebra inferior (-/-), icteric (-/-) , oedem preorbital(+/+) .Ear : Normal appereance ,Mouth : Sianosis (-), Nose: Normal appereance. Thorax Symmetrical fusiformis. Epigastrial retraction (-). HR: 86 bpm,reguler, murmur (-). RR: 24 bpm, regular,crackles (-/-) Soepel,Peristaltic (+) Normal. Liver and spleen not palpable Pulse 90 bpm, regular, adequate pressure and volume,warm acral, CRT< 3. TD: 110/70mmhg, pitting oedem(+/+).

Abdomen Extremities

A: Steroid Resistance Nephrotic Syndrome

P: Management: - Three Way - Normal diet low salt with 60 gr protein and 1900 calories -Captopril 2 x 25 mg

- Losartan 1x 0,5 tab

- Prednison 1x7 mg AD -Inj. Cefriaxone 1 gr/12 j - Inj. Lasix30 mg/ 8 jam -Aldacton 3x25 mg Planning = serial urinalisis, fluid balance per 6 hours, renal function test, renal biopsy

DISCUSSION

This paper reports a case of a 9 years old male diagnosed with Steroid Resistent Nephrotic Syndrome. Nephrotic syndrome is characterized by massive proteinuria (> 40mg/kgbb/day), hypoalbunemia (<2.5 gr/dl), hypercholesterolemia (>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: 3,8 5,4) 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.

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.

SUMMARY
JS, 9 years old male diagnosed with Steroid

Resistent 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

Thank you