2 Sindroma Nefrotik Pesentasi Lapkas Heru Rio

7/29/2019 2 Sindroma Nefrotik Pesentasi Lapkas Heru Rio

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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

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2 Sindroma Nefrotik Pesentasi Lapkas Heru Rio