Chronic Kidney Disease on

Resistant Steroid Nephrotic Syndrome

Angel Chen
Sonia Dinda Paramitha

SUPERVISOR:
dr. Muhammad Nur, Sp.A

PEDIATRIC DEPARTMENT OF
MEDICAL FACULTY OF RIAU UNIVERSITY
2019
Introduction
Indonesia 6/100.000 children/year
Incidence

Boy : Girl = 2 : 1

Polyclinic = 36 children
Arifin Achmad Hospital
Lily room = 25 children

Polyclinic = 28 children
Lily room = 27 children

Classification Steroid Sensitive Nephrotic Syndrome 80%

Steroid Resistant Nephrotic Syndrome 20%

1. Wirya IW. 2002.

2. RSUD Arifin Achmad. 2018.
36- 50% CKD 3. Rahmadi.2012
4. Nourbakhsh Meyer .2017
 Steroid Resistant Nephrotic Syndrome

57,5 % stage I

20 % stage II

CKD 15 % stage III

2,5 % stage IV

1. Prambudi, Muryawarman.2015
5 % stage V
Definition
A syndrome characterized by heavy proteinuria, hypoalbuminemia,
edema and hypercholesterolemia
Remission

Relapse

Rare Relapse

Frequently Relapse
Restrictions in the
nephrotic syndrome
Steroid Dependent

Steroid Resistant

Steroid Sensitive 1. Trihono PP, Alatas H, Tambunan T,

Pardede S. 2012.
 1. Yi ZW, He QN. 2006.
2. Obeidoya H. 2006

Pathophysiology 3.
4.
Niaudet P. 1999
Antignac C. 2005

SRNS

Impaired fungtion
Glucocorticoid or structure of the Glucocorticoid
receptor mutation glomerular toxicity
endotelial capillary

Stop of steroid Suprafisilogis dose

therapy of therapy steroid
 Case Report
Identity
– Name : An. RS
– Sex : Male
– No MR : 100677591
– Age : 10th years 2 months
– Date of admission : 21th March 2019
– Date of examination : 22th March 2019
– Date of discharge : 29th March 2019

Chief Complaint:

Seizure since 1 hour before being hospitalized

 Pediatric Assesment Triangle (PAT)

Tonus: Active
Interactiveness: Interacts Abnormal airway sounds: (-)
well with people Retraction: (-)
Consolability: Stops crying Flaring: (-)
with comforting by parents Respiration rate : 34x/min
Look: Makes eye contact with
clinician
Speech: Speech clearly

Pallor: (+)
Cyanosis: (-)

7
 History of Present Illness
-History of nephrotic syndrome
since 2 years old.
-Edema on eyes, stomach, and
feet, proteinuria +4
-Got medical therapy
(metilprednisolon 2-2-1 full
dose (FD), captopril 2x62,5 mg,
albumin) and remision in 3
months with alternating dose
(AD)
Diagnose : Initial Nephrotic
Syndrome
- Edema on eyes, stomach,
feet, and scrotum, proteinuria
+3
- Edema on eyes, stomach, - GFR : 153 ml/min/1,73 m2
and feet, proteinuria +3 - Got metilprednisolon 2-2-2,
- Got metilprednisolon 2-2-1 losartan, and albumin.
FD, captopril 2x62,5 mg and - Remision in 2 months with
remision again after AD
alternating dose. No
complaints in ±1 year. - Relapse again twice
Diagnose :Nephrotic Diagnose : Nephrotic
syndrome relapse syndrome frequent relapse

2 years old 3 to 4 years old

 History of Present Illness
- After got metilprednisolon FD,
proteinurin +3 in 4 weeks.
- GFR : 124 ml/min/1,72 m2
- Edema on eyes, face,
stomach, and feet, Diagnose : Resistant Streroid
Nephrotic Syndrome (RSNS)
proteinurine +3
- patient was advised to took
- Got metilprednisolon FD
in 4 weeks, and remision. CPA for chemotheraphy, but
parents refused.
-after got metilprednisolon
AD, patient relapse twice -Got metilprednisolon, cellcept,
captopril.
Diagnose : Dependent
Streroid Nephrotic - After 3 months, patient was
edema again, GFR :77
Syndrome
ml/min/1,72 m2 (CKD stg II), but
refused chemotherapy again
5 years old
- Parents agree to took
CPA for chemotherapy, and
after 2 years
- Edema all over body and
pale, proteinurin +3
- GFR : 16,24 ml/min/1,72
m2
Diagnose RSNS + CKD
stage IV
- never remission, next
theraphy sandimun, but
parents refused because
the cost is too expensive
7 years old
 History of Present Illness

-Edema all over body and pale,

proteinurin +3 - 1 hour before admission, patient have
seizure once. Duration 3 min, after
- GFR : 11,73 ml/min/1,72 m2 seizure patient is conscious, tonic
Diagnose RSNS + CKD stage clonic movement on hands and feet.
V - Fever (-), never had seizure before,
- Suggested to have dialysis but trauma (-), nausea and vomit (-)
parents refused.

9 years old 10 years old

Family history • Nephrotic syndrome (-)

Growth and • Impaired growth detected

development
 Physical Examination
Eyes:
Oedem palpebral(-/-)
Conjungtiva anemis (+/+)
General appearance : Looks moderate pain
Awareness : Composmentis

Vital signs
•BP : 120/80 mmHg
•Pulse : 146 x/min
•RR : 34 x/min
•T : 36,5ºC Pale on hands and feet palmar

Nutrition Upper Extremities : Abdomen :

pitting oedem (-/-)
•Weight : 25 kg Acites (-), supple, shifting dullness (-)
•Ideal weight : 26 kg
•Height : 128 cm
•Nutrition status : poor nutrition Genital :
Scrotum oedem (-)

Lower Extremities :
pitting oedem (+/+)
Laboratory Work Up
21th March 2019

Routine blood test Blood chemistry

• Hb : 4,1 g/dl • Ureum : 188 mg/dl
• Creatinin : 14,26 mg/dl
• Ht : 13,0%
• Blood glucose : 103
• WBC : 14.840/uL mg/dL
• PLT : 202.000/uL
• MCV : 83,9 fl
• MCH : 26,5 pg
• MCHC : 31,5 g/dL
Urinalysis
• Protein : +2
• Glucose : (-)
• Erythrocyte : (-)
Treatment

• Tranfusion PRC 200 cc

• Inj Ceftriaxon 500 mg/12 hour IV

• Inj Furosemid 20 mg/12 hour IV

• Spironolakton tab 2x 125 mg PO

• Multivitamin syr 1x1 cth PO

 Working Diagnosis

Severe Anemia ec Chronic Cidney Disease Stage V

with Steroid Resistant Nephrotic Syndrome(SRNS)

Prognosis
• Quo ad vitam : dubia ad bonam
• Quo ad functionam : malam
23th March 2019 24th March 2019 25th March 2019

Pale (+), seizure (-), fever (-) Pale (+), seizure (-) Pale (+), seizure (-)
BP : 100/70 mmHg BP : 110/70 mmHg BP : 110/70 mmHg

Conjungtiva anemis (+/+) edema pretibial (+/+) Conjungtiva anemis (+/+) edema pretibial (+/+) Conjungtiva anemis (+/+) edema pretibial (+/+),

Hb : 4,1 gr/dL Hb : 6,4 gr/dL BC : 323,5

Eritrosit : 1.550.000/uL Eritrosit : 2.240.000/uL Diuresis : 1,53 cc/Kg/jam

Albumin : 2,1 g/dL Albumin : 2,1 g/dL Urinalysis :

MCH : 26,5 fl Ureum : 199 mg/dL Protein : +3

MCHC : 31,5 g/dL Creatinin : 13,20 mg/dL Glucose : +1

Ureum : 188 mg/dL GFR : 5,05 ml/minute Erythocyte : 1-2 LPB

Creatinin ; 14,26 mg/dL Urinalysis :

GFR : 5,09 ml/minute Protein : +3

Urinalysis : Glucose : +1

Protein : +3 Erythocyte : 1-2/LPB

Glucose : +1

Erythocyte : 12-15/LPB

Sever Anemia ec Chronic Cidney Disease Stage V Sever Anemia ec Chronic Cidney Disease Stage V Sever Anemia ec Chronic Cidney Disease Stage V

with Steroid Resistant Nephrotic Syndrome(SRNS)
- Inj Ceftriaxon 500 mg/12 hour IV
- Inj Furosemid 20 mg/12 hour IV
- Spironolakton tab 2x 125 mg PO
- Multivitamin syr 1x1 cth PO
with Steroid Resistant Nephrotic Syndrome(SRNS)
- Inj Ceftriaxon 500 mg/12 hour IV
- Inj Furosemid 20 mg/12 hour IV
- Spironolakton tab 2x 125 mg PO
- Multivitamin syr 1x1 cth PO
with Steroid Resistant Nephrotic Syndrome(SRNS)
- Inj Ceftriaxon 500 mg/12 hour IV
- Inj Furosemid 20 mg/12 hour IV
- Spironolakton tab 2x 125 mg PO
- Multivitamin syr 1x1 cth PO
26th March 2019 27th March 2019 28th March 2019
Pale (+), seizure (-) Pale (+), seizure (-) Pale (+), seizure (-)
BP : 100/70 mmHg BP : 90/60 mmHg BP : 90/60 mmHg

Conjungtiva anemis (+/+) edema pretibial (+/+) Conjungtiva anemis (+/+) edema pretibial (+/+) Conjungtiva anemis (+/+) edema pretibial (+/+),

BC : -350 BC : -70 BC : -200

Diuresis : 2,24 cc/Kg/jam Diuresis : 1,9cc/Kg/jam Diuresis : 1,63 cc/Kg/jam

Urinalysis : Hb : 6,45gr/dL Urinalysis :

Protein : +3 Eritrosit : 2.350.000/uL Protein : +3

Glucose : +1 Albumin : 2,1 g/dL Glucose : (-)

Erythocyte : 10-15/LPB Ureum : 182 mg/dL Erythocyte : 2-4/ LPB

Creatinin : 12,24 mg/dL

GFR : 5,93 ml/minute

Urinalysis :

Protein : +3

Glucose : +1

Erythocyte : 20-25/LPB
Sever Anemia ec Chronic Cidney Disease Stage Sever Anemia ec Chronic Cidney Disease Stage Sever Anemia ec Chronic Cidney Disease Stage
V V V

with Steroid Resistant Nephrotic
Syndrome(SRNS)
- Inj Ceftriaxon 500 mg/12 hour IV
- Inj Furosemid 20 mg/12 hour IV
- Spironolakton tab 2x 125 mg PO
- Multivitamin syr 1x1 cth PO
with Steroid Resistant Nephrotic
Syndrome(SRNS)
- Transfusi PRC 150 cc
- Inj Ceftriaxon 500 mg/12 hour IV
- Inj Furosemid 20 mg/12 hour IV
- Spironolakton tab 2x 125 mg PO
- Multivitamin syr 1x1 cth PO
with Steroid Resistant Nephrotic
Syndrome(SRNS)
- Inj Ceftriaxon 500 mg/12 hour IV
- Inj Furosemid 20 mg/12 hour IV
- Spironolakton tab 2x 125 mg PO
- Multivitamin syr 1x1 cth PO
 Discussion Hypoalbuminemia

Progress of patient
condition: THEORY
Nephrotic syndrome relapse Hypoalbuminemia caused by
frequent relapse protein loss
steroid dependent
steroid resistant CKD CASE
Hypoalbuminemia and oedem

THEORY THEORY THEORY

Relapse : Give Frequent relapse Steroid resistent: THEORY
prednisone full and steroid After 7th CPA and still proteinuria +3,
Oral CPA everyday
dose till remission, dependent : the next therapy are back to steroid 
in 3-6 months or
alternating dose,
Give prednisone Puls CPA 7 cycle + Cyclosporin Micophenolate mofetil
tappering off
full dose till prednisone AD or rituximab
CASE remission, start CASE
Metilprednisolon CPA and CASE
prednisone AD 350 mg puls CPA 7
FD and AD Suggested to get cyclosporine, but
cycle +
CASE Metilprednisolon parents refused
Metilprednisolon AD
FD and AD
 Chronic kidney disease Parents factor
-always refused
therapy because afraid
of side effect of the
THEORY theraphy and cost
problem
Decreased GFR in 3
months caused by -incooperative and
long and persistent uneducational parent
proteinuria cause
damage of nephrons
CASE
Decreased GFR while
diagnosed by
ANEMIA Resistant Steroid,
SEIZURE
from stage II – IV - V.
THEORY THEORY
Decreased of High level of ureum in
erythropoetin. blood can cause
CASE encephalopathy
Tranfussion of PRC CASE
200 cc Seizure once before
admission to hospital.
Never have seizure
again in hospital.
Suggested to have
dialysis but parents
refused
BB : 25 Kg

BB edema pretibia : BB - 10% BB

25 – 10% 25
: 23

Status Gizi : BB /BBI x 100%

: 23/26 x 100%
: 88,46% (gizi kurang)

Kebutuhan kalori : BBI x RDA

: 26 x 60
: 1.560 Kkal/ hari
Karbohidrat : 936 kal
Protein : 312 kal
Lemak : 312 kal
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