FINAL EVALUATION

Name of participant: Merlien Anice Sembai

I . PATIENT IDENTITY
Name : D.A.M Father’s name : Mr. M
Age : 9 years 2 months Age : 47 y.o
Date of birth : March 27th 2009 Occupation :-
Sex : Male Education : Jr High School
MR/Reg number : C640XXX/ 960XXX Mother’s name : Mrs. S
Date of admission : June 22nd 2018 Age : 39 y.o
Ward : Pediatric Occupation : housewife
Education : Jr High School

II. ANAMNESIS
Anamnesis was performed on the 4th day of admission (June 25th 2018, 18.00
IWST) at pediatric ward to the mother, patient, and data was obtained from the medical
record.
1. Present illness:
Chief complaint: edema (referred from regional hospital with steroid-resistant
nephrotic syndrome (SRNS)
Two weeks prior to admission the child was initially presented with edema starting
from both palpebral in the morning, spreading to the face, abdomen and lower
extremities. No fever, no headache, no dyspnea, no nausea, no pallor, no jaundice, no
skin rash, no cloudy and foamy urine, decreased volume and urine frequency, no
dysuria. Once daily bowel movement with no stool changes. The child carried out
normal daily activities and still had good appetite.
Eight days prior to admission the child suddenly vomited approximately 4-5 times a
day, and had soft stool consistency twice a day. No fever, no cough, no pallor. The
child felt dizzy and weak. He was taken to emergency room in regional hospital in
Purwokerto. Suddenly he had a general tonic clonic seizure at the emergency room for
3 minutes. The child became unconscious after the seizure. He was admitted to
Pediatric Intensive Care Unit (PICU). After four day of adminssion in PICU, the child
gained consciousness and transferred to pediatric ward in the following day.

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Four days prior to admission In spite of his progress condition after the seizure, the
edema was getting worse and spread into his genitalia and became painful. The child
had abdominal discomfort, dyspnea, and nauseous. He received albumin transfussion.
One day prior to admission the edema, abdominal discomfort, and dyspnea was still
remained. No fever, no cough, no vomiting. His urine became decreased but no
dysuria. The child was later referred to Dr. Kariadi hospital with steroid-resistant
nephrotic syndrome.
Admission day 1-3
On admission the child was presented with puffy eyes, edema on both pretibial area,
ascites, and genitalia edema. He complained of abdominal discomfort and nauesa, and
a dcreased urine volume, no dysuria. No fever, no vomiting, no headache. He was
alert, active and his vital signs were : Blood Pressure (BP) 108/89 mmHg (P95 + 12
mmHg), Heart Rate (HR) 80 bpm, Respiratory Rate (RR) 24 breath/min, axillary
temperature 36,6o C. Physical examination revealed moon face, palpebral edema, chest
examination were normal; abdominal distention and shifting dullness, scrotal edema,
and pretibial edema. His hematology examination from the previous hospital (June 14th
2018) were leucocytosis (23.780), hypoalbuminemia (0,39), ureum 75,5, creatinine 3,
61, hyperkalemia (5,3). His urinary examination was proteinuria +3. The initial
assessments were SRNS and stage II hypertension with short stature. He was
commenced on intravenous infusion of D 5 ½ NS 360/15/5 gtts/min, valsartan 40
mg/24h and prednisone 5 mg 4-0-4 alternate dose (Monday, Wednesday, Friday),
cyclosporine 25mg/12h. He was admitted for further evaluation at pediatric ward.
Following his admission at the pediatric ward, his clinical symptoms were still
the same with BP of 110/90 mmHg, positive fluid balance, and urine output was 2.4
mL/Kg/h. His blood results were: Hb 11.6 g/dL, Ht 34.2 %, MCH 28.4 pg, MCV 83.6
fL, MCHC 33.9 d/dL, WBC 16.4 x 103/uL, Plt 527 x 103/uL, RDW 12,8%, blood
glucose level 111 mg/dL, albumin 1.9 g/dL, ureum 58 mg/dL, creatinine 1.1 mg/dL,
glomerular filtration rate (GFR) 60 mL/min/1.73 m2, uric acid 4.8 mg/dL, calcium 1.8
mmol/L, sodium 139 mmol/L, potassium 4.1 mmol/L, and chloride 112 mmol/L,
Anorganic phospate 4.2 mg/dL ; Urinalysis revealed protein 500 (++++), reduction
300 (+++), blood 150/uL (++++). He was assessed with SRNS on cyclosporine on 6 th
month with comorbid anasarca edema and hypoalbuminemia, hypertension stage II,
chronic kidney disease stage II ( GFR 60mL/min/1,73m 2), hypocalsemia. He got nasal
canule oxygen 2 lpm, Furosemide injection 20mg/12h, Ca gluconas injection

2
 10mL/12h, cyclosporine 25mg/12h, prednisone was discontinued and changed into
methylprednisolone 8mg 2-2-1 full dose, valsartan 40mg/12h, sucralfat syrup 5mL/8h,
albumin 20% transfusion 200 mL divided into two doses. He was planned to get
echocardiography for screening.
On the fourth day of admission (when the patient was selected as final
examination)
The child had minimal palpebral edema, but still obvious pretibial edema, ascites, and
scrotal edema, moon face, abdominal discomfort, no fever, no skin rash, no nausea and
vomiting, no headache, no foamy urine, once daily bowel movement with soft stool
consistency; he finished the meals served from the hospital. The child was scheduled
for echocardiography
2. Past medical history
− The child was diagnosed with nephrotic syndrome since May 2017 at Purwokerto
Hospital, he received prednisone full dose for a month. He had routine visits at
outpatient clinic.
− History of admission at Dr. Kariadi Hospital in July 2017 due to steroid resistant
nephrotic syndrome, he received cyclophosphamide and prednisone for 6 months,
and had kidney biopsy in September 2017.
− History of admission at Purwokerto Hospital in May 2018 due to gastrointestinal
bleeding and severe anemia (Hb 3), he received packed red cell transfusion.
− No history of skin infection
− No history of hematuria
− No history of recurrent fever, skin rash, or muscle pain
− No history of recurrent pallor, prior blood transfusion, and weight loss
− No history of mental, physical abuse or domestic violence
− No prior hospitalization
3. Family medical history
⁻ No history of hypertension, diabetes mellitus
⁻ No family history of kidney disease

Pedigree

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 I

II

III

4. Personal and social history

a. Pre-natal history
The child was born from a G2P1A0, 30-year-old mother with full term pregnancy. She had >4x
routine antenatal care to midwife during pregnancy and took regular vitamins daily. No
history of fever, rash, pain, edema, and other illness during pregnancy. She had normal blood
pressure.
b. Natal history
The child was born spontaneously, assisted by midwife. He was a vigorous baby with birth
weight of 4400 gram. No history of respiratory problems, cyanosis or jaundice.
c. Post natal history
The child had good sucking reflex, steadily gained weight. No history of jaundice, seizure, or
spontaneous bleeding..
d. History of nutrition
⁻ 0 – 5 months old : breast milk on demand.
⁻ 5 – 7 months old : banana puree 2-3 times daily.
⁻ 8 – 10 months old : baby milk cereal 2-3 times daily.
⁻ 11 – 12 months old : rice porridge 2-3 times daily.
⁻ The child was breastfed until the age of 2 years old.
⁻ Now the child has regular meals 3 times daily with various menu consists of rice,
eggs, chicken, fish, tofu, vegetables, and fruits occasionally. He eats any kind of food,
no restriction.
⁻ No history of food allergy.
Impression: exclusive breastfeeding, adequate intake quality and quantity

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 e. Growth and development history
Growth
- Birthweight was 4400 gram, no data on birth length
- No history of weight loss recorded.
- Last month body weight was 26 kg (with edema), present weight is 26 kg.
- Present height is 120 cm.
Development:
Milestones
- Sit without support : 6 months old
- Crawling : 7 months old
- Stand up by herself : 10 months
- Walk : 12 months
- Speak words : 16 months

The child is now 9-years and 3-months old. He is in 3rd grade of elementary high
school. He achieved second rank at class during 1st and 2nd grade. Since he was diagnosed
with nephrotic syndrome, he missed a lot of class due to undergoing routine treatment and a
couple of hospital admission. He has a lot of friends and used to do any activities including
sports.
⁻ Strength And Difficulties Questionnaire (SDQ: emotional symptomps, conduct
problems, hyperactivity, peer problems). Result: normal
⁻ Pediatric symptoms checklist 17 items (PSC 17). Result: normal
⁻ PedsQL : physical health summary score : 93.75 (normal), psychosocial health
summary score : 80 (normal), impaired score in school functioning (60).
⁻ Parenting style : democratic
Impression: normal developmental status, impaired score in school functioning
f. Immunization history
⁻ BCG : 1 time, 1 month (scar +)
⁻ DPT : 3 times (2,3,4 months old)
⁻ Hepatitis B : 4 times (0, 2, 3, 4 months old)
⁻ Polio : 4 times (0, 2, 3, 4 motnths old)
⁻ Measles : 1 time (9 months old)
⁻ Booster : (+)
⁻ Month of Children School Vaccinaton : Td and measles on 1st grade
Impression: complete recommended routine and booster immunization schedule

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 g. History of child’s basic needs
Care
- The child was exclusively breastfed until the age of 2 years old.
- He had received complete recommended and booster immunization from Posyandu and
during school vaccination program
- Health care services obtained from midwife and doctors at local primary health care and
hospital
- The boy lives with his mother and sister in a permanent house
Love
⁻ The boy is the 2nd child, he lives with his mother and sister and raised with full
support. He was an expected child from a happily married couple.
Stimulation
⁻ The boy had proper stimulation from both parents.
⁻ He is in 3rd grade of elementary high school and gets along well with his peers.
h. Socio-economic condition of the family
His father had passed away. His mother does not work because she has to accompany
him undergoing treatment. They receive financial aid from father’s relatives. Medical and
hospital cost are covered by the national health insurance. This family is categorized as
poor family according to the criteria of Central Bureau of Statistics.
Impression: low socioeconomic level.
Environment:
The family (mother, grandmother and sister) live in a 8x6 m2 self-owned permanent house,
with tile roof, ceramic floor, and stoned wall. The house has 2 bedrooms, a living room, a
kitchen, and 1 bathroom. It has 2 doors and exposed to sunrays through 4 windows. The
electricity source is from National Electricity with 450 watts of power. Water source is
from a well. Waste water is drained through pipes to the ditch that flows into the river, the
garbage is collected into the bins and thrown away every 2 days. The distance to the
nearest midwife and primary health care is approximately 15-minutes driving by
motorcycle; meanwhile, distance to local hospital is about 1-hour driving by motorcycle.

House Map

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III. PHYSICAL

EXAMINATION
General condition : alert, active, palpebral edema, breathing spontaneously, no
breathing difficulty.
Vital sign : Heart rate : 112 bpm
Pulse : regular, sufficient volume and pressure
Respiratory rate : 20x/minute, temperature: 36,8oC (axilar)
Blood pressure : 80/60 mmHg (P50)
P50 : 96/57 mmHg P95+12 : 128/86 mmHg
P90 : 107/60 mmHg Crisis : 168/111 mmHg
P95 : 112/74 mmHg

Pain scale : 1 (numeric pain scale): mild

Skin : dark brown
Eyes : anemic conjunctival palpebral (-/-), jaundice (-), discharge (-),
palpebral edema +/+

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Pupil : symmetry Ø3 mm/Ø3 mm, light reflexes/corneal reflexes (+/+) normal
Ears : discharge (-), retroauricular lymphadenopathy (-).
Nose : nasal flaring (-), discharge (-), epistaxis (-)
Mouth : pale(-), bleeding (-), stomatitis (-)
Tounge : papil atrophy (-), trush (-), bleeding (-)
Pharynx : hyperemic (-), tonsil: T1-T1, detritus (-), large crypte (-)
Teeth : caries (-), gingivitis (-)
Cheek : tenderness (-), moon face (+)
Neck : lympadenopathy (-/-)
Axila : lymphadenopaty (-)
Chest : symetric on static and dynamic, chest indrawing (-)
Lung
Inspection : symmetrical chest expansion, chest indrawing (-)
Palpation : equal fremitus on both sides
Percussion : sonor in all lung field
Auscultation : vesicular breath sound (+) normal; adventitious sound: wheezes (-), no
crackles or rhonci noted
Heart
Inspection : ictus cordis non visible
Palpation : ictus cordis palpable on left midclavicular line SIC V, no thrills or
heaves palpated
Percussion : right border : intercostal space IV right parasternal line
left border : intercostal space IV left midclavicle line
upper border : intercostal space II left parasternal line
Auscultation : Heart sound I and II normal, murmur (-), gallop (-)
Abdomen
Inspection : distention, striae (-), petechiae (-)
Auscultion : normal bowel sound
Percussion : tympanic, shifting dullness (+)
Palpation : tenderness (-), guarding (-), flank pain (-), abdominal circumference 74
cm
Lien : Schuffner 0
Hepar : not palpable
Inguinal : lymph node enlargement (-/-)

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Genital : Male, not circumcised, pubic hair (-), scrotal edema (+), penis edema (-)
Limbs superior inferior
Edema -/- +/+
Movement symmetrical
Tonus normotonus
Muscle Strength 555 / 555 555/555
Pallor -/- -/-
Striae -/- -/-
Cranial nerve examination : normal findings
Anthropometric
9 years 2 months old boy, birthweight: 4400 gr
Body weight : 26 kg (with anasarca edema )
Bodyweight last month: 26 kg (with anasarca edema)
Ideal bodyweight : 23 kg
Head circumference : 51 cm (Nelhauss -2 – 0 SD) Height : 120 cm
WAZ : NA
BMI : NA
HAZ : -2.883 SD (short stature)
Impression: cross sectional: short stature
Longitudinal : flat of growth

A. LABORATORY AND IMAGING EXAMINATION

Hematology Reference 22/06 Clincal chemistry Reference 22/06
00.53 00.53
Hb (gr/dL) 10.5 - 15.0 11.6 Glucose (mg/dL) 80-160 111
Ureum (mg/dL) 15-39 58
Ht (%) 36 - 44 34.2
Erythrocyte (106/uL) 3 – 5,4 4.09 Creatinine (mg/dL) 0,60-1,30 1.1
MCH ( pg) 23,00 – 31,00 28.4 Albumin (g/dL) 3.4-5.0 1.9
MCV (fL) 77 - 101 83.6 Uric acid (mg/dL) 3.5-7.2 4.8
MCHC (g/dl) 29,00 – 36,00 33.9 Na (mmol/L) 136-145 139
Leukocyte (103/uL) 5 – 13.5 16.4 K (mmol/L) 3,5-5,1 4.1
Thrombocyte (103/uL) 150 – 400 527 Cl (mmol/L) 98-107 112
RDW (%) 11,60 – 14,80 12.8 Ca (mmol/L) 2,12-2,52 1.8
Impression: leukocytosis Anorganic Phosphate 2.4-5.1 4.2
thrombocytosis GFR (mL/min/1.73 m2) 60
Impression: hypoalbuminemia
hypocalcemia
Impression: leukocytosis, thrombocytosis, hypoalbuminemia, hypocalsemia

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Urinalysis 22/06/2018
Test Units Normal range 22/06/2018
DAY #2
Color Yellow
Clarity Cloudy
Specific gravity 1,003 -1,025 1,010
Ph 4,8 – 7,4 6
Protein mg/dL Neg 500
Reduction mg/dL Neg 300
Urobilinogen mg/dL Neg Neg
Bilirubin mg/dL Neg Neg
Aceton mg/dL Neg Neg
Nitrit Neg Neg
Blood : 150/uL
Sediment
Epithel /uL 0 – 40 0-1/LPF
Tubular epithelial /uL 0–6 Neg
Leukocyte /uL 0 – 20 2-4/LPF
Eritrocyte /uL 0 – 25 4-8/LPF
Crystal /uL 0 – 10 Uric acid +/Pos
Pathologic cast /uL 0 – 0.5 1,45/ Ul
Coarse granule /LPF Neg 2-5/LPF
Fine granule /LPF Neg Neg
Hyaline cast /uL 0 – 1.2 2-4/ul
Epithelial cast /LPF Neg Neg
Erythrocyte cast /LPF Neg Neg
Leukocyte cast /LPF Neg Neg
Mucus threads /uL 0 – 0.5 Neg
Yeast cell /uL 0 – 25 Neg
Bacteria /uL 0 – 100 Neg
Sperm /uL 0–3 Neg

Chest XRay (June 22nd 2018)

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Normal heart size
Bronchopneumonia
Minimal pleural effusion in both lungs

IV. RESUME

Patient is a 16 years 11 months old boy with chief complaint of edema from 1.5
months prior to admission that initially starting on both palpebral in the morning, spreading
to the face, abdomen and lower extremities. No fever, no headache, no dyspnea, no
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nausea, no pallor, no jaundice, no skin rash, cloudy and foamy urine, normal volume and
urine frequency, no dysuria. Once daily bowel movement with no stool changes. He was
taken to private hospital, initial laboratory examination revealed significant proteinuria +3
and low serum albumin level and diagnosed with nephrotic syndrome, had routine weekly
visits to the pediatrician and started on steroid treatment of prednisone (10 tablets BID) and
furosemide (1 tablet TID). After 1.5 months steroid treatment, evaluation of urinalysis
revealed persistent proteinuria +3. He was referred to tertiary hospital with steroid-resistant
nephrotic syndrome. He had past medical history of nephrotic syndrome 5 months ago,
with complete remission, no history of skin infection, no history of hematuria, no history
of mental and physical abuse. There were no history of hypertension, diabetes mellitus, and
kidney disease. He had normal perinatal history, normal growth and development history,
impaired school functioning. He had complete recommended routine and booster
immunization schedule. He lived with his parent and brother in low socioeconomic level.
On admission the child was presented with puffy eyes, slightly edema on both pretibial
area. No fever, no dyspnea, no nausea and vomiting, no headache, no abdominal
discomfort. He complained of persistent foamy urine, no dysuria. Once daily bowel
movement with soft stool consistency. He was alert, active and his vital signs were: BP
150/90 mmHg (>P99 + 5 mmHg), HR 80 bpm, RR 18 breath/min, axillary temperature 36o
C. Physical examination revealed moon face, palpebral edema, dental caries; chest and
abdominal examination were normal; no scrotal edema, minimal pretibial edema. The
initial assessments were steroid-resistant nephrotic syndrome and stage II hypertension
with normal nutritional status and normal stature. He was commenced on intravenous
infusion of D 5 ½ NS 960/40/10 gtts/min, Captopril 25 mg/8h and Furosemide 20 mg/12h
PO daily; alternate dose of prednisone 3-3-2; 3 x 1 serving of rice, 3 x 250 ml wholemilk.
Following his admission at the pediatric ward, his clinical symptoms were
improved with BP of 120/70 mmHg, positive fluid balance, and urine output of 2.36
mL/Kg/h. His blood results were: leucocytosis, neutrophilia, hypercholesterolemia, normal
electrolyte level, normal glomerular filtration rate (GFR), and proteinuria 500 mg/dL. He
was assessed with partial remission of steroid-resistant nephrotic syndrome and stage I
hypertension. Furosemide was ceased and switched to valsartan 40 mg once daily. He was
planned to receive cycophosphamide treatment, had scheduled kidney biopsy and
extraction of teeth, screening for metabolic syndrome and consult to social pediatric and
growth development division for impaired school functioning.

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 V. DIFFERENTIAL DIAGNOSIS

1. Steroid resistant nephrotic syndrome

Dd/ Mesangioproliferative glomerulonephritis
Lupus nephritis
IgA nephropathy
2. Hypertension controlled by medication
Dd/ Secondary hypertension
Primary hypertension
3. Chronic Kidney Disease stage II
4. Short stature

VI. WORKING DIAGNOSIS

1. Main diagnosis : Steroid-resistant nephrotic syndrome (N04.0)
2. Comorbids :
Hypertension Stage II (I10)
Leucocytosis
Thrombocytosis
Hypocalcemia
3. Complication : Chronic Kidney Disease II
4. Nutritional status : Short stature

VII. PROBLEMS

1. Problem on diagnostic
- Diagnostic histopathology SRNS with kidney biopsy
2. Problems on management
- Findings evidence of management steroid-resistant nephrotic syndrome with
cyclosporine
- Psychological and social impact on patient and parents
- Nutritional care to maintain the nutritional status
3. Problems on preventive
- Vulnerability to infection emerged the need for implementing clean and healthy
lifestyle
- Preventing the end stage renal disease
4. Problems on monitoring
- Monitor side effects of corticosteroid
- Monitor side effects of cyclosporine
- Monitor compliance of therapy
- Monitor renal function
- Monitor of nutritional status
5. Problems on prognosis
- Steroid resistant nephrotic syndrome may develop end stage renal disease

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 VIII. MANAGEMENT PLAN
1. Emergency management:
When the patient was pointed as case, no emergency had been found
2. Diagnostic investigation: ANA, AntiDsDNA level, parathyroid hotmone,
3. Medical therapy :
⁻ IVFD D5 2 NS 240/10 ml/hour
⁻ Injection: Furosemide 20 mg/12 hour
Gluconas calcicus 10 ml/12 hour
⁻ Per Oral:
Cyclosporine 25 mg/12 hour
Methylprednisolone 8 mg2 – 2 – 1 (Full dose)
Valsartan 40 mg/24 hour
4. Non medical therapy:
⁻ Consultation to cardiology division
⁻ Consultation to division of developmental-behavioral pediatric for impaired school
functioning
⁻ Cosultation to ophthalmologist to rule out any ocular complication due to long term
steroid
⁻ Cosultation to nutrition and metabolic division
⁻ Life style modification for treatment of hypertension
5. Pediatric nutrition care :
Boy, 9 years 2 months, BW: 26 kg, Height: 120 cm.
Nutritional management with 5 steps of pediatric nutrition care (PNC) :
1. Assesment: WAZ : NA, BMI : NA, HAZ: -2.883 SD,
Impression: short stature
2. Nutritional requirements: Calories 40 kcal/kgbw/day, protein 1 gram/kgbw/day.
Total calories: 2,100 kcal/day, protein: 53 gr/day, fluid (Darrow): 2,160 ml/day
3. Route of administration: oral
4. Formula : 3x1 rice meal 1.566 kcal, 3x250 ml milk, intravenous fluid D5 ¼ NS 480 ml/24 hrs
Total calorie 2.076 kcal, protein 63.4 gram. (calorie 99% of RDA, protein 119% of RDA)
5. Monitoring: acceptability, tolerance, affectivity (weight gain)
6. Nursing plan :
⁻ Preventing infection with universal precautions (5 moments of washing hand)
⁻ Preventing infection with oral hygiene
⁻ Providing information on patient’s illness and conditions
⁻ Performing pain evaluation

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 ⁻ Keep the room clean, create comfortable ambience to patient
7. Monitoring plan
⁻ Monitoring on fluid balance and diuresis
⁻ Diet acceptability, tolerance, weight gain, growth
⁻ Monitoring cyclosporine and corticosteroid side effects
⁻ Monitoring blood pressure
⁻ Evaluation of proteinuria, serum electrolyte level, ureum and creatinine level
⁻
8. Communication, information and education plan
⁻ Providing information about the disease and diagnostic procedures
⁻ Providing information about the clinical importance of maintaining fluid balance
⁻ Call the nurse or doctor in charge if there is any emergency or deterioration of patient
condition, for example: seizure, fever, and other complaints.
⁻ Providing education and information to keep the environment clean and routinely
practicing five moments of hand-hygiene
⁻ Providing information about the importance of oral hygiene
⁻ Make sure the child to finish her meals
⁻ Providing information on life style changes to control blood pressure
⁻ Providing information about the patient's prognosis based on possible
histopathological outcomes
⁻ Providing information about the side effects of steroids and cyclosporine treatmen

IX. FOLLOW-UP
Day of Follow up day 1 (Admission day 5) Follow up day 2 (Admission day 6)
Admission (26/6/2018) (27/06/2018)
Time 07.00 iwst 07.00 iwst
Fever (-), cough (-), edema (+), Fever (-), cough (-), nausea (-),
abdominal discomfort (-), scrotal pain vomiting (-), anasarca edema (+),
Subjective
(+) abdominal discomfort (-), scrotal pain
(+)
Objectives

General Alert, active Alert, active

Vital signs HR : 102 bpm t : 36,6°C HR : 102 bpm t : 36,7 °C
RR : 24 times/min BP : 120/80 mmHg RR : 22 times/min BP : 100 /70
(P95) mmHg (P90)
P : regular, adequate volume/pressure P : regular, adequate volume/pressure
Physical BW : 26 kg, Height : 120 cm, BSA : BW : 26 kg, Height : 120 cm, BSA :
examinati 0,87 m2 0,87 m2
on Face : moon face (+) Face : moon face (+)
Eye : anemic (-/-), palpebral edema (+/ Eye : anemic (-/-), palpebral edema (+↓/
+) +↓)
Nose : nasal flaring (-) Nose : nasal flaring (-)
Mouth : cyanosis (-) Mouth : cyanosis (-)

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 Chest : symmetry, retraction (-) Chest : symmetry, retraction (-)
Cor : Heart sound s1-s2 N, gallop (-), Cor : Heart sound s1-s2 N, gallop (-),
murmur (-) murmur (-)
Lung : vesicular breath sound (+)/(+), Lung : vesicular breath sound (+)/(+),
fremitus (+)/(+) minimal, wheezes (-)/ fremitus (+)/(+) minimal, wheezes (-)/
(-) (-)
Abdomen : distention, bowel sound (+) Abdomen : distention, bowel sound (+)
N, shifting dullness (+), liver not N, shifting dullness (+), liver not
palpable, lien Schuffner 0 palpable, lien Schuffner 0
Extremity : warm (+), CRT <2” (+), Extremity : warm (+), CRT <2” (+),
pitting edema -/- +/+ pitting edema -/- +/+
Genitalia : scrotal edema (+), penis edema Genitalia : scrotal edema (+), penis
(-) edema (-)
Additional Fluid balance : -1160 cc Lab evaluation 27/06/2018
examinati Diuresis: 2,96 ml/kgbw/hour Urinalysis 26/06/2018
on Color : Yellow
Lab evaluation 26/06/2018 Clarity : clear
Albumin 1,9 g/dL Specific gravity : 1.015
Sodium 131 mmol/L pH : 6
Potassium 4,0 mmol/L Protein : 500 mg/dL
Chloride 102 mmol/L Reduction : 300 mg/dL
Calcium 2.34 mmol/L Urobilinogen : Negative
Bilirubin : Negative
Echocardiography 26/06/2018 Aceton : Negative
Conclusion : Nitrit : Negative
- Heart structure and function within Blood : 150/uL
normal Epithel 35.8 (1-2/LPF)
- Decreased aortic elasticity Tubular epithelial 32.9 (0-1/LPF)
Recommendation: Oval fat bodies +/Positive
- There’s no additional therapy WBC 38.3 (3-5/LPF)
- Echocardiography evaluation in 6 RBC 311 (15-18/LPF)
months Crystal : 0.5/uL
Oxalat calcium +/Positive
Coarse granule : Negative
Fine granule : 0-1/LPF
Hyaline cast : 2.45 (1-3/LPF)
Epithel cast : Negative
RBC cast : Negative
WBC cast : Negative
Mucus : 0.72/uL
Yeast cell : Negative
Bacteria : 352.7/uL
Sperm : Negative

Assesment  Steroid-resistant nephrotic syndrome on  Steroid-resistant nephrotic syndrome on

6th month cyclosphorine cyclosphorine
 hypertension stage I  hypertension stage I
 CKD stage II  CKD stage II

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  Minimal pleural effusion in both lungs  Minimal pleural effusion in both lungs
 Short stature  Short stature

Medical -IVFD D5 1/2 NS 240/10 mL/hour -IVFD D5 1/2 NS 240/10 mL/hour

therapy -Injection : -Injection :
Furosemide 20 mg/12h IV Furosemide 20 mg/12h IV
(1.5mg/kg/day) (1.5mg/kg/day)
Gluconas calcicus 10 ml/12 hour - Per Oral:
(discontinued) Cyclosporine 25 mg/12 hour

Per Oral: Methylprednisolone 8 mg2 – 2 – 1

Cyclosporine 25 mg/12 hour (Full dose)
Methylprednisolone 8 mg2 – 2 – 1 Valsartan 40 mg/24 hour
Plan

(Full dose)
Valsartan 40 mg/24 hour
Nutrition 3 x 1 serving of low sodium rice 3 x 1 serving of low sodium rice
3 x 200 mL nefrisol D 3 x 200 mL nefrisol
Program - Fluid balance and diuresis/12h - Fluid balance and diuresis/12h
- Lab examination : HDL, LDL, Total - Waiting for laboratory result of ANA, anti
Cholesterol, Triglyceride, ANA, anti DS- DS-DNA, HDL, LDL, Total cholesterol,
DNA, urinalysis Triglyceride
- Consultation with division of nutrition
and metabolic disease

X. PROGNOSIS
Quo ad vitam : dubia ad bonam
Quo ad sanam : ad malam
Quo ad fungsionam : dubia ad malam

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 XI. a. COURSE OF THE DISEASE
Day 1-3 Day 4 Day 5 Day 6
Follow up day 1 Follow up day 2
Puffy eyes, pretibial and scrotal Anasarca edema (+), Anasarca edema (+), fever (-), Anasarca edema (+),
edema, ascites. scrotal pain fever (-), scrotal pain
Leukocytosis 16.400 / µL aabdominal discomfort (-),
BP: 120/80 mmHg (p95) BP: 100/70 mmHg
Hypoalbuminemia 1.9 g/dL scrotal pain (+)
Albumin 1.9 g/dL
Hypocalsemia 1.8 mmol/L
Proteinuria 500 mg/dL, blood 150/uL BP 80/60 mmHg (p50-p90) Ca : 2.34 mmol/L
GFR 60 mL/min/1.73 m2
BP: 110/90 mmHg (> p95 + 12 mmHg Albumin : 2.1 g/dL Asessment:
Asessment: Steroid-resistant nephrotic Steroid-resistant
SRNS on 6th month cyclosporine syndrome on 6th month nephrotic syndrome on
cyclosphorine 6th month
Asessment:
Steroid resistant nephrotic syndrome Hypertension stage II cyclosphorine
hypertension stage I
(SRNS) on 6th month cyclosporine
Hypoalbuminemia hypertension stage I
Hypertension stage II CKD stage II
Hypocalsemia
CKD stage II
Hypoalbuminemia Minimal pleural effusion in both
Program :
Chronic kidney disease stage II Program :
lungs Minimal
Hypocalsemia D5 1/2stature
NS 240 / 10mL/h D5 1/2 NS 240 / 10mL/h D5 1/2 NSpleural effusion
240 / 10mL/h
Short in both lungs
Short stature
Chronic kidney disease stage II Furosemide inj 20 mg/12h Furosemide inj 20 mg/12h Furosemide inj 20 mg/12h
Program : (1.5mg/kg/day) (1.5mg/kg/day) (1.5mg/kg/day)
Short stature
D 5 ½ NS 360/15/5 gtts/min Gluconas calcius inj 10ml/12h Valsartan 40 mg/24h Valsartan 40 mg/24h
Valsartan 40 mg/24h
Valsartan 40 mg/24h Methylprednisolone 8mg 2-2-1 full Methylprednisolone 8mg
Methylprednisolone 8mg 2-2-1 full dose dose 2-2-1 full dose
Methylprednisolone 8mg 2-2-1 full Cyclosporine 25mg/12h
Cyclosporine 25mg/12h Cyclosporine 25mg/12h
dose Fluid balance & diuresis/12h
Furosemide injection 20mg/12h Fluid balance &
Ca gluconas injection 10mL/12h
Cyclosporine 25mg/12h Lab examination : HDL, LDL, Total diuresis/12h
Albumin 20% transfusion 200 mL Fluid balance & diuresis/12h Cholesterol, Triglyceride, ANA, Waiting for laboratory
Echocardiography
anti DS-DNA, urinalysis result of ANA, anti DS-
Consultation with division of DNA
nutrition and metabolic disease 18
XI.b. SCHEME OF CASE ANALYSIS
I.b. SKEMA ANALISIS KASUS

19
20
 XII. CASE ANALYSIS

This patient is a 16 years 11 months old boy, complained about edema from 1.5
month prior to admission that initially starting on both palpebral in the morning, spreading
to the face, abdomen and lower extremities. He is diagnosed with steroid resistant
nephrotic syndrome. The adolescent population signifies the transitory period where the
frequent occurrence of different histopathological lesions in patients with nephrotic
syndrome (NS) is different from that seen in young children as well as that seen in adults.
Approximately 85% of children present primary NS associated with “minimal change
disease” (MCD) in 90% of cases and being associated usually with a good prognosis, 93%
of cases entering complete remission after 4-8 weeks of corticosteroid therapy. Other
histological types like mesangioproliferativ glomerulonephritis and focal-segmental
glomerulosclerosis (FSGS) is more frequent in adolescents.1ﾧ In Atlanta, the characteristic
of patient with childhood nephrotic syndrome diagnosed between 2006 and 2012, we can
find 14 patient 13-18 years old, 6 patient (13%) with steroid resistent nephrotic syndrome
(SRNS) and 8 patient (50%) with steroid dependent nephrotic syndrome (SDNS) and 0
patient with steroid sensitive nephrotic syndrome (SSNS).2ﾧ
SRNS is associated with increased risk of complications due to persistent
proteinuria and therapeutic drug side effects. Bacterial infections, malnutrition,
hyperlipidemia, thromboembolic phenomena and progression to end stage renal disease
(ESRD) are usually seen during the course of SRNS.3ﾧ
The most prevalent histological pattern in SRNS is FSGS, which is the major
glomerular etiology of ESRD in children. The probability of occurrence of ESRD in 10
years in children with SRNS varies between 34-64%. Several risk factors for progression
to SRNS, as persistent proteinuria, older age at onset, initial renal impairment, and
extensive focal sclerosis in biopsy specimens have been reported.4ﾧ (Level of evidence 3)
Risk factors associated with SRNS are NPHS2 gene mutation, gender, and atopic history. SRNS
patients with an NPHS2 gene mutation had more severe clinical manifestations compared to SRNS patients
without the NPHS2 gene mutation. NPHS2 gene mutations have been found in 20-30% of SRNS patients,
mostly present in the third exon of the gene. A total of 153 subjects, consisting of 88 SRNS cases and 65
control subjects were enrolled during the study period. The majority were boys in SRNS group (77.3%) and
in the control group (60.0%). History of atopy was more common in the SRNS group (28.4%) than in the
control group (12.3%).5ﾧ (Level of evidence 3) Risk factors found in the patient are male gender and history

21
of atopy. While the risk factor of NPHS2 gene mutation has not been ruled out because genetic examination
is not performed.
Nephrotic syndrome is a clinical syndrome marked with massive proteinuria (>40
mg/m2 BSA/hour or 50 mg/kg/day or urinary protein/creatinine ratio > 2 mg/mg or
dipstick ≥ 2+), hypoalbuminemia < 2,5 g/dL, edema, and hypercholesterolemia > 200
mg/dL.3 ﾧ Patient with nephrotic syndrome typically present with periorbital swelling
(puffiness), more noticeable in the morning, which progress to generalized edema over
days or weeks. Edema is gravity dependent, localized to the lower extremity in upright
position. Abdominal wall edema and ascites or pleural effussions and scrotal or vulval
edema may be seen. This edema is soft and pitting, keeping the marks of clothes or finger
pressure. Microscopic hematuria is found in up to 30% patients, but gross hematuria is
unusual in idiopathic nephrotic syndrone (INS) and suggest an alternative primary
diagnosis. Intravascular volume depletion may happen despite a fluid gain up to 20% of
body weight. Cardiovascular shock is not unusual, secondary to sudden fall of plasma
albumin, with abdominal pain and symptoms of peripheral circulatory failure with cold
extremities and hypotension. Blood pressure is usually normal but sometimes elevated.6,7ﾧ
This patient had symptoms of edema of the palpebral spreading to the abdomen and lower
limbs, hypertension; laboratory investigations revealed significant proteinuria,
hypoalbuminemia at the onset of the symptoms that turned to normal by the time of
examination and hypercholesterolemia so diganosis of nephrotic syndrome was
established. While the diagnosis of steroid-resistant was based on the response to steroid
therapy, this patient achieved no remission despite having a 4-week course of prednisone
FD. The patient was given prednisone with a daily dose exceeding the recommended dose
of 100 mg / day for 6 weeks but persistent proteinuria 500 mg / dL was remained. Based on
the response to glucocorticoid therapy, nephrotic syndrome is classified as steroid sensitive
nephrotic syndrome (SSNS) and steroid resistant nephrotic syndrome (SRNS). SRNS is
defined as failure to achieve remission despite daily therapy of oral prednisone at full dose
(2 mg/kg/day) for 4 weeks.3ﾧ Urinary protein carbonyl content (UPCC) examined before
starting steroid therapy can be used to predict the steroid dependence and resistance in
children with idiopathic nephrotic syndrome. Using cutoff limit of 5.10 nmoles/mg of
protein, UPCC can predict steroid dependence or resistance cases with 83.3% sensitivity
and 85.2% specificity.8 ﾧ (Level of evidence 2) When the patient was first diagnosed at
previous health care facility, UPCC assay was not measured before starting steroid therapy.
This patient was programmed for a kidney biopsy to determine any histopathologic

22
abnormalities of his kidneys. Indication for renal biopsy in this patient is the onset of
nephrotic syndrome ≥16 years and SRNS.3 Histopathology may help predicting the
prognosis more specifically.
Nephrotic syndrome patients should undergo urinalysis and microscopy, urinary
protein to creatine ratio (Upc) or 24-h urinary protein estimation, serum albumin, total
protein, cholesterol, and creatinine. Infectious disease workup including PPD (mantoux)
skin test and chest x ray in the epidemic areas, and HIV, HBV, or HCV serology or PCR
for patient at risk. Kidney biopsy is reserved for infant and older children, and those with
“atypical” presentation and for SRNS. Kidney biopsy is necessary to exclude secondary
causes of nephrotic syndrome, and assess the extent of interstitial and glomerular
fibrosis.6,9ﾧ
Currently, there is no consensus about the most appropriate secondline agent for
treating children with refractory childhood nephrotic syndrome. Immunosuppressive
agents (ie, cyclophosphamide, cyclosporine, tacrolimus and mycophenolate mofetil) are
the commonly used as second-line therapeutic agents for refractory childhood nephrotic
syndrome. According to KDIGO 2012, treatment recommendation for SRNS are
calcineurin inhibitor (CNI) as initial therapy for children with SRNS (LoE 1B) for a
minimum of 6 months and then stopped if a partial or complete remission of proteiunuria is
not achieved (2C), and be contiunued for a minimum of 12 months when at least a partial
remission is achieved by 6 months with low dose corticosteroid theraphy be combined with
CNI theraphy. Additional to this regimens is ACEI or ARBs for children with SRNS.10ﾧ
These patients received ACEI and ARB therapy to control the blood pressure and to reduce
proteinuria. Hypertension in this patient has been improved with both agents. In addition to
medical treatment, this patient is also encouraged to modify lifestyles changes such as
having regular excercise, maintaining ideal body weight,and reducing salt consumption to
1.5g/day.11
Systematic review report that cyclosporin significantly increased the number with
complete or partial remission compared with IV cyclophosphamide (RR 3.40, 95% CI 1.12
to 10.28). There was no significant difference in the number who achieved complete
remission between oral cyclophosphamide with prednisone versus prednisone alone (RR
1.06, 95% CI 0.61 to 1.87), IV versus oral cyclophosphamide (RR 3.13, 95% CI 0.81 to
12.06), IV cyclophosphamide versus oral cyclophosphamide with IV dexamethasone (RR
1.13, 95% CI 0.65 to 1.96), tacrolimus versus cyclosporin (RR 0.86, 95% CI 0.44 to 1.66)

23
and azathioprine with prednisone versus prednisone alone (RR 0.94, 95% CI 0.15 to 5.84).
ACEi significantly reduced proteinuria.11 (Level of evidence 1)
Systematic review and metaanalysis found that treatment with mycophenolate
mofetil had the greatest odds of relapse compared with tacrolimus, cyclophosphamide or
cyclosporine. Rank probability analysis found that cyclophosphamide was the best
treatment with the lowest relapse rate as compared with other treatments, and tacrolimus
was ranked as the second best with respect to relapse.12ﾧ (Level of evidence 1) Another
systematic review and metaanalysis suggest that tacrolimus and cyclosporine may be
preferred initial treatments for children with SRNS. MMF may be another option for this
patient population.13
This patient is treated with sequential IV cyclophosphamid at a dose of 500-750
mg/m2/month for 7 times combine with prednisone 40 mg/m2 alternate dose for 6 months
and tappreing off for 2 months according to the protocol CPA for SRNS.3 A RCT with 50
subject conclude that this iv regiment have rates of induction of complete remission were
52% when versus oral regimen. Incidence of side effects (both major and minor) was 36%
in IVCP versus 20% in OCP group. The actuarial cumulative sustained remission was 12%
in IVCP compared with 16% in OCP at 1 year after completion of therapy.14 Side effects
of CPA administration are nausea, vomiting, bone marrow suppression, alopecia,
haemorrhagic cystitis, azospermia, and long term use might cause malignancy. Therefore it
is necessary to monitor complete blood count such as hemoglobin, leukocyte, platelet
counts 1-2 times a week. CPA administration should be omitted if the leukocyte count
drops below <3000 / μL, hemoglogin <8 g / dL, and platelet count <100,000 / μL; patient
might continue receiveng treatment after leukocytes count increased to > 5,000 / μL,
hemoglobin > 8 g / dL, platelet > 100,000 / μL.3 Laboratory evaluation of this patient
revealed Hb 12.1 g / dL, leukocytes 8,800 / μL, 252,000 / μL platelets 252,000/ µL. The
effect of CPA toxicity on gonad and malignancy occur if total cumulative dose reaches ≥
200 – 300 mg / KgBW. This patient will receive a total dose of 118 mg/Kg intravenous
CPA on 6 months period given in 7x administration; this total dose is within safe limit. 3ﾧ
Monitoring for haemorrhagic cystitis was performed by evaluation of urinalysis after 3 day
from IVCP that revealed no gross hematuria or hematuria microscopic.
Systemic corticosteroids are used for the treatment of nephrotic syndrome in initial
phase and maintenance phase. This patient was given oral prednison full dose, and now
alternate dose for 6 months. Despite the beneficial effects, long term systemic use of these
agents is associated with well-known adverse events (Aes) including: cushingoid feature
24
(19,4%), behavioral changes (8,1%), increase appetite (18,5%), weight gain (21,1%),
hypertension (5,6%), infection (8,7%), growth retardation (18,1%), gastrointestinal upset
(15,8%), hyperglycaemia (3,4%), hypercholesterolaemia, fatigue (2,0%), myopathy
(3,0%), insomnia (9,9%), headache (4,3%), cataracts (5,9%), gastric wall abnormalities
(10,4%), osteoporosis (0,8%), dizziness (4,3%), edema (0.8%), acne (10,8%), skin striae
(4,4%), hirsutism (12,1%), HPA axis suppression (58,6%), fever (3,2%), pigmentation,
rash (5,8%), and other rare advers event increase intraocular pressure, pancreatitis,
obstructive sleep apnoea, oral ulceration, hypocalcemia, hypokalemia. Glucocorticosteroid
(GC)-associated toxicity appears to be related to both the average dose and cumulative
duration of GC use. The adverse events of corticosteroid use should be monitored
regularly. Spesific recommendation for the assessment and monitoring of BMD and
fracture risk, diabetes, CV risk and dyslipidemia, adrenal supression, growth and
ophthalmologic event.15,16 ﾧ (Level of evidence 1) This patient had received high-dose
corticosteroid therapy for 1.5 months. Side effects encountered in the form of increased
appetite, cushingoid feature, skin striae, hypertension, and hypercholesterolemia. While
hypertension and hypercholesterolaemia are the symptomp of the nephrotic syndrome.
GCs have been shown to stimulate osteoclastic activity initially (first 6-12 months
of therapy). The use ≥ 5 mg/day of prednisolone (or equivalent) was associated with
significant reduction in bone mineral density (BMD) and increase in fracture risk within 3
to 6 months of treatment initiation. Prolonged corticosteroid therapy commonly causes
weigt gain and redistribution of adipose tissue that result in Cushingoid features. A survey
of 2.167 long term GC users (mean prednisone dose = 16 ± 14 mg/day for ≥ 60 days)
found weight gain to be the most common self-reported AE (70%). This patient have
truncal obesity, facial adipose tissue [i.e., moon face], and dorsocervical adipose tissue)
and also increase of appetite. This patient also have increase risk of hyperglichemia
substantially with increase risk daily steroid dose (OR: 1.77).17ﾧ (Level of evidence 2). In
children with Cushingoid feature, HPA-axis function should be evaluated. Other Aes were
metabolic syndrome in dose > 10 mg/day, that are known to be abdominal obesity with ≥ 2
parameters (hypertension, HDL ≤ 40 mg/dL, triglyserid ≥ 110 mg/dL, and fasting glucose
≥ 100 mg/dL). This patient has hyperlipidemia complication characterized by elevated total
cholesterol (344 mg/dL), LDL (217 mg/dL), and triglycerides (214 mg/dL) that have
atherogenic and thrombogenic effects; this condition might increase cardiovascular
morbidity and mortality, and aggravate progressivity of glomerulosclerosis. In SRNS it is
recommended to maintain a normal weight for height and eat a healthy diet low in
25
saturated fats before considering dyslipidemia medication such as statins. This patient’s
waist circumference is normal so it has not met the criteria for metabolic syndrome despite
having hypertension and hypertriglyceridemia. However, metabolic syndrome should be
monitored closely as a complication of long-term steroid use.
GC use is typically associated with the development of posterior subcapsular
cataracts. Time until onset is at least 1 year with dose ≥ 10 mg/day of oral prednisone.
Patient on low to moderate doses of systemic corticosteroids for more than 6-12 months
should undergo annual examination by an ophthalmologst. An earlier examination is
required in patients with blurred vision.16 ﾧ (Level of evidence 1) Eye examination
revealed no blurred vision, no cataract or glaucoma. This is probably due to duration of
therapy which is less than 6 months. Although there are currently no eye abnormalities, yet
the patient require periodic check ups every 3-6 months while on steroid treatment.
This patient also have increased risk of infection (RR: 1.6; 95% CI, 1.3 – 1.9; P <
0.001) in dose > 10 mg/dL. For children receiving GC therapy, growth should be
monitored every 6 months (ideally using stadiometry measurements) and measurements
should be plotted on an appropriate growth curve. If after 6 months, growth velocity
appears to be inadequate, ih should be consider all posible etiologies, including adrenal
suppression. It also important to rule out malnutrition as a cause of poor growth. Long term
GC in children with nephrotic syndrome leads to decreased growth for doses exceeding 0.2
mg/kg/day, and dose above 0.4 mg/kg/day may be deleterious on skeletal development.18ﾧ
(Level of evidence 3) This patient need intensive growth monitoring and plotting on
growth curve every 6 month while on prednisone AD treatment.
For the summary, we should to assessing and monitoring at the baseline before GC
theraphy for wight, height, BMI, blood pressure and investigate laboratory measurement
CBC, glucose (FPG, A1C, 2-h OGTT or casual PG), lipids (LDL,-C, HDL-C, TC, non-
HDL-C ± apo B) and BMD. For the subsequent monitoring we should monitore about
bone health (repeat BMD and lateral spine x-ray in children receiving ≥ 3 months of GC
therapy typically yearly if there is a persistence of risk factors), HPA-axis if > 2 consecutif
weeks or > 3 cumulative weeks in the last 6 months or has persistent symptoms of AS,
growth every 6 months and plot on growth curve, dyslipidemia and CV risk 1 month after
GC initiation, then every 6-12 months, hyperglycemia for at least 48 hours after GC
initiation then every 3-6 months for first year, and ophthalmology exammination and
intraocular presure assessment.16ﾧ (Level of evidence 1)

26
 In addition to medical therapy as mentioned above, patients also require balanced
diet and supportive treatment. A normal protein of 1.5-2 g / KgBW / day and adequate
calories is recommended for children with nephrotic syndrome to prevent growth
retardation; low salt diet (1-2 grams / day) is restricted to children suffering from edema.3
Nutritional requirements of this patient are as follows : calories 40 kcal/kgbw/day, protein
1 gram/kgbw/day. Total calories: 2,100 kcal/day, protein: 53 gr/day, fluid (Darrow): 2,160
ml/day. Patients were given 3x1 serving of rice (1,566 kcal), wholemilk 3x250 ml, IVFD
D5 ¼ NS 480 ml / 24 hours with total calories of 2,076 kcal, 63.4 gram protein (99%
calories and 119% protein requirements according to RDA). Patient is in
immunosuppressed condition due to CPA treatment long-term use of systemic
corticosteroids. Infection prevention and control programs should be kept in both the
hospital and at home. At this time the patient has multiple impacted teeth and multiple
necrotic pulp treated with Ampicillin injection 500 mg / 6 hours and teeth extraction
procedure to remove source of focal infection. On routine blood evaluation, the leukocyte
count has decreased from 17,900 / μL to 8,800 / μL after three days of antibiotics therapy.
All killed vaccines included in IDAI programme should be offered to these children
preferably while receiving alternate day prednisolone. Parents must be made aware that
live vaccines are contraindicated while on treatments with steroids.19ﾧ (Level of evidence
3) Live vaccines are contraindicated in children receiving high dose systemic steroids
(prednisolone 2 mg/Kg/day or 20 mg/day in children > 10 kg body weight) until the
steroids have been discontinued for 3 months.19 ﾧ (Level of evidence 3). The patient is
immunosupressed due to CPA and prednisone AD therapy. Immunization should be given 3
months after the completion of therapy.
Recent guidelines from both the American Academy of Pediatrics (AAP) and the
Advisory Committee on Imunization Practices (ACIP) regarding the use of varicella
vaccine in children on steroid therapy suggest that live attenuated varicella vaccine may be
safely given in children receiving prednisone less than 2 mg/kg per day or less than 20
mg/day in a child who wighs more than 10 kg. the ACIP states that short term, low to
moderate dose steroids and long term alternate day treatment with short acting preparations
usually do not contraindicate administration of live virus vaccine.20ﾧ
Children with SRNS generally have good survival rates, although during the course
of the disease they may develop decreased kidney function, leading to a condition where
the kidney function is not compatible with life, called endstage renal disease (ESRD). A

27
retrospective cohort study in Department of Child Health, Cipto Mangunkusumo Hospital
showed that live survival rates of subject at the first, second, third, fourth and fifth years
after diagnosis were 93%, 84%,80%,72% and 61% respectively. Kidney survival rates
determined by the lack of doubling of base creatinine levels at the first, second, third,
fourth and fifth years were 92%, 72%, 56%,42% and 34% respectively, while kidney
survival rates determined by the lack of ESRD were 97%, 88%, 81%,70% and 58%
respectively. Age onset, initial kidney function, hypertension at onset and type of
resistance, did not significantly affect the survival of children with SRNS.21ﾧ (Level of
evidence 3) The prognosis of the patients with SRNS is poor, with 30–40% developing
end-stage renal disease (ESRD) and requiring dialysis and transplantation.22 ﾧ (Level of
evidence 3) The patient is steroid-resistant nephrotic syndrome with adolescent onset and
normal GFR. However, the prognosis of SRNS patients is poor as 30-40% patients will
develop into ESRD and require dialysis and transplantation.
Disease characteristics inherent to nephrotic syndrome including edema, repeated
corticosteroid exposures, and the relapsing nature of the disease pose challenges to
patient’s HRQOL (Health Related Quality of Life).23ﾧ (Level of evidence 3)
The PROMIS (Patient Reported Outcomes Measurement Information System)
scores were significantly worse in prevalent patients compared to incident patients in the
domains of pain interference (p < 0.01) and peer relationships (p = 0.01). HRQOL in
children with active nephrotic syndrome, showing that children with prevalent disease have
a significantly worse HRQOL than those with incident disease.23ﾧ (Level of evidence 3)
Patients with MCNS (Minimal Change Nephrotic Syndrome) has a benign
prognosis, and QoL of patients with MCNS may be better than that of CKD patients.24ﾧ
(Level of evidence 3). In a study conducted by Dotis et al, in the 8- to 11- year age group
children with kidney disease had higher social acceptances scores than did those in the
validated sample of the greek population. On the contrary, in the at older age group (12 –
18 years), there was tendency towards a less good social acceptance in children with
kidney disease in comparison to the children in control group. This result demonstrates that
younger children with kidney disease do not feel intimidated, that they enjoy social
acceptance by others and that they are treated with respect, probably due to a greater
sensitivity for social relationships at these ages. Unfortunately, these findings are reversed
in older children.25ﾧ (Level of evidence 3) The onset of nephrotic syndrome in this patient
is started at age 16, socially unacceptable. Patient loss self-esteem because of his illness so

28
the child chose to drop out of school. To deal with the issue, the child is consulted to
division of developmental and growth pediatrics.

Comprehensive management
a. Curative:
Treatment is based on SRNS protocol, using CPA and AD prednisone for 6 months. Blood
pressure control is implemented by giving antihypertensive agents ACE-I and ARB. Teeth
extraction procedures to remove necrotic pulp.
b. Preventive:
Current treatment emphasized on prevention of the development of end stage renal disease,
risk of hospital malnutrition, and risk of infection.
c. Promotive:
Providing education about personal hygiene, healthy lifestyle, and balanced diet to the
patients and the family. Vaccinations should be delayed during the immunosuppressive
treatment.
d. Rehabilitative:
Rehabilitation is focused on physical, emotional and social aspects. Supporting parent and the
child to cope with the illness and to complete the treatment, encourage the child to re-
associate with his peers and to continue his education, and to face the upcoming days. Other
rehabilitative treatments is according to recommendations from division of developmental
and behavioral pediatrics.

Holistic management
The ecology system of child basic needs
a. Micro environment (the child)
This patient had impaired physical health functioning including SRNS, hypertension, necrotic
pulp and impaired school functioning.
b. Mini environment:
The fulfillment of child basic needs: love, care, and stimulation by the parents and all family
members.
Family, including parents support in maintaining patient compliance to treatment will help
improving the child health.
c. Meso environment:
Community, school environment, peer group, and health care providers play important role in
supporting the ongoing long term treatment of the child.
d. Macro environment:
The medical cost of the family is covered by national health insurance.

29
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histopathological spectrum in adolescent onset nephrotic syndrome in a
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