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SYSTEMIC LUPUS ERYTHEMATOSUS (M32.

9), CHRONIC
KIDNEY DISEASE STAGE V (N18.5) DUE TO LUPUS
NEPHRITIS (N08.5), URINARY TRACT INFECTION (N39.0),
AND WELL NOURISHED.

Sri Wahyuni Djoko

POSTGRADUATE PEDIATRIC TRAINING PROGRAMME


DEPARTMENT OF CHILD HEALTH FACULTY OF MEDICINE UNUD/SANGLAH HOSPITAL DENPASAR
Ausgust 19TH 2019
Patient Identity
Case Parents

Father Mother
Name : IMAS
GD NMO
Age of case determination : 7 years 11 months old
Date of birth : September 15th, 2011 38 years old 38 years old
Sex : Male
Senior High Junior High
Address : Indonesia
Date of admission : August 4th, 2019 School School
Date of case determination : August 8th, 2019
Medical record number : 19035650 Tour Driver Traders
SUBJECTIVE
History of Present Illness
Chief complaint: pale

• Patient was complained pale since 4 months before admission Pale was found in face and all
of extremities. And experienced fatigue characterized by decreased of physical activities
and loss of appetite. History of transfusion administration twice was done at K State
Hospital.
• Joint stiffness, on the hip joint, knee and ankle joint accompanied with pain and was not
accompanied by joint swelling, warm sensation and painful on touch. The joint pain is
felt more profuse during night time, early morning, or when the weather is cold. He took
painkiller (paracetamol) everytime the pain comes, which helped reduce the pain. History of
physical trauma before illness was denied.
• Seizures twice before admitted to Sanglah hospital
History of Present Illness
SUBJECTIVE
• The first seizure occured on August 1st 2019, at home, for 3-4 minutes. The seizure
noted as stiffness of arms and legs, clenched jaws, eyelids were blinking rhythmically.
Stopped by itself, and afterwards patient fell asleep.
• The second and the third seizure occured with same duration and characteristics with
the previous seizure.
• Shortness of breath was noticed since August 1st 2019, his breath was said to be deep
and fast.
• vomiting since approximately 2 weeks before admitted to hospital, which occured
everytime he ate. The vomit consisted of the food he had eaten, were not projectile. He
also felt nauseous almost every time.
• Darkened skin which also felt stiff and crackling, and peeling since 6 months ago. It
started first as reddish of skin everytime he played outside. The reddish skin then
continued with multiple blisters, which dried out and left a scar with whitish concave
patch with dark edges.
History of Past Illness and Medication

• On July 13-18th 2019  admitted to K State Hospital , with paleness and received
red blood transfusion twice. Hemoglobin before transfusion was 7.24 g/dL,
thrombocyte was 13.4. He was then discharged.
• On August 1st 2019,  admitted to BM K Hospital due to shortness of breath,
paleness, and vomiting. On August 4th, 2019, a lab examination was performed,
and results were: hemoglobin 9.9, thrombocyte 72, ureum 355.6, creatinine 11.63,
potassium 4.64, sodium 117.8, calcium 6.29, chloride 91.54  patient was said
to have decrease of kidney function, and therefore was referred to Sanglah
Hospital.
Family Medical History
Social History
• Patient was born from a 31-year-old mother with full term
pregnancy.
Prenatal history • Mother had routine antenatal care with doctor more than 3 times
during pregnancy. Mother only took vitamin supplement given by
doctor, without any other medications. She had normal blood pressure.
There was no X-ray
• exposure, no history of fever, rash, and other illness during pregnancy.
• Conclusion: no problem during pregnancy.

• Patient was born spontaneously assisted by doctor at private clinic.


Intranatal history • He was vigorous and his body weight was 3500 gram. He was given
vitamin K and Hepatitis B immunization.
• There was no history of shortness of breath, bluish of lips and
tounge, and yellowish color of skin.
• Conclusion: no problem during delivery
Social History
Postnatal history • Patient was breastfed immediately after birth. No history of
hospitalization.
• Conclusion: no problem during postnatal period.

• Patient was breastfed until 18 months and did not consume


formula milk since born.
• Patient’s daily food combination consist of: rice, pork, fish, eggs,
and vegetables. No food allergy was reported.
• Before admitted to Sanglah Hospital, patient consumed half
Nutritional history a portion of rice porridge, half piece of chicken, two sticks of
pork satay, and half an apple. Total calorie and protein intake for
24 hours were 637.5 kcal and 24 grams of protein. Patient daily
intake only fulfilled 32% of energy requirement according to
recommended dietary allowanced (RDA).
• Conclusion: nutritional intake is considered insufficient
Social History
• Patient’s body weight increased every month during the first year of
life.
• Patient plays often with her friend outside the house at
Growth history and daytime.
develomental history • His development is similar to his peers. However, he does not
measure his body weight regularly. His body weight was measured
at BM K Hospital, and was 20 kg.
Conclusion: his growth and development had appropriate for
age.
• Parents said that patient got the immunizations completely
according to the schedule by Ministry of Health in primary
health care.
Immunization history • They also stated that patient also had two other immunizations
in elementary school (Japanese encephalitis and measles-
rubella). BCG scar was found on the patient's right arm
Conclusion: complete immunization.
Basic Needs History
 Patient received sufficient stimulation from parents. He was able to
Stimulation get along well with his peers.

 Patient is the second child of the family and was conceived from a
happy marriage. He was loved and supported by both parents, even
Parenting though both parents work. Affection was also given from his
grandmother and aunt .

 Patient was breastfed until 18 months. Immunization was received at primary


health care. Health care was received from a midwife & doctors from
Caring primary health care and hospital.
 He lives with his parents in a permanent house.

Conclusion: Basic needs were fulfilled.


Family Socio-economic Condition
History during admission until determination as case
On the first-two days of hospitalization (August 4 th– 5th, 2019)
Laboratory evaluation:
Planning therapy:
S : paleness,
Leucocytes
Fluid was
requirementshortness wasof/µL
7.78x10 3
breath,
1500 nausea
(neutrophils
mL/day 6.49andx10
fulfilled vomiting.
orally, Fever and
calorie wasprotein
not present. His urinewere
3/µL (83.52%); lymphocytes 1.18 x103/µL (15.9%),
requirements
monocyte
color was 0.07 (0.96%), hemoglobin
appropriate whitish
with like heated
renal end stage is11.27
egg white mg/dL;
2000and frothy.
kcal/day mean corpuscular volume (MCV) 76.05 fL; mean
and 25 grams/day (given as meals three
corpuscular hemoglobin (MCH) 25.25 pg; mean corpuscular hemoglobin concentration (MCHC) 33.20 g/dL,
O : Physical
times examination:
daily33.95%;
and snacks
haematocrit platelet twice daily,
59.05.7x10 and
3/µL, ureummilk213.40
2 glasses
mg/dL,percreatinine
day), cito haemodyalisis,
16.42 mg/dL, sodiumand 119
th
Present
consult status:
to alert
Thoracic with
and moderately
cardiovascular ill, blood
surgerypressure
mmol/L, potassium 6.18 mmol/L, chloride 81.0 mmol/L, calcium 5,2 mg/dL. was
department 130/90-150/100
for insertionmmHg
of (>99
double
percentile),
lumen pulse Captopril
catheter, rate 80-100 beats/minute,
0,3systemic
mg/kgBW/dose respiratory
equal torate
lupus erytematosus 6 22-24
mg everytimes/minutes,
8 hours, body and
temperature
Urinalisis
hyponatremiashowed: 36,6-37°C, andjuvenile
protein +3, blood
correction.
differential diagnose peripheral
+2, glucose +1.oxygen saturation ranged between 97-98% in
disconnective tissue, acute kidney injury stage
room air. failure, hypertension grade II, electrolyte imbalance (hyponatremia,
Blood gas analysis showed Metabolic acidosis pH 7.10; PCO2 19.9 mmHg; PO2 203.20 mmHg; BEecf -23.8
General status:
Planning no palpebral
monitoring: edema, no malar
hypocalcemia) rash, there was multiple limphadenopathy in
mmol/L; HCO3 -6.00; SO2c 99%; TCO2 6.60 mmol/L. and wellreticulocyte
Corrected nourished. was 0.3,
-submandibular
Check for anti-ds dextra et sinistra
DNA, with sized
blood smear, ANA 0,5-1IF, ANA cm, mobileand
profile, and sharp edge. His thorax was
C3.
-symmetricalLupus on
Consulted
Systemic to static and
cardiology
International dynamic. Heart
division Clinics
Collaborating for and 2012
lungfulfilled
echocardiography.
(SLICC) examination
5 criteria was normal. Abdominal
(acute/subacute rash 1, joint
examination
-involvement
Monitoring 1, normal.
for thisNo≥3
proteinuria peripheral edema.
1, thrombocytopenia
patient includes vital1,Evaluation
lymphocyte
sign, SLEDAIof Pediatric
< 1500 Gait, Arm,
1). pGALs,
score, Leg, Spine
measuring body
(pGALS)
weight, showed
mid uppermild difficulty. Abdominal (MUAC),
arm circumference circumferencewaist was 51 cm. His body
circumference, weight
and fluid (BW)
balance
SLEDAI score was 9.
was 20 kg,
every body height (BH) was 120 cm, ideal body weight was 22 kg, and nutritional status
day.
based on waterlow was 91% (well nourished).
History during admission until determination as case

On the therapy:
Planning
Laboratory third and fourth day of hospitalization (June 6-7th, 2019)
evaluation:
•Blood
S :High
paleness.
smear:
dose methylprednisolon
Eritrocyte; normochromic
30 mg/kg,normositter
~ 600 mgpoikilositosis
in 250 ml NaCl
(burr0.9%
cell, inovalocyte,
4 hours
O (intravenously),
: Physical examination:
fragmentocyte). Leukocyte; normal, relative netrophilia. Trombhocyte; decrease, no
•clumping,
Present status:
Ranitidine blood
no 1giant
mg/kg ~ pressure
platelet.
20 mg in decreased
Phospat
20 ml
7.7.NaClto0.9%
Ureum 110/70
and
in 20- 130/90
creatinin
minutes,mmHg
after
given (90-P99th
hemodyalisis percentile),
30-60 minutes was 82.10
prior
pulse
mg/dL rate
to high 100-112
anddose beats/minute,
Systemic respiratory
7.79methylprednisolone,
mg/dL. rate 22-24Collaborating
Lupus International times/minutes, body (SLICC)
Clinics temperature
2012
•fulfilled
range
Oral between
captopril
6 criteria36.7-36.9 0C, and peripheral oxygen saturation range between 98-99% in
0,3(acute/subacute
mg/kgBW/dose equal
rash to1,6 mg
joint
every
involvement
8 hours. 1, proteinuria ≥3 1,
systemic lupus erythematosus, lupus
•thrombocytopenia
room
Bloodair.sugar and 1, blood
lymphocyte
pressure <was1500monitored
1, haemolytic
before anemia
and after
1) administration
and SLEDAI score of high
was
nephritis stage IV-V, hypertension grade II, electrolyte imbalance (improved)
General
11. status:
dose methylprednisolone. He was also monitored for SLEDAI score and fluid balance daily.
and well nourished.
pale on conjunctiva and mouth, no palpebral edema, no malar rash, there was multiple
limphadenopathy
Radiologic in submandibular dextra et sinistra with sized 0,5-1 cm, mobile and
evaluation:
sharp
His edge.
chest Hisevaluation
X-ray thorax wasrevealed
symmetrical
normalonpulmonary
static and dynamic. Heart
and cardiac. and lung examination
Electrocardiography was
is normal.
normal (noAbdominal
PR interval examination
prolongation).normal. No peripheral
Echocardiography edema.
revealed noEvaluation
pericardialof effusion,
Pediatric
Gait,
no Arm, effusion,
pleural Leg, Spine mild(pGALS) showed
tricuspid mild difficulty. Abdominal circumference was 51 cm.
regurgitation.
His body weight (BW) was 20 kg, body height (BH) was 120 cm, ideal body weight was 22 kg,
and nutritional status based on waterlow was 91% (well nourished).
Physical examination (Objective) on August 9th, 2019
PRESENT STATUS

General condition : moderately ill


Level of consciousness: E4V5M6 15/15 (Compos mentis)
Blood pressure : 140/90 mmHg (>99thpercentile)
50 th percentile ~ 99/59 mmHg
90 th percentile ~ 113/73 mmHg
95 th percentile ~ 116/77 mmHg
99 th percentile ~ 124/84 mmHg
Pulse rate : 98 beats/minutes, regular, sufficient volume
Respiration rate : 24 times/minutes, regular
Oxygen saturation : 99% in room air
Axila temperature : 36.9°C
Pain scale (NRS) : 2 (mild pain)
Physical examination (Objective) on July 8th, 2019
GENERAL STATUS
Head : normocephaly, black hair, not easily plucked, no alopecia.
Face : no abnormality, no malar rash, no syndromic facies.
Eye : pale conjunctiva, palpebral edema, no sunken eyes, symmetrical eyelids, no
icteric sclera, round pupils with diameter of 3 mm and good light reflexes, no deviation
conjugee neither strabismus.
Ear : normal shape, no secretion.
Nose : no nostril flare, no secrete, no bleeding neither septal deviation.
Throat : no hyperemic in pharynx and tonsil T1/T1 no hyperemic.
Mouth : no cyanosis on surrounding lips, tongue and gum, symmetrical corners of
the mouth, no drooling, no tongue enlargement, no white plaque on tongue and
mouth, no mucosa ulcer.
Neck : no neck stiffness, there was enlargement of left submandibular lymph
nodes with diameters of 1.0 cm and right submandibular lymph nodes with the sizes
of 0.5-1.0 cm, mobile, and sharp edges.
Physical examination (Objective) on July 8th, 2019
GENERAL STATUS
Chest
Heart :
Inspection : no visible precordial bulging and ictus cordis
Palpation : ictus cordis was palpable in left midclavicular line on fourth intercostals
space without thrill, LV lift and RV heave were not palpable.
Auscultation : normal heart sounds, regular, M1>T1, A2>P2, no murmur, no friction-rub.
Lung :
Inspection : normal chest shape, no pectus excavatum, no pectus carinatum,
symmetrical on static and dynamic, no subcostal retraction.
Palpation : symmetrical chest movement. Normal vocal fremitus in lower both
sides.
Percusion : sonor in both sides.
Auscultation : vesicular breath sound on right and left lung, without rales and wheezing,
no friction-rub.
Physical examination (Objective) on July 8th, 2019
GENERAL STATUS
Abdomen
Inspection : minimal distention, superficial vein not visible
Auscultation : normal peristaltic sound
Palpation : liver and spleen were not palpable, no tenderness, No costovertebral angle
or tenderness.
Percusion : tymphanic, undulation-test negative, shifting dullness negative, puddle sign
positive, waist circumference of 51 cm.
Inguinal : no enlargement of inguinal lymph nodes.
Genital : no pubic hair, oedema on pubis and scrotum.
Limbs
Upper : oedema on dorsum manus, no cyanosis on fingers, palms were pale, warm on
palpation, capillary refill time was 1 second.
Lower : pitting edema was found on both of lower limbs and dorsum pedis, no
strechmark was found, no cyanosis on fingers, plantar was pale, warm on
palpation, capillary refill time 1 second.
Skin : pale, no cutis marmorata, no maculopapular rash, there is scar with whitish
concave patch and dark edges on the trunk
Physical examination (Objective) on July 8th, 2019
NEUROLOGICAL EXAMINATION
Meningeal signs
Neck stiffness : negative.
Kernig sign : negative.
Brudzinski I sign: negative.
Brudzinski II sign: negative.

Cranial nerves
CN I, II : no olfactory and visual disorders.
CN III, IV, VI : no palpebral retraction, no ptosis, pupil reflects +/+ equally, normal eyes
movement.
CN V : touch, pain, and temperature sense, and jaw jerk reflex still normal.
CN VII : equal nasolabial folds, symmetric facial movement.
CN VIII : no hearing disorders.
CN IX, X : swallowing reflect (+).
CN XI, XII : no atrophy, asymmetry and fasciculation of the tongue.
Physical examination (Objective) on July 8th, 2019
NEUROLOGICAL EXAMINATION
Motoric system and extremities reflexes
Upper Right Left
Power 555 555
Tone normal normal
Trophy normal normal
Physiologic reflexes
Biseps ++ ++
Triceps ++ ++
Lower Right Left
Power 555 555
Tone normal normal
Trophy normal normal
Physiologic reflexes
APR ++ ++
KPR ++ ++
Pathologic reflexes
Babinski - -
Involuntary movement - -
Physical examination (Objective) on July 8th, 2019
PUBERTAL STATUS
Genitalia : no sign of puberty
Pubic hair : no pubic hair
Appropriate with G1P1 pubertal status according to Tanner stage I)
ANTHROPOMETRIC STATUS
Weight, weight for age : 25 kg (with edema), P50thpercentile
Height, height for age : 120 cm, 10th - 25thpercentile
Weight for height : 85th - 90thpercentile
Ideal body weight : 22 kg
MUAC : 18.2 cm
Standard MUAC : 18.7 cm
Nutritional status (MUAC): 90% (well nourished)
Father height : 165 cm
Mother height : 165 cm
Genetic height potential : 163-180 cm, Mid Pareteral Height is 171.5 cm, body height is in
normal range from his genetic height potential).
Physical examination (Objective) on July 8th, 2019
DEVELOPMENTAL STATUS

Pediatric Symptom Checklist (PSC 17): total score: 3.


Conclusion : no behavior disturbance.
PedsQL score : mean PedsQL score for patient 58.69; mean PedsQL score for parents 53.26.
Conclusion : there were impairment of quality of life.
RESUME
A male patient, 7 years 11 months old, complained pale sinced 4 months before admission,
accompanied by fatigue and joint stiffness. History of transfusion administration twice was done at K
State Hospital. Complaint of seizures twice before admitted. Shortness of breath was noticed since
August 1st2019, breath was deep and fast. Patient also complained of vomiting since approximately
2 weeks before admitted to hospital. Eyes got easily tired and painful since 2 weeks whenever he
was exposed to prolonged light. He enjoyed drinking bottled tea every day.

Physical examination revealed moderately ill appearance, blood pressure was over percentile 99
mmHg. Palpebral edema and pitting edema on both of lower limbs.

Blood smear is appropriate for hemolytic anemia. GFR was reduced > 50%
(6.01ml/min/1.73m2), Urine analysis revealed proteinuria. Blood gas analysis showed metabolic
acidosis. Coomb’s test was negative. Calsium 5.2 mg/dL, Phospat 7.7 mg/dL. Chest X-ray revealed
normal pulmonary and cardiac. Echocardiography revealed no pericardial effusion, no pleural
effusion, mild tricuspid regurgitation. SLICC fulfilled 6 criteria, SLEDAI score 11. Patient began to
receive pulse dose methylprednisolone for three consecutive days accompanied by captopril as
an oral antihypertensive.
DIAGNOSIS

Systemic lupus erythematosus (M32.1), Chronic kidney


disease stage V (N18.5) due to Lupus nephritis (N08.5),
Urinary tract infection (N93.0) and well nourished
Problems
Planning

Emergency management
Currently this patient is in stable hemodynamic. There is no emergency
in this patient

Planning of diagnosis
• Diagnostic planning of this patient includes examination anti-
dsDNA, Coomb’s test, ANA IF, ANA profile, C3, Parathyroid hormone,
Vitamin D, and renal USG.
Planning
Systemic lupus erythematosus treatment

• pulse dose methylprednisolone 30 mg/kgBW/dose equal to 600 mg


in 250 ml of NaCL 0.9% in 4 hours intravenously, administered for 3
days and
• continued with metylprednisolone 1 mg/kgBW/day equivalent 10
mg every 12 hour for 4 weeks.
• cyclofosfamide 500 mg in 250 ml NaCl 0.9% in 4 hour (intravenously)
after 3 days high dose of metylprednisolone and continue every 2
weeks for the first three months.
• Monitoring of recurrent symptoms by using SLEDAI scoring system.
Planning
Hypertension grade II management

Hypertension is treated with antihypertensive oral captopril 1.8


mg/kgBW equivalent to 12.5 mg every 8 hours with a target of
blood pressure decreased within 50th – 90th percentile range.
Planning
Acute kidney injury stage failure management

Hemodialysis was performed on the patient. The patient will get


hemodialysis again after three days of cyclophosphamide are given.
Monitoring of kidney function is done by examining blood ureum
and creatinine and also fluid balance after hemodialysis is done.
Planning
Pediatric Nutritional Care
• Nutritional assessment: well nourished
• Nutritional requirement: Energy requirement according to RDA.
Total calorie requirement is acording to end stage renal
disease is 100 kcal/kgBW/day which equals to 2000 kcals/day,
protein requirement is 1.1-1.5 gram/kgBW/day which equals to 25
grams/day, fluid requirement 1500 ml/day.
• Nutritional route: oral route.
• Nutritional selection: he is given 3 portion of rice (1300 kcals),
with 2 portions of snacks (180 kcals), 2 glasses of milk (520 kcals).
• Nutritional monitoring: intake, tolerance, accuration, vomiting,
diarrhea, fluid balance and weight gain.
Planning
Planning monitoring
• Monitoring vital signs (blood pressure, respiratory rate, heart rate,
axillary temperature), were monitored every 3-4 hours.
• Monitoring side effect of medication such as hypertension, arithmia,
electrolyte imbalance, psychosis, gastrointestinal disturbance, or infection
were done within therapy period
• Monitoring of nutritional status and general condition were done
everyday during treatment in hospital.
• Monitoring of SLEDAI score and pGALS screening.
• Monitoring of BUN, creatinine and urinalysis evaluation every 3 days.
• Monitoring blood sugar before and after metylprednisolone pulse
dose administration
Follow Up After Case Determination
Day 1-3 (August 10-12th, 2019)
S : Fever up and down, shortness of breath , no headache, slightly distention of stomach, yellowish urine, 100-
200 ml/times in volume, frequency 4-5 per day, decreased of appetite

Present status General status


General condition : moderately ill Eye: pale conjunctiva (+), edema palpebral (+)
Consciousness : alert Mouth : Oral ulcer (-)
Blood pressure : 110/70-130/90 mmHg (>p95-99) Neck : enlargement of lymph nodes submandibulla
Pulse rate : 84-100 beats/minute, regular regio sinistra with size 1x1cm, region dextra 0,5-1cm
Respiratory rate : 22 times/minute, regular Lung: normal vocal phremitus, vesicular, no rales and
Temperature : 36.8-39,6oC wheezing
Waist circumference : 53 cm Heart: normal heart sound, no friction rub nor
Pain scale (NRS) : 2 murmur. Abdomen: abdominal distension (+), shifting
Fall risk (Humpty-Dumpty) : 7 dullness (+), puddle sign (+), tenderness (-)
Anthropometric status Extremities: pitting edema (+) Skin: rash (-), scar (+),
BW 25 kg dark skin (+)
MUAC 18.2 cm
Nutritional status based on MUAC 90%
Follow Up After Case Determination
Laboratory results Na 137mmol/L
Blood count K 4.85mmol/L
Leucocyte 6.62 x103/µL Cl 96.6mmol/L
Neu% 74.62 x103/µL (#4.94)
Lymp 20.50 x103/µL (#1.36) Cumulative fluid balance : -18,5 ml
Hb 7.78 g/dL Urine production : 1.9-2 ml/kg/hour
Mcv 75.75 fL
Mch 25.63 pg SLEDAI score: 9
Hct 22.98%
Plt 74.08 x103/µL

BUN 59.10 mg/dL


Creatinine 5.22 mg/dL
GFR 12.64 ml/min/1.73m2
Follow Up After Case Determination
Systemic lupus erythematosus (M32.1), Chronic kidney disease stage V (N18.5) due to Lupus nephritis
(N08.5), Urinary tract infection (N93.0) and well nourished

Planning therapy Planning diagnostic


- Fluid requirement 1500 ml/day - able to drink 250 • Planning for PTH, Vitamin D examination
ml ~ 1250 ml/day ~ aff infus, use stopper. (August, 10th 2019)
- Calories requirement 2000 kcal/day, protein • Urology ultrasonography examination
(August,12th 2019)
requirement 25 gr/day  use 50% of RDA calories is
Planning monitoring
1000kcal/day, and protein 24 gr/day • Vital signs, SLEDAI - body weight - fluid balance
- Low salt diet 3 x meals (750 kcal) 2 x snacks (100
kcal) 2 x 100 ml milk (total calories 50% of RDA)
- Intravenous Metylprednisolone 1mg/kgBW/dose
~ 10mg every 12 hour intravenous
- Captopril 0.75 mg/kgBW/dose ~ 12.5 mg every 8
hour oral
Follow Up After Case Determination
Day 4-5 (August 13-14th, 2019)
S : Fever up and down 4-5 times daily, no shortness of breath, no headache, slightly distention of stomach
yellowish urine, 100-200 ml/times in volume, frequency 4-5 per day, decreased of appetite

Present status General status


General condition : moderately ill Eye: pale conjunctiva (+), edema palpebral (+)
Consciousness : alert Mouth : Oral ulcer (-)
Blood pressure : 130/90-150/100 mmHg (p95-99) Neck : enlargement of lymph nodes submandibulla
Pulse rate : 86-100 beats/minute, regular regio sinistra with size 1x1cm, region dextra 0,5-1cm
Respiratory rate : 24-26 times/minute, regular Lung: normal vocal phremitus, vesicular, no rales and
Temperature : 37.0-39.5oC wheezing
Pain scale (NRS) : 3 Heart: normal heart sound, no friction rub nor
Fall risk (Humpty-Dumpty) : 9 (low risk to fall) murmur.
Abdominal circumference : 58 cm Abdomen: abdominal distension (+), shifting dullness
(+), puddle sign (+), tenderness (-)
Anthropometric status
Extremities: pitting edema (+) Skin: rash (-), scar (+),
Anthropometric status BW 23 kg
darkskin (+)
MUAC 18.2 cm, nutritional status based on MUAC 90%
Follow Up After Case Determination
Laboratory results Alb 3.30g/dL
Blood count Total cholesterol 174mg/dL,
Leucocyte 6.62 x103/µL LDL 102mg/dL, HDL 52mg/dL
Neu% 74.62 x103/µL (#4.94) PTH 458.9 pg/mL (high)
Lymp 20.50 x103/µL (#1.36) Vitamin D25(OH) 10.2 ng/mL (low)
Hb 7.78 g/dL Total cholesterol 174 mg/dL
Mcv 75.75 fL Na 137mmol/L,
Mch 25.63 pg K 4.85mmol/L, Cl 96.6mmol/L
Hct 22.98% Ca 6.2mmol/L.
Plt 74.08 x103/µL
Cumulative fluid balance : 220 ml
BUN 59.10 mg/dL Urine production : 1.6-1.8 ml/kg/hour
Creatinine 5.22 mg/dL SLEDAI score: 9
GFR 12.64 ml/min/1.73m2
Follow Up After Case Determination
Systemic lupus erythematosus (M32.1), Chronic kidney disease stage V (N18.5) due to Lupus nephritis
(N08.5), Urinary tract infection (N93.0) and well nourished
Planning therapy
- Fluid requirement 1500 ml/day - able to drink 250 ml ~
1250 ml/day ~ aff infus,use stopper.
Planning diagnostic
- Calories requirement 2000 kcal/day, protein requirement • Waiting for urine culture and blood culture
25 gr/day  use 75% of RDA calories is 1500 kcal/day, and result
protein 24 gr/day. Planning monitoring
- Low sodium diet 3 x meals (990 kcal) 2 x snacks (300 • Vital signs
kcal) 2 x 150 ml milk (total calories 75% of RDA) • SLEDAI
- Intravenous Metylprednisolone 1mg/kgBW/dose ~ 10mg • Body weight
every 12 hour intravenous • Fluid balance
- Captopril was continued
- Amilodipine 12.5 mg daily - CaCO3
53mg/kgBW/day~250mg every 8 hours oral.
- Paracetamol 10-15 mg/kgBW/dose~ 250 mg ~ ½ tablet
every 4 hours (if body temperature > 38.5oC or pain scale
≥4
- Calcitriol 0.25mcg/dose, two times daily
Follow Up After Case Determination
Day 6-7 (August 15rd-16th , 2019)
S : Fever all day up and down with antipyretic, Nno shortness of breath, no headache, no distention of stomach
yellowish urine, 100-200 ml/times in volume frequency 4-5 per day, increased of appetite

Present status General status


General condition : moderately ill Eye: pale conjunctiva (+), edema palpebral (+)
Consciousness : alert Mouth : Oral ulcer (-)
Blood pressure : 110/90-130/100 mmHg (p95-99) Neck : enlargement of lymph nodes submandibulla
Pulse rate : 98-100 beats/minute, regular regio sinistra with size 1x1cm, region dextra 0,5-1cm
Respiratory rate : 22-24 times/minute, regular Lung: normal vocal phremitus, vesicular, no rales and
Temperature : 36.7-39.9oC wheezing
Pain scale (NRS) : 2 Heart: normal heart sound, no friction rub nor
Fall risk (Humpty-Dumpty) : 9 (low risk to fall) murmur
Abdominal circumference : 52 cm Abdomen: abdominal distension (-), shifting dullness
(-), puddle sign (-), tenderness (-)
Anthropometric status
Extremities: pitting edema (+) Skin: rash (-), scar (+),
Anthropometric status BW 23 kg
dark skin (+)
MUAC 18.2 cm, nutritional status based on MUAC 90%
Follow Up After Case Determination
Laboratory results Sodium 129 mmol/L
Blood count Potassium 4.20 mmol/L
Leucocyte 4.3 x103/µL Cloride 87.5 mmol/L
Neu% 79.36 x103/µL (#3.42) Calsium 7.1 mg/dl.
Lymp 5.76 x103/µL (#0.25)
Hb 8.97 g/dL Cumulative fluid balance : 25.4 ml
Mcv 81.38 fL Urine production : 1.4-2 ml/kg/hour
Mch 27.65 pg
Hct 26.38% SLEDAI score: 10
Plt 63.43 x103/µL

Urine culture: Acitenobacter baumani, entobacter Consult to tropic infection division  approve of
cloacae (sensitive to cefoperazone sulbactam or antibiotics administration and join in patient care
cefepime)  evaluate urine culture again

BUN 36.9 mg/dL, Creatinine 3.52 mg/dL


GFR 18.75 ml/min/1.73m2 (decreased 49.3 %).
Follow Up After Case Determination
Systemic lupus erythematosus (M32.1), Chronic kidney disease stage V (N18.5) due to Lupus nephritis
(N08.5), Urinary tract infection (N93.0) and well nourished
Planning therapy
- Fluid requirement 1500 ml/day - able to drink 250 ml ~ Planning diagnostic
1250 ml/day ~ stop iv fluid, use stopper.
• Planning for percutaneous renal biopsy - Waiting
for blood culture, and gram tes.
- Calories requirement 2000 kcal/day, protein requirement
Planning monitoring
25 gr/day  use 80% of RDA  1600 kcal/day Low salt
• Vital signs, SLEDAI
diet 3 x meals (1000 kcal) 2 x snacks (400 kcal) 2 x 100 ml
• Body weight
milk (total calories 90% of RDA)
• Fluid balance
- Intravenous Methylprednisolone 1mg/kgBW/dose ~
10mg every 12 hour intravenous
- Captopril was continued
- Amilodipine 12.5 mg daily
- CaCO3 53mg/kgBW/day~250 mg every 8 hours oral.
- Paracetamol 10-15 mg/kgBW/dose~ 250 mg ~ ½ tablet
every 4 hours (if body temperature > 38.5oC or pain scale
≥4
- Calcitriol 0.25mcg/dose, two times daily - Cefoperazone
Sulbactam 50mg/kg/dose ~1 gram every 8 h
Follow Up After Case Determination
Communication, information, and education plan
1. Explanation about the disease, treatment plan and length of treatment
given, and prognosis of the disease.
2. Sanitation and personal hygiene of SLE when patient undergoing
corticosteroid therapy
3. Avoid triggering factors
4. Monitor the possibility that the patient may experience complications due
to the disease, long term monitoring of the progression of treatment,
monitoring of patient’s growth and development
5. Systemic Lupus Erythematosus is categorized as a chronic disease with a
high risk of developing psychopathology, most commonly manifests as
depression and anxiety
Prognosis
Ad vitam Ad functionam Ad sanactionam

Patient possibility have Patients with systemic


Patient with complication of steroid lupus erythematosus
complications in vital treatment that include are at risk of flare or
organs, especially the skin discoloration, recurrence. Therefore,
kidneys. Patient have weight change, monitoring clinical
decreased glomerular myopathy, cataracts symptoms, laboratory
filtration rate and and glaucoma and examination and
hypertension, resulting increases the risk of medication adherence
in poor prognosis.. diabetes and should be done
hypertension. regurally.

DUBIUS AD BONAM DUBIUS AD DUBIA DUBIUS AD DUBIA


SCHEME
OF
ILLNESS
HISTORY
SCHEME
OF
CASE

ANALYSIS
Case Analysis

Apnea of prematurity is a condition


where the breath stopped for more Management of AOP includes the use
APNEA OF
than 20 seconds, associated with
the presence of bradycardia or
PREMATURITY
of methylxhantine and Continous
Positive Airway Pressure (CPAP)
cyanosis in infants with gestational support.
age less than 37 weeks.

In this case, the patient had AOP on 12nd


day of treatment and received intravenous
aminophylline therapy
Aminophylline (loadingusedose continued
is the medication
with maintenance dose).
for treating apnea Aminophylline was well
of prematurity
toleratedand(nofacilitating
sign ofendotracheal
tachycardia, vomiting nor
extubation in preterm infants
convulsion) were found in the patient.
Case Analysis

Preterm was defined by babies born alive


before 37 weeks pregnancy are completed. Low birth weight infant into 3 categories, low
There are sub-categories of preterm birth weight (LBW) infant if the birth weight
birth (900) , Very
Extremely Low Birth Weight less
Preterm
than 2500
Infant
grams, very
(28birth
low
weeks)
weight
based on gestational age, moderate-late
preterm (32 to <37 weeks), very preterm (28 (VLBW) infant if the birth weight less than
to <32 weeks), and extremely preterm (<28 1500 grams, and extremely low birth weight
weeks), with In this case,
decreasing patient
gestation agewas
at born preterm with
(ELBW) anbirth
if the estimated
weight less than 1000
gestational
birth associated agerisk
with increased ofof28 weeks and extremely lowgrams birth
weight (900 grams). The patient is appropriate for
mortality.
gestational age because her birth weight was1180 grams
and 38 cm length which was between 50th and 75th
percentile in Lubchenco curve.
To know differences in clinical manifestation
based on age of disease onset in children with
SLE

JOURNAL SEARCHING
“Differential manifestations of prepubescent, pubescent, and postpubescent
pediatric patients with systemic lupus erythematosus: A retrospective study of 96
Chinese children and adolescents”
Chiang Li-Lan et al. in Pediatric rheumatology year 2012
(Level of Evidance 2B, grade of recommendation B).

Conclusion: Prepubescent showed significantly more sphlenomeghaly and anti-Jo-1 antibody positivity
when compared with pubescent and postpubescent. Renal involvement and leukopenia are more frequent
for postpubescent compared to pubescent and prepubescent, showing positive correlation, with p<0,001.
Case Analysis

NEC is an acute inflammatory necrosis of the bowel


. Necrotizing enterocolitis
that primarily affects preterm infants and remains a
(NEC) is a devastating
In this case, leading cause of mortalitysupplementation
and morbidity in neonatal
morbidity usually seenpatient
in received probiotics
intensive care units (NICU).
and did
preterm not
infants, had any symptoms
with of NEC.
Risk factors for classic NEC include prematurity, a
extremely preterm neonates
feeding insult, abnormal bacterial flora, and intestinal
(EPT ≤28 weeks) considered at
ischemia/reperfusion injury with activation of
highest risk
proinflammatory cytokines.
Case Analysis

. From return to birth weight through discharge, the goal of


enteral nutritional management of these infants should
include requirements for catch-up growth, and should be set
for weight gain 10-15 g/kg/day and head circumference
increase upIn to this case, patient
0.8 cm/week, because got breastrate
this growth milk and human milk
was associated with better neurodevelopmental and
fortifier, the body weight was increased 10-15 g/kg/day.
growth outcomes.

Two major strategies are in common clinical use:


multinutrient fortification of human breast milk for
breastfed infants and nutrient- enriched formula for
formula-fed infants
to understand whether there are any differences in outcome
Lupusto Nephritis
progression end-stage renal disease based on clinical feature in
pediatric SLE

Journal Searching
Lupus nephritis (LN) is the most frequent manifestation of lupus, being
observed in 30%-75%
In this ofcase:
patients, either
patient at the
was time of onset or during
male
“Lupus nephritis in males: Clinical features, course, and prognostic factors
the couse of the disease.
with chronic kidney disease due to lupus nephritis
for End-Stage renal disease”
in Kidney International report, 2017.
and GFR <60ml/min/1.73
Evidence
SLE in males basedassociated
has been analysis valid,
withimportant,
greaterand applicable
severity and poorer
(level of evidence
prognoses, particularly as a 2b, grade
result of of recommendation
the B)
presence of serositis and
greater renal, neurologic, and hematologic impairement.
Conclusion: This study showed that LN in males usually presents as
nephrotic syndrome, and type IV LN is the most frequent from. An estimated
glomerular filtration rate <60ml/min/1.73 m2 persisted as a risk factor for
progression to end-stage renal disease. When compared with a cohort of
female patients with LN, there were no significant differences in remission or
renal survival.
Case Analysis

The effects of iron deficiency


are pervasive and involve
The Hb nadir is lower and multiple organ systems. Poor
The premature infant tend to occurs earlier in more physical growth, gastrointestinal
develop negative iron premature (gestational age disturbances, thyroid dysfunction,
balance due to a number of 28–32 weeks) infants when altered immunity and temperature
factors. compared with those born instability has been attributed to
at a gestational age of 33– iron deficiency in very low birth
36 weeks weight (VLBW, birth weight <1500
g) infants.
Case Analysis

Dietary iron deficiency during early infancy is


associated with long-term neurodevelopmental
impairments that appear to be irreversible in spite of
In this case, this patient was given
iron supplementation. Long-term cognitive
iron
supplementation
abnormalities have also been demonstrated in full- at 2 weeks.
term infants with iron deficiency in the neonatal
period.

infants and young children with iron deficiency


anemia are at risk of developmental difficulties
involving cognitive, social-emotional, and adaptive
functions.
Case Analysis

Kangaroo mother care (KMC) is a method of treatment


for low birth weight infants by approaching skin to skin
The infant mortality rate due to low birth weight is
contact with mother. Direct contact between mother
between 60 to 80% of the total number of perinatal
and infant skin will fulfill infant’s basic needs such
deaths. .
In this case, patient was underwent KMC since
warmth, breastfeeding, protection from infection,
stimulation, safety and love.
the mother was ready.

This method provides stimulus to the hypothalamus that release


corticotrophin releasing hormone (CRF) and endorphins that will
make infants feel convenient and quiet, reducing stress hormone
such cortisol and will lead to maturity of immune system in
preterm infants
to understand whether there are any differences in outcome
Lupusto Nephritis
progression end-stage renal disease based on clinical feature in
pediatric SLE

Journal Searching
Lupus nephritis (LN) is the most frequent manifestation of lupus, being
observed in 30%-75%
In this ofcase:
patients, either
patient at the
was time of onset or during
male
“Lupus nephritis in males: Clinical features, course, and prognostic factors
the couse of the disease.
with chronic kidney disease due to lupus nephritis
for End-Stage renal disease”
in Kidney International report, 2017.
and GFR <60ml/min/1.73
Evidence
SLE in males basedassociated
has been analysis valid,
withimportant,
greaterand applicable
severity and poorer
(level of evidence
prognoses, particularly as a 2b, grade
result of of recommendation
the B)
presence of serositis and
greater renal, neurologic, and hematologic impairement.
Conclusion: This study showed that LN in males usually presents as
nephrotic syndrome, and type IV LN is the most frequent from. An estimated
glomerular filtration rate <60ml/min/1.73 m2 persisted as a risk factor for
progression to end-stage renal disease. When compared with a cohort of
female patients with LN, there were no significant differences in remission or
renal survival.
Case Analysis

In this case, patient was born very preterm (28


weeks) and extremely low birth weight (900
grams) with respiratory distress syndrome, using
In preterm infants receiving oxygen therapy,
ventilation support with totalRisk factors of ROP
oxygen use include
more gestational
retinopathy of prematurity (ROP) is the most
age, birth weight <1,500 grams, apnea,
than seven days. concluding the
common visual impairment. The incidence of
bloodpatient
transfusion,ishyaline
at risk
membrane
retinopathy of prematurity in very preterm
infants (<32 weeks) and low birth weight for ROP.disease, phototherapy, ventilator support,
oxygen use more than seven days and
infants (<1,500 grams) in Indonesia ranged
surfactant use.
from 11.9–30.5%
to understand whether there are any differences in outcome
Lupusto Nephritis
progression end-stage renal disease based on clinical feature in
pediatric SLE

Journal Searching
Lupus nephritis (LN) is the most frequent manifestation of lupus, being
observed in 30%-75%
In this ofcase:
patients, either
patient at the
was time of onset or during
male
“Lupus nephritis in males: Clinical features, course, and prognostic factors
the couse of the disease.
with chronic kidney disease due to lupus nephritis
for End-Stage renal disease”
in Kidney International report, 2017.
and GFR <60ml/min/1.73
Evidence
SLE in males basedassociated
has been analysis valid,
withimportant,
greaterand applicable
severity and poorer
(level of evidence
prognoses, particularly as a 2b, grade
result of of recommendation
the B)
presence of serositis and
greater renal, neurologic, and hematologic impairement.
Conclusion: This study showed that LN in males usually presents as
nephrotic syndrome, and type IV LN is the most frequent from. An estimated
glomerular filtration rate <60ml/min/1.73 m2 persisted as a risk factor for
progression to end-stage renal disease. When compared with a cohort of
female patients with LN, there were no significant differences in remission or
renal survival.
Case Analysis

Risk factors of ROP include gestational


age, birth weight <1,500 grams, apnea,
blood transfusion, hyaline membrane
disease, phototherapy, ventilator support,
This patient requires long-term monitoring due oxygen use more than seven days and
to her prematurity and very low birth weight surfactant use.

In this case, patient was born very preterm (28


weeks) and extremely low birth weight (900
CASE ANALYSIS

Coomb’s test is one of the examinations used to diagnose hemolytic anemia.


The sensitivity and specificity ofIn
Coomb’s test is 38.5% and 98.5%, respectively.12
this case:
About 3-11% SLE patients with hemolytic anemia in bloodsmear show a negative
• Patient’s bloodsmear showed poikilocytosis
Coomb’s test which is appropriate
for hemolytic anemia. However, his Coomb’s test was negative.
This was due to a low corrected reticulocyte count.
CASE ANALYSIS

Treatment goals in SLE

control inflammation, achieve followed by


remission condition (without administration of Disease Lupus nephritis (LN) with poor
clinical manifestation), Modifying Anti Rheumatic outcome which was treated by
improve quality of life, no Drugs (DMARDs), include steroids improved significantly with
severe exacerbation, prevent cyclophosphamide, the introduction of
serious organ damage and Mycophenolate mofetil, cyclophosphamide.
reduce mortality rate or Azathioprine
to know the outcomes of intravenous cyclophosphamide treatment in lupus
nephritis chidren between patients with normal and abnormal initial renal function

Journal Searching
In this case,
Patient was given cyclophosphamide
“Intravenous high dose methylprednisolone pulse
combined with dose for
steroids 3 days then
in pediatric
continued with methylprednisolone
onset severe 1mg/kgBW/day and cyclophosphamide 500
lupus nephritis”
mg/BSA/time intravenously every
In Int Urol2Nephrol
weeks as many
year as 12 cycles or 6 months,
2012
followed by cyclophosphamide
Evidence based analysis valid, maintenance every
important, and 3 months.
applicable
Patient had decreased
(level of renal
of evidence function
2b, grade before started intravenous
of recommendation B)
cyclophosphamide with the GFR was 7 ml/min/1.73 m2.
Conclusion: three years of cyclophosphamide intravenous treatment
provided similar outcomes in both normal and abnormal renal function
groups
To understand the predicting factor in children
with SLE

Journal Searching
In this case,
Patient had
“Predictors fever for
of mortality several days
in Children which showed
with systemic a possibility of
lupus erythematosus”
infection. Urine culture showed
in Paediatrica Acinetobacter
Indonesiana 2019 baumanii and
Enterobacter cloacae
Evidence basedssp. cloacae,
analysis in which Acinetobacter
valid, important, and applicable baumanii as
the dominant(levelorganisms,
of evidence 2b,
with grade of recommendation
colonization B)
over 100.000 colony/ml.
Conclusion: This study concluded that, among the factors studied, infection
is significantly associated with mortality in children with SLE.
We searched for the journal which showed characteristic of urinary
tract infection in Lupus Erythematosus patient.

Journal Searching

“Risk Factor and Bacterial Profiles of Urinary Tract Infection in Patient with
Systemic Lupus Erythematosus” in Asian Pacific Journal of
Allergy and Immunology 2007

• But, the mean age of subject in the journal were 44.4 + 15.8 years old, sample was
taken from 18-74 years old subject.
• In journal stated that 100% of UTI subject suffered from fever with p < 0.05.
Dysuria only found in 38.5%, frequency 46.2%, urgency 15.4%, flank knocking pain
in 30.8%, abdominal pain 70.7%, nausea and vomiting 23.1%, p value > 0.05.
• In journal also stated that UTI in children with SLE, will be more unlikely with sign
and symptom, but only presented with fever.
THANK YOU
Problems
Problem 1

According to the problems, PICO can be described as follows:


•P (Patient/Problem) : children with systemic lupus erythematosus
•I (Intervention) : classification age group
•C (Comparation/Control) :-
•O (Outcome) : clinical manifestation based on age of disease onset

CLINICAL QUESTION
• In children with systemic lupus erythematosus, are there any differences in clincial
• manifestation based on age of disease onset?

JOURNAL SEARCHING STRATEGY


Keywords: children AND systemic lupus erythematosus AND clinical manifestation
“Differential manifestations of prepubescent, pubescent and postpubescent
pediatric patients with systemic lupus erythematosus: a retrospective study of
96 Chinese children and adolescent”
Chiang L, Lin Y, Chan H, Chiang B
Pediatric Rheumatology, 2012;10:2-9
“Differential manifestations of prepubescent, pubescent and postpubescent
pediatric patients with systemic lupus erythematosus: a retrospective study of
96 Chinese children and adolescent”
Chiang L, Lin Y, Chan H, Chiang B
Pediatric Rheumatology, 2012;10:2-9
EVIDENCE BASED MEDICINE (PROGNOSTIC ASPECT)
I. Are the results of this prognostic study valid?

1 Was a defined, representative sample of patients assembled at a Yes, patients were recruited in early
common (usually early) point in the course of their disease? diagnose of SLE
2 Was patient follow-up sufficiently long and complete? Yes, mean values were 21.3 months for
group A, 9.2 months for group B and 4.4
months for group C
3 If subgroups with different prognoses are identified, was there Yes, cumulative disease activity at diagnosis
adjustment for important prognostic factors? was measured using the SLEDAI score

4 Is the test validated in an independent second patient There is no subgroup identified.


group?
5 Was there validation in an independent group (“test set”) of This study is not validated with other
patients? groups

This study is valid


EVIDENCE BASED MEDICINE (PROGNOSTIC ASPECT)

II. Is this valid prognostic study important?

1 How likely are the outcomes over time? How The disease activity (SLEDAI-2K score) correlated
precise are the prognostic estimates? positively with age at disease onset and negatively with
disease duration before diagnosis (p = 0.011). 30 years
survival rate analysis by Kaplan-Meier was 100% in group
A, 88.3% in group B and 97.4% in group C.

This study is important


EVIDENCE BASED MEDICINE (PROGNOSTIC ASPECT)

III. Can we apply evidence of this valid and important evidence about prognosis in caring for our patient?

1 Were the study patients similar to our own? Yes, characteristic type of subjects in this study similar
with our patients

2 Will this evidence make a clinically important impact Yes, this evidence is worth for our clinical practices
on our conclusions about what to offer or tell our
patient?

This study is applicable


Conclusion: valid, important, and applicable
Level of Evidence 2B
Grade of recommendation B
Problem 2

According to the problems, PICO can be described as follows:


•P (Patient/Problem) : male children with lupus nephritis
•I (Intervention) : clinical feature
•C (Comparation/Control) :-
•O (Outcome) : end-stage renal disease

CLINICAL QUESTION
• In male children with lupus nephritis, are there any differences in outcome progression to
end-stage renal disease based on clinical feature?

JOURNAL SEARCHING STRATEGY


• Keywords: male children AND lupus nephritis AND clinical feature AND end-stage renal
disease
“Lupus Nephritis in Males: Clinical Features, Course, and Prognostic Factors for
End-Stage Renal Disease”
Urrestarazu A, Otatti G, Silvarino R, Garau M, Coitino R, Alvarez A, et al.
Kidney Int Rep. 2017:(2):905-12
“Lupus Nephritis in Males: Clinical Features, Course, and Prognostic Factors for
End-Stage Renal Disease”
Urrestarazu A, Otatti G, Silvarino R, Garau M, Coitino R, Alvarez A, et al.
Kidney Int Rep. 2017:(2):905-12
EVIDENCE BASED MEDICINE (PROGNOSTIC ASPECT)
I. Are the results of this prognostic study valid?

1 Was a defined, representative sample of patients assembled at a Yes, patients were recruited in early
common (usually early) point in the course of their disease? diagnose of SLE and lupus nephritis
2 Was patient follow-up sufficiently long and complete? Yes, the cohort had a median follow up
of 54 months (range, 2–360 months).

3 If subgroups with different prognoses are identified, was there Yes, subgroup based on remission of the
adjustment for important prognostic factors? lupus nephritis were adjustment for
important prognostic factors.

4 Are clinical outcomes measured in the same way in both groups? Yes, clinical presentation and outcomes of
males with lupus nephritis compared with
matched females.
5 Was there validation in an independent group (“test set”) of This study is not validated with other
patients? groups

This study is valid


EVIDENCE BASED MEDICINE (PROGNOSTIC ASPECT)

II. Is this valid prognostic study important?

1 How likely are the outcomes over time? Five-year renal survival was close to 78% and more than
70% at 10 years.

2 How precise are the prognostic estimates? Remission of the lupus nephritis had an impact on renal
survival. Significant differences were found in the renal
survival of the complete remission group, the partial
remission group (P = 0.005), and the non remission group
(P < 0.001).

This study is important


EVIDENCE BASED MEDICINE (PROGNOSTIC ASPECT)

III. Can we apply evidence of this valid and important evidence about prognosis in caring for our patient?

1 Were the study patients similar to our own? Yes, characteristic type of subjects in this study similar
with our patients

2 Will this evidence make a clinically important impact Yes, this evidence is worth for our clinical practices
on our conclusions about what to offer or tell our
patient?

This study is applicable


Conclusion: valid, important, and applicable
Level of Evidence 2B
Grade of recommendation B
Problems 3

According to the problems, PICO can be described as follows:


•P (Patient/Problem) : pediatric onset lupus nephritis
•I (Intervention) : intravenous cyclophosphamide and steroid therapy
•C (Comparation/Control) :-
•O (Outcome) : long term outcome

CLINICAL QUESTION
• In children with lupus nephritis, are there any differences in long term outcome when using
intravenous cyclophosphamide combined with steroid therapy?

JOURNAL SEARCHING STRATEGY


• Keywords: children AND lupus nephritis AND intravenous cyclophosphamide AND steroid
AND outcome
“Intravenous cyclophosphamide combined with steroids in pediatric onset severe
lupus nephritis”
• Vanchvanichsanong P, Dissaneeweate P, McNeil E.
• Int Urol Nephrol. 2013;45:1301-1308
“Intravenous cyclophosphamide combined with steroids in pediatric onset severe
lupus nephritis”
• Vanchvanichsanong P, Dissaneeweate P, McNeil E.
• Int Urol Nephrol. 2013;45:1301-1308
EVIDENCE BASED MEDICINE (PROGNOSTIC ASPECT)
I. Are the results of this prognostic study valid?

1 Was a defined, representative sample of patients Yes, patients who attended the Department of
assembled at a common (usually early) point in the Pediatrics newly diagnosed with lupus nephritis
course of their disease? class IV or severe glomerulo-nephritis were
included in this study.
2 Was patient follow-up sufficiently long and complete? Yes, the observation was according to the total
duration of treatments (total 36 months).
3 If subgroups with different prognoses are identified, was Yes, subgroup based on remission of the lupus
there adjustment for important prognostic factors? nephritis were adjustment for important prognostic
factors.
4 Are clinical outcomes measured in the same way in both Yes, clinical presentation and outcomes of patients
groups? were measured between the two groups with
different initial creatinine.
5 Was there validation in an independent group (“test set”) This study is not validated with other
of patients? groups

This study is valid


EVIDENCE BASED MEDICINE (PROGNOSTIC ASPECT)

II. Are the results of this prognostic study important?

1 How likely are the outcomes over time? How Crude mortality rate was higher in the group of patients
precise are the prognostic estimates? with abnormal renal function at the start of treatment
(10/72 versus 13/36, p=0.02). The overall survival rates at
2 and 5 years were 93% and 78%. The median survival
time for the abnormal renal function group was 8.7 years
(p=0.15)

This study is important


EVIDENCE BASED MEDICINE (PROGNOSTIC ASPECT)

III. Can we apply evidence of this valid and important evidence about prognosis in caring for our patient?

1 Were the study patients similar to our own? Yes, characteristic type of subjects in this study similar
with our patients

2 Will this evidence make a clinically important impact Yes, this evidence is worth for our clinical practices
on our conclusions about what to offer or tell our
patient?

This study is applicable


Conclusion: valid, important, and applicable
Level of Evidence 2B
Grade of recommendation B
Problem 4

According to the problems, PICO can be described as follows:


•P (Patient/Problem) : children with systemic lupus erythematosus
•I (Intervention) : predictor
•C (Comparation/Control) :-
•O (Outcome) : mortality

CLINICAL QUESTION
In children with systemic lupus erythematosus, are there any predictors for mortality?

JOURNAL SEARCHING STRATEGY


Keywords: children AND systemic lupus erithematosus AND predictor AND mortality
“Predictors of mortality in children with systemic lupus erythematosus””
• Listiyono F, Sumadiono, Satria CD, Murni IK
• Paediatrica Indonesiana. 2019;59:1-6.
“Predictors of mortality in children with systemic lupus erythematosus””
• Listiyono F, Sumadiono, Satria CD, Murni IK
• Paediatrica Indonesiana. 2019;59:1-6.
EVIDENCE BASED MEDICINE (CAUSATION ASPECT)
I. Is this evidence about causation study valid?

1 Were there clearly defined groups of patients, similar in Yes, clearly defined group of patients. This study
all important ways other than exposure to the treatment was divided into Died group and Survived group.
or other cause?
2 Were treatment/exposure and clinical outcomes Yes, predictors of mortality in children with
measured in the same ways in both groups? (Was the systemic lupus erythematosus in all groups base on
assessment of outcomes blinded to exposure?) medical record data.
3 Was the follow-up of the study patients sufficiently long Yes, the study period was 8 years.
(for the outcome to occur) and complete?
EVIDENCE BASED MEDICINE (CAUSATION ASPECT)
I. Is this evidence about causation study valid?

4 Do the results of the causation study fulfill some of the


diagnostic tests for causation?
• Is it clear that the exposure preceded the onset of the • Yes
outcome?
• Not described in journal
• Is there a dose-response gradient?
• Not described in journal
• Is there any positive evidence from a “dechallenge-
rechallenge” study?
• Is the association consistent from study to study? • Not described in
• Does the association make biological sense? • Yes
This study is valid
EVIDENCE BASED MEDICINE (CAUSATION ASPECT)

II. Are the valid results of this causation study important?

1 What is the precision of the estimate of the Infection significantly affected mortality in children with
association between the exposure and the systemic lupus erythematosus (Odds Ratio 3.22 and 95%
outcome? 1.15 to 9.05, P value = 0.02).

This study is important


EVIDENCE BASED MEDICINE (CAUSATION ASPECT)

III. Guides for deciding whether valid important evidence about causation can be applied to our patient?

1 Is our patient so different from those included in the No, our patient not so different from those included in
study that its result cannot apply? the study.

2 Will this evidence make a clinically important impact Yes, this evidence provide data on the predictors of
on our conclusions about what to offer or tell our mortality in children with systemic lupus erythematosus.
patient?

This study is applicable


Conclusion: valid, important, and applicable
Level of Evidence 2B
Grade of recommendation B

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