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UL MB WD Edit WD 17 - 8 - 19 17.45
UL MB WD Edit WD 17 - 8 - 19 17.45
9), CHRONIC
KIDNEY DISEASE STAGE V (N18.5) DUE TO LUPUS
NEPHRITIS (N08.5), URINARY TRACT INFECTION (N39.0),
AND WELL NOURISHED.
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 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 .
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
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
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
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
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
ANALYSIS
Case Analysis
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
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
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
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
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
CLINICAL QUESTION
• In children with systemic lupus erythematosus, are there any differences in clincial
• manifestation based on age of disease onset?
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
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.
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?
CLINICAL QUESTION
• In male children with lupus nephritis, are there any differences in outcome progression to
end-stage renal disease based on clinical feature?
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
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).
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?
CLINICAL QUESTION
• In children with lupus nephritis, are there any differences in long term outcome when using
intravenous cyclophosphamide combined with steroid therapy?
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
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)
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?
CLINICAL QUESTION
In children with systemic lupus erythematosus, are there any predictors for mortality?
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?
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).
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?