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MBBS Program Year 3 CASE WRITE-UP

PAEDIATRIC POSTING

NAME: KHOO ER HAU

STUDENT NO.: 16UMB05703

ACADEMIC YEAR: 2018/2019

GROUP: 4B

LECTURER IN-CHARGE: Dr. Veronica Poulsaeman

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Faculty of Medicine and Health Sciences, UTAR
Score sheet for CASE Write-Up
In Paediatric Posting
Year 3 MBBS

Student Name: KHOO ER HAU

ID No.: 16UMB05703 Year: 3

Name of lecturer: Dr. Veronica Poulsaeman

Marks allocated for each section of Case Write-up

Chief complaints /5 Investigations (with indications, /10


results & interpretation of
results)

History of present illness is /20 Basic principles of management, /7


chronologically clear with clinical course of patient in the
relevant positive & negative ward and final diagnosis at
findings discharge or death

Other history (Birth, feeding, /10 Discussion on lessons learned /10


immunization, development, from the clinical features ,
family history, social history, investigations and management
past history, drug history, of this case, including issues on
allergy history, etc) professionalism, ethics, patient
safety and communications.

Physical Examination findings /15 References /3


clearly documented

Summary of case clear and /5 Proper use of English language, /5


concise and clear and logical write-up
Discussion of /10 Total score /100
diagnosis/Differential diagnosis

Signature of Lecture: ________________Date:___________________________

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PATIENT IDENTIFICATION
Patient’s Initial: M R/N: AM00580975 Age: 10 years old
Gender: Male Ethnic group: Indian
Date of admission: 29/8/2018 Date of discharge: 3/9/2018
Date of clerking: 29/8/2018 Source of History: Parents

CLINICAL FINDINGS & DISCUSSION


Chief Complaint:
M, a 10-years old Indian boy, with underlying steroid sensitive nephrotic syndrome was
admitted to the hospital due to fever, cough and runny nose for 3 days, and abdominal pain for
2 days.

History of Present Illness:

M, was diagnosed with nephrotic syndrome one year prior to admission (end of
September 2017) and has been on corticosteroid maintenance. Upon defaulting on scheduled
appointment, M has stopped medication since 26/7/18, one month prior to admission.
Subsequently, urine dipstick noted proteinuria 2+ for 6 consecutive days (6/8/2018 – 11/8/201)
and proteinuria 3+ for 9 days consecutively (14/8/2018-23/8/2018). During this period of time,
M was asymptomatic and did not present to the hospital due to logistics problem. 3 days prior
to admission, upon returning from holiday with family at Rawang, M started to have fever.
Initially, the mother felt M was warm to touch, and documented temperature at GP clinic was
38oC. He has no chills, rigors or convulsions. On the same day, he started to have cough and
runny nose. The cough was chesty and productive; intermittent in nature; not bark-like; not
prolonged; and occurring a few times in a day, with post-tussive vomiting once. There was no
history of posseting, no cough after feeding, nor any facial congestion, or peri-oral cyanosis.
As for running nose, the nasal discharge was always clear.
Ever since the onset of fever, M started noticing to have tea-colored urine. According
to M, the urine started becoming very frothy and turning darker in colour day by day. However,
there was no increase in frequency, no dysuria or incontinence.
2 days prior to admission, M started complaining of abdominal pain over the umbilical
and epigastric region. The pain occurred mostly postprandial. M was not able to describe the
character of the pain but rated the pain 5/10. Mother did not noted any relieving or exacerbating
factor. The same day itself, M vomited 1-2 times and the vomiting persisted for 2 days. The

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vomitus mostly contained food particles and fluid with no blood. The vomiting was
characterised to be non-bilious and non-projectile. Despite that, M did not seek medical
attention for the abdominal pain and vomiting. One day prior to admission, M also had 2
episodes of loose stool. The stool was watery, green with no blood. Ever since the vomiting,
diarrhoea and abdominal pain occurred, M started having decrease appetite, unable to finish
more than ½ portion of his meal, taking only 2 meals per day, in which normally he was able
to finish all 3 meals in complete portion. Apart from that, his mother had noticed M had some
swelling over both of his legs and facial puffiness.
On presentation, M appears alert, no longer febrile, with intermittent cough and runny
nose. Apart from that, there was notable bilateral lower limb swelling, periorbital swelling and
facial puffiness. Otherwise, there was no sore throat, rash, headache, abdominal distension or
any bleeding tendencies. M has no ill contact recently. On admission, dipstick was noted to be
proteinuria 3+, according to mother the dipstick has been showing proteinuria 2+ ever since
onset of fever, cough and runny nose (24/8/2018-28/8/2018).

Systemic Review:
Constitutional: Increased in weight recently, not irritable, no change in activity or energy level

Cardiovascular: No shortness of breath, no chest pain, no palpitations, no sweating, no color


changes with feeding, no history of murmur, no dizziness with activity

Respiratory system: No increased work of breathing, no wheezing, no epistaxis, no hemoptysis

Gastrointestinal: No constipation, no hematemesis, no abnormal passing of gas, no


hematemesis

Genitourinary: No history of UTI, no dysuria, no increase in frequency, no urgency, no


hematuria

Dermatological: No itchiness, no rashes or eczema, no bruising or petechiae, no other abnormal


skin lesions

Endocrinology: No polyuria, no polydipsia, no history of heat or cold intolerance

Central nervous system: No dizziness, no loss of consciousness, no difficulty with gait or


balance

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HEENT: Vision not clear, no photophobia / phonophobia, no eye discharge / itchiness or
excessive tearing, no ear discharge, no sore throat, no neck pain

Musculoskeletal: Has back pain, arthralgia and myalgia with no history of fracture or recent
trauma, all limbs and joint move freely, no redness or swelling at joints, no muscle or joint
stiffness, no cramping or weakness

Antenatal History:
During the antenatal period, M’s mother was healthy with no complication (no
gestational diabetes, hypertension or epilepsy). Prenatal infective screening revealed a negative
Hepatitis B antigen, negative HIV screen, non-reactive RPR, immune to Rubella and GBS
negative. There were also no intrauterine infection or perinatal infections. No abnormalities
were seen during her scheduled check-up and antenatal ultrasound showed no anomaly.

Birth History:
M was born termed at 38 weeks via spontaneous vaginal delivery at Hospital Ampang.
His birth weight was 3.97kg, considered large for gestational age. However there were no
complication during and after the delivery. APGAR’s are unknown but according to mother,
M was pink, cried at birth, did not need resuscitation and was active in flexing his arms and
legs. Newborn screen was performed prior to discharge, both G6PD and TSH were normal.

Neonatal History:
After birth, M had physiological jaundice and was placed under phototherapy for 1
week, to which his jaundice was aborted after that. Otherwise, M did not have fever, sepsis or
respiratory distress. Following that, he was discharged together with his mother.

Feeding History:
M was breastfed exclusively for 4 months. After that he was given formula milk for 2
months and started weaning ever since 6 months old, Currently, M’s able to tolerate adult diet,
which consists mainly of balanced nutrition, with adequate protein intake. Ever since diagnosed
with nephrotic syndrome, he has been having taking food with low salt content only. He is not
a picky eater, takes 3 meals per day and no other supplementation.

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Vaccination History:
Vaccination was up to date .According to the mother, he has not received any additional
vaccination shots from private hospitals. The table below shows the list of vaccination he has
received to this day:
Age Vaccination
At birth BCG (scar can be seen) (Single dose)
Hepatitis B (1st dose)
1 month Hepatitis B (2nd dose)
2 months Haemophilus influenza B (Hib), Diphtheria, Tetanus and
Pertussis (DTaP), Inactivated poliovirus (IPV) (1st dose)
3 months Hib, DTaP and IPV (2nd dose)
5 months Hib, DTaP and IPV (3rd dose)
6 months Hepatitis B dose 3
12 months Mumps, Measles, Rubella (1st dose)
18 months Hib, DTaP and IPV (4th dose)
7 years old Mumps, Measles, Rubella (2nd dose)
Diphteria Tetanus booster

Developmental History:
M is currently attending primary school in SK Cochrane. Academically his performance is
average, studying in the 4th class. He’s not active in sports. However, socially, he mixes well
with his peers. Development is appropriate for his age.

Past Medical and Surgical History:


M was diagnosed in late September 2017 with nephrotic syndrome. He was hospitalised
for 8 days for the 1st time. Induction was given and he completed maintenance on end of March
2018. Following that, he defaulted on scheduled appointment to review his progress and
continued prednisolone up to 19th April 2018.
Upon stopping prednisolone, relapse occurred on 20th April 2018 and urine dipstick
show proteinuria 3+ for 3 consecutive days. He was presented to the ED, and was identified as
breakthrough proteinuria to which he was admitted for 2 days. Again, he was started on
induction (60mg OD in two divided doses) and subsequently with tapering of dose (40mg OD
in two divided doses). He was supposed to complete maintenance up to 31/5/2018. Upon

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discharge, he was scheduled to attend appointment after 1 week however he defaulted again
and continue 20mg OD up to 20/7/2018.
On early July 2018 (4/7/2018), he was admitted to the hospital due to complaint of
musculoskeletal pain and presented with limping due to long term misuse. He was advised to
stop the medication. In the ward, his dose was tapered down from 30mg OD to 20mg OD to
10mg OD to reduce the effect of corticosteroid. Subsequently he was able to run and walk prior
to discharge. He was allowed to be discharge with tapered dose of prednisolone, and was
scheduled for an appointment after 6 weeks after completion of prednisolone. However the 2nd
relapse occurred, urine dipstick showed protein 2+/3+ for 5 consecutive days on 6/8/2018
onwards, and although asymptomatic, he did not present to the hospital due to logistic reasons.
This is the 3rd hospitalisation. Below is a summary of his progress.

Date Dosage Urine dipstick


30/9/2017-29/10/2017 30 mg BD (60mg OD) Negative
30/10/2017-29/11/2017 20 mg BD (40mg OD) Negative
1/12/2017-31/12/2017 15 mg BD (30mg OD) Negative
1/1/2018-31/1/2018 15 mg BD (25mg OD) Negative
1/2/2018-28/2/2018 15 mg BD (25mg OD) Negative
1/3/2018-31/3/2018 10 mg BD (20mg OD) Negative
1/4/2018-20/4/2018 15 mg OD -> stop Negative
20/4/2018-28/4/2018 Reinduction 30 mg BD (60mg OD) 21/4/2018–23/4/2018 2+
24/4/2018-25/4/2018 1+
26/4/2018-28/4/2018 negative
29/4/2018-3/7/2018 20 mg BD (40mg OD) 29/4/2018-2/6/2018 negative
3/6/2018-4/6/2018 1+
5/6/2018-15/6/2018 negative
16/6/2018-17/6/2018 1+
17/6/2018- 4/7/2018 negative
4/7/2018 -11/7/2018 15 mg BD (30 mg OD) 5/7/2018-7/7/2018 1+
8/7/2018-10/7/2018 negative
12/7/2018-18/7/2018 10 mg BD (20mg OD) 11/7/2018-12/7/2018 +1
13/7/2018-18/7/2018 negative

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19/7/2018-25/7/2018 10 mg OD 19/7/2018-25/7/2018 1+
26/7/2018 ->.admission Stop maintenance 26/7/2018-27/7/2018 1+
28/7/2018 2+
29/7/2018-5/8/2018 1+
6/8/2018-11/8/2018 2+
12/8/2018-13/8/2018 1+
14/8/2018-23/8/2018 3+
24/8/2018-28/8/2018 2+

Other than the nephrotic syndrome, he has no other medical illnesses. No history of
hospitalisation or surgery done.

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Medication history & history of allergy:
M is on prednisolone maintenance due to nephrotic syndrome. Prior to admission, he
has not taken any over-the-counter drugs nor was he prescribed any medication. M was not on
any NSAID medication. No traditional herbal medication or health supplements was taken.
There are no known allergy towards food and medication.

Family History:
Father Mother
57 years old 47 years old

Male

23yo 21yo 16yo 14yo 14yo M Female


12 yo

Currently his father has diabetes mellitus, hypertension and hyperthyroidism, while his
mother is healthy with no known medical illness. There were no history of consanguinity in
the family. His uncle (maternal side) has nephrotic syndrome and no other family members has
history of renal diseases. Other than that, there was no family history of Hodgkin’s disease,
amyloidosis, systemic vasculatides or heart failure.

Environmental History:
M lives with his mother and 4 of siblings, in total 7 people under a roof in a small
apartment house at Cheras.

Social History:
His father works as a driver in Penang whereas his mother is a housewife. Currently
he’s being taken care by his mother. His mother is requesting for financial aid as family income
is less than RM1500. In addition, his mother does not have a car to send his son to the hospital
as well when needed. Despite the financial burden, his mother claims M’s daily needs are met.
No family members in the house smokes or consumes alcohol.

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Physical Examination:
Anthropometric measurements
Height: 137 cm
Weight: 39.2 kg
BMI: 20.9 kg/m2

(Refer behind for growth charts)

Interpretation: According to growth chart, M is thriving on the 75th to 90th percentile for
weight, 25th percentile for height. His BMI is normal. Overall height and weight are
satisfactory.

Vital signs:
Temperature: 36.7°C (high)

Blood Pressure: 136/80mm Hg (normal)

Pulse rate (Awake): 81 beats/ min (normal)

Respiratory rate: 26 breaths/ min (normal)

SpO₂: 98% on room air

Interpretation: She is currently afebrile. She is not tachycardic and not tachypneic. Other vital
signs are normal.

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General Examination:
The patient was lying comfortably on the bed. He looks pink, conscious, active and
alert to his surroundings. He was not tachypneic, not septic looking and look well perfused. He
is orientated to time and place. Branula was seen on the left dorsum of the hand. His general
appearance looks normal and he was afebrile to touch. Glasgow Coma Scale (GSC) was
assessed and AW achieved a score of 15/15. His eyes were able to open spontaneously (4
points). There’s good verbal response (5 points), and he was able to move spontaneously and
purposefully (6 points). He has good cognitive ability with no speech impairment.

Hydration status
M did not present with any signs of dehydration - no sunken eyes seen, skin turgor is
good. There is no peeling or scaling of the skin and his lips are moist. His hands however are
quite dry. His hydration status is moderate.

Nutrition
His hair is well black, has good amount and was not frail, could not be easily plucked.
He appears to have good muscle bulk with no muscle wasting. There is no angular stomatitis,
bitot spots or macroglossia.

Skin
There are no rashes seen at the upper limb and lower limbs, trunk, back and face.
Multiple well-healed injury scars are seen on the lower limbs. No signs of bleeding tendencies
such as petechiae, purpura or ecchymosis are observed.

HEENT
There are no scleral icterus or conjunctival pallor. Periorbital swelling is noted.
Horizontal and vertical movements of the eyes are normal, no nystagmus, or strabismus. There
are no dysmorphic features e.g. upslanted palpebral fissures, low set ears, flat nose bridge,
epicanthic folds or hypertelorism. There’s no facial asymmetry but facial puffiness is present.
Ears and nose are normal appearing with no deformity, discharge or congestion. Throat is
mildly injected and tonsils are enlarged to half the distance of the uvula (Grade II), with no
tonsillar exudates. Other than that, the teeths and gums look healthy with no bleeding. No
central cyanosis observed from the tongue.

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Lymph nodes
There are no palpable submental, submandibular, pre-auricular, post-auricular,
occipital, anterior or posterior cervical, supraclavicular lymph node, epitrochlear, popliteal and
inguinal nodes.

Respiratory System
On inspection, there’s no nasal flaring and nasal septum is not deviated. There’s no
pursed lip breathing. No stridor. The chest is normal in shape, bilaterally symmetrical without
undue elevation or recession, and moves with respiration. The anterior posterior diameter of
the chest was less than the lateral diameter. There is no deformity, no surgical scar or dilated
veins. There’s no obvious use of accessory muscles in breathing. The respiratory rate was 26
breaths per minute, not tachypneic. There’s no tracheal deviation and good symmetrical chest
expansion is observed. There’s also no tracheal tug and no tactile fremitus. The lungs are
resonant to percussion. Upper border of liver percussed to be at the right 5th intercostal space.
On auscultation, normal vesicular breath sounds are heard equally in both lungs with no
crepitations, wheezing or ronchi.

Cardiovascular System
There was no koilonychia, no leukocynia, no peripheral cyanosis, no Janeway lesion,
no Osler’s node and no splinter haemorrhage seen. Peripheries were warm. Capillary refill time
was less than 2 seconds. Pulse rate measured was 81 bpm with regular rhythm, good character
and volume. There was no radioradial delay or radiofemoral delay. Jugular venous pulsation
was not prominent and thyroid was not enlarged. Carotid pulse were felt bilaterally on the neck.
There was no clubbing on both hands and feet. Both the dorsalis pedis and posterior tibial pulse
could be felt.

On inspection of the chest, the chest wall is symmetrical on both sides, no deformity,
no visible pulsation and no precordial bulge. Apex beat is not seen but palpable at the left 5th
intercostal space medial to the midclavicular line. No thrills on other areas of the heart could
be felt. There’s no parasternal heave.

On auscultation, both S1 and S2 hearts could be heard with no murmur. There’s no


carotid bruit.

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Abdominal:
No palmar erythema, duputyren’s contracture or flapping tremor is noted on the hands.
Pitting edema is noted over both of the lower limb up to the mid shin. There’s no sacral or
scrotal edema. There’s no anasarca. Upon inspection of the abdomen, the abdomen is not
distended, not protuberant and moves with respiration. Umbilicus is centrally located and
inverted. There’s no flank fullness, abdominal straie, surgical scars, visible peristalsis, or
dilated veins over the abdomen. There’s no spider naevi or gynecomastia noted on the chest.
On palpation, the abdomen is soft. There’s tenderness over the epigastric area but no guarding,
rigidity or mass could be felt. The liver and spleen are both not palpable. Kidneys were not
ballotable. Upon tapping the costovertebral angle, there’s no tenderness, renal punch is
negative.

On percussion, there is normal liver dulless heard at right 5th intercostal space in the
midclavicular line. Traube’s space is resonance to percussion. Shifting dullness was positive
while no fluid thrill, indicating mild ascites. On auscultation, there’s normal bowel sound with
no liver or renal bruit.

Musculoskeletal System
On inspection, there are no bone deformities over the upper and lower limbs. There’s
no abnormal curvature of the spine. All four limbs are able to move freely without limitations
during passive movement. On palpation, there’s no swelling, tenderness or redness over the
joints. There’s no limb length discrepancy. Muscle bulk is normal with no atrophy. Peripheries
are warm and equal for all four limbs.

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Neurological Examination
Kernig’s sign and Brudzinski’s sign are negative. Below are the findings of the cranial nerve
examination done,

Cranial Nerves Findings


Olfactory nerve (I) No deformity in the nose. Able to identify coffee bean
Optic nerve (II) Visual acuity is 6/6, no myopia or presbyopia.
Normal color vision.
Normal visual field (around 100 degrees)
Direct and consensual light reflex were normal
On fundoscopy, red reflex is present. Optic disk and fundus
could be seen with no abnormality. No macular haemorrhage,
optic nerve atrophy, or papilledema.
Oculomotor, trochlear, No ptosis, strabismus, nystagmus.
abducens nerve (III, IV, VI) Pupil is normal in size and circular shape.
Ocular movements are normal, able to follow finger through
abduction, adduction, elevation, depression, intorsion and
extorsion.
Trigeminal nerve Able to feel light touch over ophthalmic area, maxillary area
and mandibular area.
No muscle wasting of temporal or masseter muscles on both
sides of the face upon clenching teeth.
Jaw jerk and corneal reflex are not elicited.
Facial nerve (VII) No facial asymmetry
Able to raise eyebrows, smile and close eyes tightly
No drooping of eyelids or angle of mouth
Taste sensation and secretory function not tested.
Facial reflexes not tested.
Vesitbulocochlear nerve No deformity in external auditory meatus and tympanic
membrane.
Able to hear properly through rubbing of fingers and
whispering
Unable to test Rinne’s and Weber’s, and vestibular function

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Glossopharyngeal, vagus Able to swallow food and water
nerve (IX, X) No hoarseness, whispering or nasal speech
Palatal arch constricts and elevates with no displacement of
uvula
Gag reflex not tested
Spinal accessory nerve (XI) No dropping of shoulder or abnormal rotation of scapula
No wasting of trapezius or sternocleidomastoid muscle
Trapezius and sternocleidomastoid have muscle power of
5/5.
Hypoglossal (XII) No wasting, tremor, atrophy or inability to protrude the
tongue

All cranial nerves are intact. On sensory examination, M is able to feel crude touch and
has vibratory sensation at all distal and proximal sites on extremities, equal on both sides.
Spinothalamic tract and dorsum column are intact. He’s able to perceive solid objects by touch,
positive for stereognosis, has good cortical sensation.

There’s no tremor, muscle wasting or fasciculation on both upper limb and lower limb.
No involuntary movements are noted. The tone on all four limbs were normal. Normal deep
tendon reflexes were seen at the brachioradialis, biceps, triceps, knee-jerk and Achilles tendon.
Power on both upper and lower limbs are 5/5. Babinski sign was downgoing on plantar reflexes.
There’s no ankle clonus.

On cerebellar examination, he’s able to tandem walk in a straight line and turn without
falling. There’s no abnormality in gait and balance. Romberg’s test is negative, able to perform
finger nose test, heel to shin test and rapid alternating movements of supination and pronation
of hands.

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Summary of the case:
M, a 10-years old Indian boy with underlying steroid sensitive nephrotic syndrome
presented to the hospital due to complaints of fever, cough and runny nose for 3 days,
postprandial epigastric pain with vomiting for 2 days, 2 episodes of loose stool, decreased oral
appetite, chest heaviness and frothy tea-colored urine since onset of fever. Prior to admission,
urine dipstick started showing proteinuria 2+/3+ since 3 weeks ago upon stopping
corticosteroid maintenance and upon onset of fever. Admission dipstick shows proteinuria of
3+. There’s complaints of increase in weight, back pain, myalgia and arthralgia.
On physical examination, his weight and height are satisfactory, vital signs are normal.
There’s notable facial puffiness with periorbital swelling, and bilateral lower limb swelling, no
sacral and scrotal edema. Throat is mildly injected, tonsils are enlarged (Grade II) with no
exudates. Tenderness is noted over epigastric area. Shifting dullness is positive but no fluid
thrill. No hepatosplenomegaly and renal punch is negative. Otherwise, all other systems are
normal.

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Provisional Diagnosis:
Relapse of steroid-sensitive nephrotic syndrome secondary to upper respiratory tract infection
with primary peritonitis and suspected septic arthritis
As M was previously diagnosed with nephrotic syndrome, this is postulated as a relapse
as there’s 3+ protein on urine dipstick for more than 3 consecutive days. In addition, M presents
with swelling over the lower limbs, around the orbit and facial puffiness, thereby supporting
the diagnosis of nephrotic syndrome which classically presents with edema. M has a positive
family history of nephrotic syndrome as well (his uncle), making the diagnosis of nephrotic
syndrome indisputable in the absence of secondary systemic diseases (Systemic lupus
erythematous, vasculitides etc). Judging from past medical history, M is considered to have
frequent relapse as there’s more than 2 relapses within 6 months of initial corticosteroid
response. As noted in the past, M has attained complete remission within 4 weeks of
corticosteroid therapy, hence he’s considered to be steroid-sensitive.
As he presents with cough, runny nose and fever, this relapse might be secondary to
upper respiratory tract infection which is suspected in this case despite no sore throat. On
physical examination, the mildly injected throat and enlarged Grade II tonsils favours the
diagnosis. As nephrotic syndrome complicates by increasing risk of infection, this supports the
idea that this relapse occurred secondary to upper respiratory tract infection. In fact, M has
stopped corticosteroid therapy, thereby increasing the risks. According to an observational
study from India observing children with nephrotic syndrome who were not receiving
corticosteroid therapy, 32% of the children presents with upper respiratory tract infection.
As there’s intercurrent infection, the persistent 2+/3+ protein on urine dipstick might
suggest breakthrough proteinuria. However, the child has edema and despite the resolved fever,
proteinuria remains, thereby excluding breakthrough proteinuria and this episode should be
treated as a relapse.
From the history, fever and abdominal pain points towards primary peritonitis as a
complication of his nephrotic syndrome. The rapid fluid accumulation causes positive shifting
dullness and abdominal tenderness.
His history of presenting arthralgia and myalgia must prompt the possibility of septic
arthritis as complication of infection following nephrotic syndrome due to loss of protective
immunoglobulins in the urine.
As there’s gross hematuria from the observed tea-colored urine, although more
commonly associated with glomerulonephritis, it can happen as well very rarely, in idiopathic
nephrotic syndrome.

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Differential Diagnosis:

Complications of chronic corticosteroid therapy (steroid toxicity)

M’s presenting signs and symptoms must not rule out possibility of corticosteroid
overdose (steroid toxicity). As he was on long term corticosteroid maintenance, despite
stopping medication for one month, the prolonged use might cause weight gain, nausea,
vomiting, abdominal pain and leg swelling, all of which M presents with. As M complaints of
blurring vision, cataract must be suspected as a side effect of long term use of prednisolone as
well. Long term corticosteroid might cause osteoporosis resulting in myalgia and arthralgia.
Steroid toxicity also increases susceptibility to infection, such as in M’s case where symptoms
of upper respiratory tract infection is present. Other warning signs of steroid toxicity such as
hypertension, ulcer, impaired glucose metabolism, acne and hirsutism which is absent in M
must not be missed.

Chronic hepatitis

As there’s hematuria and symptoms of nephrotic syndrome present, nephrotic


syndrome secondary to chronic hepatitis B or C infection must be ruled out. Loss of immune
factors might predispose one to increased risk of hepatitis infection. As M has a history of
frequent relapse, doing a hepatitis B and C screen can be useful to diagnose whether the
persistent proteinuria is a result of the infection. When present, there are symptoms of acute
infection including fever, nausea, vomiting, abdominal tenderness, all of which are suggestive
in M’s case.

Allergic reactions

In this case isolating out the proteinuria, M presents with signs and symptoms of allergy
such as runny nose, cough, nausea, vomiting and swelling. This serves as a differential
diagnosis to the oedematous presentation. However this is a diagnosis of exclusion as M was
already previously diagnosed with nephrotic syndrome, and this swelling is a result of the
glomerular proteinuria and glomerular structural change. Allergic reaction on top of the
nephrotic syndrome as a cause of his signs and symptoms are highly unlikely. M has no history
of allergen exposure as well, and the precipitating signs are not severe.

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Sinusitis

As a differential to tonsillopharyngitis, sinusitis might the cause of his acute


presentation causing the relapse to occur. Points in M’s history that suggest sinusitis as
productive cough, watery nasal discharge, fever for at least 3 consecutively days. Complication
of intracranial extension leads to vomiting and periorbital swelling. This must be ruled out
against other causes of upper respiratory tract infection in this case.

Streptococcal pharyngitis leading to nephritis

Putting aside the previous diagnosis of nephrotic syndrome, streptococcal nephritis can
be considered in this case as the clinical picture resembles. M has periorbital puffiness,
hematuria, edema (periorbital and facial puffiness) and proteinuria (which can reach nephrotic
range). In addition to that, he has symptoms of upper respiratory tract infection (fever, cough,
runny nose). Hence streptococcal infection of the throat must be ruled out through throat swab
and antistreptolysin O/anti D-nase B titres. However, oliguria, hypertension are not present.

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Relevant Investigations done:

1. Complete blood count and white blood cell differential count: Routine investigation to
determine white blood cell count for current infection and also to determine the
severity of infection present. The differential white count allows to differentiate viral
infection and bacterial infection. A rise in platelet count is also useful in identifying
platelet aggregation in the setting of inflammation. Abnormality in haemoglobin level
may present in setting of nephrotic syndrome.
2. Renal profile: To monitor proper functioning of the kidneys through observing serum
creatinine and to rule out acute kidney injury as complication of nephrotic syndrome
3. Urinalysis to detect amount of proteinuria for evaluation of nephrotic syndrome and
detect urine casts which may present in nephritic syndrome.
4. Liver function test to detect hypoalbunemia and hypogammaglobulinemia to confirm
nephrotic syndrome and to evaluate extend of relapse

Result of Investigations:

1. Full blood count on 29/8/2018

Blood component Results Reference range


RBC 5.38 x 106 /μl 4.53- 5.95
Hb 14.3 g/ dL 13.5-17.4
Hematocrit 42.0 % 40.1- 50.6
MCV 78 fL 80.6- 95.5
MCH 26.6 pg 26.9-32.3
MCHC 34.0 g/dL 31.9-35.3
Platelet count 473 K/μl 142- 350
RDW 12.8 % 12.0 14.8
WBC 7.8 K/μl 4.1-11.4
Immature granulocyte 0.02 -
count
Immature granulocyte (%) 0.3 -
Absolute Neutrophil 3.17 K/μl 3.9- 7.1
Absolute Lymphocyte 3.58 K/μl 1.8- 4.8
Absolute Monocyte 0.7 K/μl 0.4- 1.1

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Absolute Eosinophil 0.4 K/μl 0.0- 0.8
Absolute Basophil 0.0 K/μl 0.0- 0.1
% of Neutrophils 40.4
% of Lymphocytes 45.7
% of Monocyte 8.3
% of Eosinophil 5.2
% of Basophil 0.4

Interpretation:

Red blood cell count, haemoglobin and haematocrit are within normal range of limit. However
red blood cell indices such as MCH and MCV is slightly decrease. As the decrease is minimal,
the reduction in size (microcytosis) or haemoglobin concentration per red blood cell maybe
insignificant, as children are commonly iron deficient without anemia. Full blood count should
be repeated to make sure patient does not present with anemia or other related conditions.

There is marked thrombocytosis from the platelet count, which this is particularly common in
nephrotic syndrome as a compensation to the decrease plasma levels of natural anticoagulants
(free protein S, antithrombin III). The platelet alterations might be also be probably due to
changes in plasma levels of platelet-interfering proteins and lipid changes, as a consequence of
nephrosis.

Absolute neutrophil count is lower than lowest limit, this rules out nephritis and the low
neutrophil count might be associated with neutrophil dysplasia in the setting of severe nephrotic
syndrome. White blood cell count is within normal limits, suggesting that there might not be a
intercurrent infection.

2. Renal profile on 29/8/2018


Components Results Reference range
Urea 5.50 mmol/L 2.76- 8.07
Sodium 137 mmol/L 136- 146
Potassium 4.1 mmol/L 3.4- 4.5
Chloride 104 mmol/L 98- 107
Creatinine 60µmol/L 34-65

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Interpretation:
All components are within reference range indicating no signs of acute kidney injury following
nephrotic syndrome. There’s proper clearance of creatinine. There’s no hyponatremia as well
which can common occur in nephrotic syndrome following hypovolemic stimulation release
of antidiuretic hormone.
3. Urinalysis
Reference
Component Results
Range

29/8 29/8 30/8 31/8 1/9 2/9 3/9

Specific 1.016 –
1.020 1.015 1.016 1.015 1.022 1.021 1.018
gravity 1.022

pH 5.0 6.0 6.0 6.0 6.0 6.0 6.0 4.8 – 7.4

Leucocytes 1+ - - - - - - Negative

Nitrite - - - - - - - Negative

Protein 4+ 4+ 4+ 4+ 4+ 4+ 4+ Negative

Glucose,
- - - - - - - Negative
Urine
Ketone 1+ - - - - - - Negative
Urobilinogen - - - - - - - Negative
Bilirubin - - - - - - - Negative
3+ 2+ 2+ 1+ 1+ 1+ 1+ Negative
Haemoglobin
to trace

Colour Yellow Yellow Yellow Yellow Yellow Yellow Yellow Yellow

Slightly Slightly Slightly Slightly Slightly Slightly


Clarity Clear Clear
cloudy cloudy cloudy cloudy cloudy cloudy

Interpretation:
On admission day, the presence of leucocytes 1+ suggest possible urinary tract infection.
However, on the subsequent days, no leucocyte is present, hence ruling out UTI. There’s
persistent proteinuria (as suggested by 4+ protein) due to nephrotic syndrome over the entire

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clinical course (from admission til day of discharge). No improvement of proteinuria is seen.
The presence of ketone 1+ on day of admission is negligible as there’s no similar observation
over the next few days.
From admission, there’s glomerular hematuria present, most likely as a rare complication of
nephrotic syndrome, suggesting presence of glomerular inflammation.
4. Liver function test

Components Result Reference range


25/7
Total bilirubin (µmol/L) 1.9 <21
Total protein (g/L) 55 64-83
Alkaline phosphatase (U/L) 171 150-420
Albumin (g/L) 25 35-50
Globulin (g/L) 30 28-34
Albumin / Globulin ratio - -
Alanine Transaminase (U/L) 9 5-45

Interpretation:
Total bilirubin is normal. Total serum protein is low following persistent glomerular
proteinuria in this case. There’s hypoalbuminemia as a consequence of loss of albumin in
nephrotic syndrome. It is one of the criteria that defines nephrotic syndrome. Amount of
serum globulin is still within normal limits. This is true in the case of nephrotic syndrome
whereby total globulins are relatively preserved in patients with idiopathic nephrotic
syndrome.

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Management:

The patient was immediately brought to the hospital when his mother noted he still has
fever. When he arrived at the emergency department, his blood was taken for full blood count,
renal profile, liver function test. He was immediately started on prednisolone 75mg once daily.
As his fever has subsided on presentation at ED, no paracetamol was given. Subsequent
management plan includes monitoring urinalysis results through daily dipstick with urine
FEME, nephrotic charting, input/output charting.

As patient is readministered with corticosteroid, signs of steroid toxicity must be


monitored to prevent unfavourable side effects of the corticosteroid use. Other vital signs such
as blood pressure, pulse rate and oxygen saturation were also measured from time to time to
ensure no sudden collapse in vital functions such as in the case of hypovolemia / hypervolemia
which will result in emergency resuscitation. He was started on syrup penicillin-V 500mg BD
(15mg/kg/dose) for 10 days,

Over the course of hospital stay, below are a summary of his vital signs

Date Weight Temp Respiratory Pulse Blood SPO2


(kg) (oC) rate rate pressure (%)
(breaths/min) (bpm) (mmHg) #
29/7/2018 39.1 36.7 22-26 90 137/89 96-99
30/7/2018 39.1 36.5 22-24 85 119/60 98-100
31/7/2018 38.9 37. 22-24 82 91/54 99-100
1/8/2018 39.4 36.2 24-26 81 106/60 98-99
2/8/2018 39.3 37.0 22-30 80 108/70 98-100
3/8/2018 39.3 36.6 24-28 62 101/56 99-100
*Three measurements was taken per day, the range is the lowest limit to the highest limit of
the measurements taken #Taken at night

From the 1st day of admission to the 6th day of admission, his vital signs are normal.
There are no signs of hypovolemia (poor pulse volume, hypotension, hemoconcentration).
Blood pressure does not show any signs of hypervolemia as well. Upon admission, patient
was no longer febrile. Daily weight charting was done to ensure no fluid overload which
might significantly increase the weight.

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Clinically, his facial puffiness and periorbital swelling was resolving over the course.
However, bilateral lower limb pitting edema was present from day 1 to day of discharge with
no improvement seen, the level of edema was always up to the mid shin. As for abdominal
pain, there was no longer any complaint and abdominal tenderness was only observed once
on day 3 after admission. During the 6 days, mild ascites was always present from the
positive shifting dullness, and fluid thrill was not demonstrable. Otherwise, he looks pink,
appear alert and conscious, very playful, tolerating his meals well, and have normal urine and
bowel output over the span of 6 days. Starting day 5 onwards, his throat is no longer injected
and tonsils have recovered.

Over the course of prednisolone induction, there was no improvement seen (refer
above urinalysis results), and proteinuria of 4+ persisted. There are no signs of remission
which slight improvement should be seen. Typically patient should normally achieve
remission by 28 days of induction. According to Malaysian protocol, induction for frequent
relapse with oral prednisolone is as follow:

 Initially 60 mg/m²/day ( maximum 80 mg/day ) until remission followed by


 40 mg/m²/EOD (maximum 60 mg) for 4 weeks only.
 Taper Prednisolone dose every 2 weeks and keep on as low an alternate day dose as
possible for 6 months.

As patient is compliant to his medication and clinically he was well, he was allowed to be
discharge with continuation of medication, and scheduled for an appointment in the following
week to review the nephrotic chart and monitor progress.

Final diagnosis

Relapse of steroid sensitive nephrotic syndrome secondary to tonsillopharyngitis

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Discussion of case: A) Discuss on what you have learned from the clinical features of this
case, the diagnostic procedures, management and literature review of the case.

Relevant definitions in our case of nephrotic syndrome are


1. Nephrotic syndrome (NS) - Oedema with protein excretion >40 mg/m2 per h or
urine protein:creatinine ratio ≥2000 mg/g (≥200 mg/mmol) or >3+ proteinuria on
dipstick with serum albumin <2·5 g/dL (25 g/L)
2. Remission - Urine albumin trace or negative on dipstick or proteinuria <4 mg/m2
per h or urinary protein:creatinine ratio <200 mg/g (20 mg/mmol) for 3 consecutive
days
3. Relapse - Urine albumin 3+ or 4+ or proteinuria >40 mg/m2 per h or urinary
protein:creatinine ratio >200 mg/g (20 mg/mmol) for 3 consecutive days
4. Frequently relapsing NS - ≥2 relapses within 6 months of initial response or ≥4 in
any 12 month period
5. Steroid-sensitive NS - attainment of complete remission within initial 4 weeks of
corticosteroid therapy
6. Steroid-dependent NS - 2 consecutive relapses occurring while weaning to
alternate day steroids or within 2 weeks of steroid discontinuation
7. Steroid-resistant NS - Persistent proteinuria despite 60 mg/m2 or 2 mg/kg for 8
weeks, after ensuring no infection or non-adherence to medication

In this case, primarily we are looking at the frequent relapse of nephrotic syndrome
which becomes our concern. And the present patient, M received inappropriately prolonged
therapy with daily prednisolone resulting in severe steroid toxicity. Following stoppage of
corticosteroid therapy, he showed a fast relapse, which is a predictor of frequent relapses and
thereby further steroid side effects. The management of this patient therefore comprises of
induction and maintenance of remission, and prompt therapy for complications of the nephrotic
state. Since the disease is expected to resolve by adulthood in most children with steroid
sensitive nephrotic syndrome, limiting adverse effects of medications is an important objective
of management.
In my opinion there’s a lack of investigation done, to support the current diagnosis.
Only full blood count, renal profile, urine FEME, and liver function test is being done, which
this cannot exclusively rule out other mentioned differential diagnosis. Although this patient

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has already been diagnosed with nephrotic syndrome, it is important that we carry out the below
investigations as well to evaluate renal function and estimate the severity of illness
1. 24-hour urine collection to measure urine protein excretion
2. First morning void to measure urinary protein to creatinine ratio. This more accurately
quantifies the amount of protein loss in relation with creatinine
3. Serum cholesterol to identify hyperlipidemia as it is a characteristic feature of nephrotic
syndrome, the increase in cholesterol is inversely correlated to the serum albumin
concentration
4. Erythrocyte sedimentation rate or C-reactive protein to estimate extend of inflammation
as patient presents with hematuria
5. Blood tests include antinuclear antibody level to rule out systemic lupus erythematosus
(SLE), and serology for hepatitis B, C, and human immunodeficiency virus (HIV)
which can cause secondary nephrotic syndrome
6. To identify hypovolemia by measuring blood urea nitrogen and glomerular filtration
rate
7. Complement studies to diagnose a specific renal or systemic disease that presents with
nephrotic syndrome. Low C3 levels are typically seen in patients with
membranoproliferative glomerulonephritis (MPGN) and postinfectious
glomerulonephritis, while both low C3 and C4 are seen in patients with lupus nephritis.
Serum complement is normal in patients with idiopathic nephrotic syndrome

For M, the chief concern for him is the peripheral edema and possible signs of sepsis
(peritonitis/ septic arthritis), hence our management should aimed at reducing them. Oral
corticosteroids are still the most effective agents for inducing remission in M with steroid
sensitive disease. The response to therapy with prednisolone is predictable and rapid (8-14
days). In terms of alternative, other corticosteroid preparations including IV
methylprednisolone and deflazacort are not recommended. Similarly, there is no experience
with the use of cyclophosphamide. levamisole and mycophenolate mofetil for inducing
remission in such children. The other class of agents that might be considered for inducing
remission is calcineurin inhibitors (cyclosporine, tacrolimus), which reduce proteinuria by 2-4
weeks and induce complete remission by 6-8 weeks. However, the slower response to therapy
and associated adverse effects do not justify their use in patients with steroid sensitive nephrotic
syndrome.

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According to pediatric protocol and recommended practice by American Academy of
Pediatrics, M should be treated with prednisolone in the standard dose until remission, and
then on alternate days for 4 weeks. Subsequently, in order to maintain remission, therapy with
oral cyclophosphamide (2-2.5 mg/kg daily) and alternate day prednisolone (20-30 mg/m2) is
recommended. Judging by his previous course of prednisolone and how its tapered, it is not
particularly efficient in promoting remission and preventing relapse which is what we hope for
this patient. A 12-weeks’ course of such treatment is likely to result in sustained remission in
a significant proportion of patients. Therapy with calcineurin inhibitors can considered as well
if either steroid therapy is contraindicated.
To manage the edema, we should recommend M salt and fluid restriction with addition
of a loop diuretic (furosemide) if his symptoms of edema do not resolve. As diuresis and
natriuresis is not particular important in this case since he does not have severe edema, addition
of albumin infusion is not required.
Apart from that, in M’s case, his nephrotic syndrome may develop into many
complications include protein malnutrition, hypovolemia, acute kidney injury, urinary loss of
hormones, hyperlipidemia and the potential for accelerated atherosclerosis, a tendency to
venous or arterial thrombosis, and increased susceptibility to infection. Hence M is given
penicillin prophylaxis, and this should the standard of care.

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B) Also carry out a discussion on Professionalism, ethical issues, professional judgment,
communication issues, spiritual/traditional/alternative medicine (whichever relevant), and
lifelong learning issues which are relevant to this case.

In this case, M’s nephrotic syndrome relapse was primarily due to defaulting on follow
up, not seeking medical attention when urine dipstick had showed 2+/3+ for more than 20 days.
The biggest challenge here perhaps is the issue of compliance and lack of patient education
that is needed to ensure good clinical outcome. As we can observe from his progress notes in
the past medical history section, we can conclude that patient is ignorant towards his
deteriorating condition, despite alarming proteinuria seen on dipstick. This instils the
importance of a good doctor-patient relationship, how it’s vital to enhancing compliance with
medical instructions and advice. M would not have suffered so many episodes of relapse had
it not been for the lack of patient centred focus.
Talking about patient-centred focus, it focuses on three key areas, the patient and doctor
mutually: defining the problem; establishing the goals of treatment and/or management; and
identifying the roles to be assumed by patient and doctor
As a doctor, we need to find a common ground with our patients, to understand why
the patient is defaulting on follow-up appointments, their difficulty in seeking medical attention
when needed, why the patient is not actively participating in the effort of promoting his/her
own health, whether was there any healthcare experiences that leads to their poor conformity.
We need to understand them, and seek ways to improve their liability and burden if they could
not meet the basic privileges of obtaining healthcare. Perhaps there’s some truth to the saying
of always “understand our patients as a whole”, only through that, we can know the deep
struggles of our patients. In fact, many of the healthcare related issues nowadays could be
resolved if, healthcare providers like doctors, spend a little bit more time to holistically
passionately care for the patient.
Some of the skills to enhance compliance can be be taken into consideration in this case
 Giving rationale for why M must follow the prescribed dosage of prednisolone
accurately
 Educate M and his mother on the potential side effects of the medication and its harm
if misused
 Explaining the predictors of relapse so M and his mother would know when to seek
medical attention when their signs arise

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 Explaining the importance of keeping his nephrotic syndrome under control to
minimize injury to his kidney and to prevent complications such as hypovolemia,
infections, thromboembolism

 Allow M and his mother to repeat advices and to check on their understanding
 Show empathy and explain to M and his family the importance of resolving financial
burden through possible means e.g. applying financial aid from Jabatan Kebajikan
Masyarakat to ensure their basic medical needs could be met
 Taking their feedback in future visits

Managing patients with nephrotic syndrome remains difficult for the practicing
paediatrician. This often young patient population is faced with a debilitating, relapsing and
remitting disease with non-specific treatment options that sometimes could be poorly tolerated.
Clinicians managing these complex condition with frequent relapse must attempt to apply
disease-specific evidence while considering the individual patient's clinical and personal
situation.
The frequent hospital admission and relapse could result in a poor quality of life, and
causing M to miss attendance in school. It is importance that as a clinician, we take this into
consideration when deciding the best treatment for them, reducing the complexity and duration
of care needed. Although it has rather good prognosis, but the impact on social functioning of
the patient should not be neglected.
As patient is discharged despite persistent protein 4+ in urine dipstick, I’ve learned the
principle of “continuity of care”. As this patient has been under my watch ever since I clerked
this patient on the day of his admission, it is important that I continue to follow up on his
progress even after making a full recovery. This is a particular important value especially in
future practices, that we treat all patients like how we wish we be cared for. To quote, Rachel
Naomi Remen simply put it
“The healing of our present rounded may lie in recognising and reclaiming the
capacity we all have to heal each other, the enormous power in the simplest of human
relationship, the strength of a touch, the grace of someone else taking you just as you are
finding in you an unexpected goodness”.
Finally ethically, the patient provided verbal consent, as is common practice in the
country, to report his clinical case in this case write up.

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References:
1. Pediatric Protocol for Malaysian Hospitals
2. Illustrated Textbook of Paediatrics, 5th Edition
3. Nelson’s Textbook of Paediatrics, 20th Edition
4. DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 - .
Record No. 900359, Nephrotic syndrome in children;
5. Nephrotic syndrome in children: prediction of histopathology from clinical and
laboratory characteristics at time of diagnosis. A report of the International Study of
Kidney Disease in Children Kidney Int, 13 (1978), pp. 159-165
6. EM Ruth, MJ Kemper, EP Leumann, GF Laube, TJ Neuhaus Children with steroid-
sensitive nephrotic syndrome come of age: long-term outcome. J Pediatr, 147 (2005),
pp. 202-207
7. PedsCases: Evaluation of Proteinuria by Peter Gill and Dr. Verna Yiu for
Pedscases.com. January 10, 2010.
8. Hinkes BG, Mucha B, Vlangos CN, et al. Nephrotic syndrome in the first year of life:
two thirds of cases are caused by mutations in 4 genes (NPHS1, NPHS2, WT1, and
LAMB2). Pediatrics 2007; 119:e907
9. White RH, Glasgow EF, Mills RJ. Clinicopathological study of nephrotic syndrome in
childhood. Lancet 1970; 1:1353.
10. Gipson DS, Massengill SF, Yao L, et al. Management of childhood onset nephrotic
syndrome. Pediatrics 2009; 124:747.
11. Barness LA, Moll GH, Janeway CA. Nephrotic Syndrome. I. Natural History of the
Disease. Pediatrics 1950; 5:486.
12. KDIGO clinical practice guideline for glomerulonephritis. Kidney Int Suppl, 2 (2012),
pp. 139-274
13. F Fakhouri, N Bocquet, P Taupin, et al. Steroid-sensitive nephrotic syndrome: from
childhood to adulthood. Am J Kidney Dis, 41 (2003), pp. 550-557

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