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School of Medicine

Bachelor of Medicine; Bachelor of Surgery

Paediatrics 2
Case Write-Up
Name: Charlotte Ee Sze Lyn
Student ID: 0313464
Date of Posting: 14/11/16-23/11/16
Case write-up number: 1
Item
History including patient data
Physical Examination
Diagnosis/Differential Diagnosis
Investigation
Management
Discussion including evidence-based
medicine (EBM) and references
Total
Name of Lecturer:

Date:

History Taking
Identification Data
Name

: SNA

R/N

: SB00047407

Age

: 12 years and 11 months old

Gender

: Female

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Marks
Allocated
20
20
15
10
15
20
100
Signature:

Marks
Awarded

Ethnicity

: Malay

Citizenship

: Malaysian

Address

: Payu Jaras

Date of admission

: 24th November 2016

Date of clerking

: 25th November 2016

Source of history

: Patient, patients files and patients mother

Chief Complaint
The patient SNA is a 13 year old Malay girl who is a known case of steroid dependent
nephrotic syndrome with frequent relapses. She was referred to Hospital Sungai Buloh and
presents with a chief complaint periorbital swelling for 2 days followed by general body
swelling.

History of Presenting Illness


The patient was previously well until 3 years ago in October 2013 when she developed
gradual onset of generalized swelling for 2 days. The swelling started at the periorbital area
then to facial puffiness and progressed to the abdomen then to the limbs. Prior to developing
these symptoms, she had preceding diarrhoea, fever and cough which lasted for a week prior
to admission. The fever was initially measured by the mothers crude touch and she also
noticed SNA had a decrease in activity. SNAs mother cannot remember the documented
temperature at the time but feels the fever was low grade. The fever was continuous in nature.
There was no diurnal variation of the temperature and there were no chills or rigors. There is
no excessive sweating or night sweating. During this period of illness, the patient appeared to
be less active but is able to do daily activities. There was no loss of appetite and no weight
loss.
The patients cough was non-productive with no diurnal variation. The cough had intermittent
short bouts of coughing without any obvious triggers. There was no special characteristic of
the cough and the mother cannot remember if there was diurnal variation. SNA also suffered
from diarrhoea in which she had loose stool for increased frequency of defecation. However,
the patient and her mother cannot remember much else about the diarrhoea. There is no
recollection of the stool colour, consistency or amount but it was not blood or mucus stained.
After developing generalized swelling, SNAs mother rushed her to Hospital Sungai Buloh
and after the doctors assessment was diagnosed with nephrotic syndrome and consequently
started on oral steroids to which she was responsive to. She achieved remission for 2 years.
However 2 years later, in February 2015, my patient had her first relapse in which following
an upper respiratory tract infection, she developed generalized swelling. She achieved
remission after being reinduced with oral prednisolone 60mg/m2/day and completed her
maintenance oral prednisolone (40mg/m2/day). However as they were tapering down the

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dose she had another relapse. Her mother could not remember the exact dosage at which she
developed the relapse.
Her second relapse was in May 2015, this time she had a relapse following an episode of
acute gastroenteritis. The relapse was found by breakthrough proteinuria on home dipstick
monitoring where it was more than 2+ for 3 consecutive days. She had also developed facial
puffiness. She was admitted for 10 days and was given oral antibiotics, a diuretic and
prednisolone. She achieved remission after being reinduced with oral prednisolone
60mg/m2/day and completed her maintenance oral prednisolone (40mg/m2/day). Once again
as they were tapering down the dose she had another relapse. Her mother could not remember
the exact dosage at which she developed the relapse.
Her third and fourth relapses were in August and October 2016 respectively. These episodes
also occurred after a preceding upper respiratory tract infection and she responded steroid
treatment as she did with the previous 2 episodes. After her third relapse, she achieved
remission in only 3 days. However she subsequently defaulted follow up and her maintenance
dose of steroids. After her fourth relapse, the mother did not taper down the pills and
continued giving high dose prednisolone without tapering down until the pills ran out because
she had run out of urine dipsticks and could not afford more at the time due to financial
constraints. However my, patient did not have any lower back pain, abdomen pain, severe
vomiting and diarrhea, loss of consciousness or other symptoms suggestive of an Addisonian
crisis.
SNAs fifth relapse was in March 2016. At the time, she once again presented with
generalized edema associated with fever and diarrhoea for 3 days. Her urine dipstick readings
had been 4+ for 3 days. She had had sick contact with her sibling prior to developing the
symptoms. She achieved remission after being reinduced with oral prednisolone
60mg/m2/day and completed her maintenance oral prednisolone (40mg/m2/day) before
gradually tapering down the medication.
The sixth relapse was in May 2016, the seventh in August 2016, the eighth in September
2016 and the ninth in October 2016. They were all similar to the previous episodes with each
episode being preceded by either an upper respiratory tract infection or acute gastroenteritis
and characterized by either by breakthrough proteinuria which becomes persistent proteinuria
or generalized swelling which started around the eyes and facial puffiness. She achieved
remission after being reinduced with oral prednisolone 60mg/m2/day but would relapse either
while on her maintenance oral prednisolone (40mg/m2/day) or while tapering down further.
This current admission is my patients 10th relapse. Initially she was supposed to follow up
earlier as they were to discuss the oral prednisolone dosage with the doctor to see if she could
start tapering down the dosage but defaulted due to her mother being too busy looking after
my patients sibling as he had fallen sick. Regarding her 10th relapse, 1 week ago, she
developed a fever after sick contact with her sibling. The fever was low-grade (measured by
mothers crude touch), continuous, not associated with chills or rigors and had no diurnal
variation. She also developed diarrhoea 4 days ago where there was loose stool and an
increased frequency of defecation where she went thrice a day to the toilet instead of once
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which was her usual. The stool was neither blood nor mucus stained. 2 days before
admission, she also developed sudden periorbital swelling which she noticed on waking up
which progressed to facial puffiness, then, abdominal and labial swelling, then swelling of the
limbs. At the time, my patient was on a dose of 35mg oral prednisolone EOD. Upon
developing the swelling, my patient did a urine dipstick test which revealed 4+ proteinuria.
She was then brought to the emergency department.
Throughout these 3 years there has been no report of fullness and rounding of the face (socalled "moon facies"), added fat on back of neck (so-called "buffalo hump"), no easy bruising
of the skin, no stretch marks (striae), no excessive weight gain, most marked in the abdominal
region, no excess hair growth, no muscle weakness, no hypertension or hyperglycaemia or
other symptoms suggesting corticosteroid toxicity. There was also never any tea coloured
urine though at times her urine did appear cloudy.
My patient also denies having a facial rash in the distribution of her cheeks and nasal bridge
especially after exposure to sunlight, joint pain, balding, oral ulcers or joint swelling.

Systematic Enquiry
Systemic review was not significant. Other than the symptoms mentioned above, the
following negatives were elicited:
Central nervous system
Respiratory system

Absence of weakness, faint, behavioural changes and seizures


Absence of chest pain, shortness of breath, tachypnea or
noticeable chest recession.

Cardiovascular system

Absence of palpitations, syncope and evident oedema

Gastrointestinal system
Genitourinary system

No constipation.
Absence of change in frequency of micturition, hesitancy,

Musculoskeletal system

haematuria, and dysuria


Absence of joint pain, movement limitations and bruising

Past Medical History


There is no past medical history other than that mentioned as part of the history of presenting
illness (kindly refer above).

Birth History
The mother had obtained regular antenatal care with booking done at around 16 weeks period
of gestation and frequent ultrasound scans done with no significant findings. She took
supplements regularly. She received immunisation against all significant infections.
Screening was also done on the pregnant mother but the result is insignificant. During
pregnancy, the mother did not suffer from any maternal illness such as chickenpox, rubella
and toxoplasmosis. The pregnant mother was not exposed to drugs or alcohol but was
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exposed to passive smoking. The mother did not suffer from any pregnancy complication
such as pregnancy induced hypertension or gestational diabetes.
The mothers age during the delivery of NSB is 18 years old. The patient was born by at term
with a birth weight of 3.2kg. The delivery method is spontaneous vaginal delivery with no
intervention (forceps or ventouse) done. The baby cried immediately after delivery. There
was no need for NICU admission.
Postnatally, the baby was healthy but developed neonatal jaundice at day 2 of life but
recovered after phototherapy was given. The jaundice lasted 2 weeks.

Immunisation History
The immunisation is up to age with the last vaccination being at the time where the patient
was 13 years old. Unfortunately, the mother is unable to recall the name of the vaccine given
but according to that Malaysian paediatric protocol it was probably the HPV vaccine. The
patient has not taken any additional vaccines such as the pneumococcal or chicken pox
vaccine.

Feeding History
The patient was exclusively breastfed for about 6 months. Formulated milk was never used.
Weaning was started when the patient was 6 months old. But supplementary breastfeeding
was continued until the patient was 6 years old. The patient now consumes an adult diet of
solid food. There was no feeding difficulty by the mother. A typical diet consists of rice, meat
and vegetables. The patient is not a picky eater.

Developmental history
The development of the patient is appropriate to her age. The mother reports that the patient
performs well at school and gets along well with her siblings and peers. There is no report of
peer pressure or sibling rivalry. My patient has been in the first class of her school since
Standard 3 and performed reasonably well in her UPSR(3As2Bs) despite frequent absences
from school.(due to her frequent admissions)
Gross motor: The patient wass able walk without support by the age of 10 months.
Vision and fine motor: The patient was able to hold a pencil and and scribble by the age of
18 months.
Hearing, speech and language: the patient could speak 3-4 meaningful words by the age of
9-10 months.
Social and emotional: She could dress and feed herself by the time she was 2 years old. She
was toilet-trained by the age of 18 months. She has never had any difficulty forming
friendships with other children.

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Drug History and Allergic History


The patient is on frequent if not constant steroid use. However, there have been no signs of
steroid toxicity. She is currently on high dose oral steroids(60mg/m2/day) and a prophylactic
dose of oral penicillin V.
She does not take any other drugs, supplements or traditional medicine. There is no history of
drug or food allergy.

Family History
The patient is the oldest child among 4 siblings of a non-consanguineous marriage. The
patient has 2 younger sisters of ages 10 and 5 respectively and a younger brother who is 8
years old. Her brother was born with cleft lip but after surgery has now made a full recovery.
Her siblings are all currently are healthy.
My patients father was a heavy smoker and had underlying diabetes and hypertension. He
passed away recently at the age of 32 due to a myocardial infraction. He was a manager at
Syabas Sdn. Bhd. before his untimely demise. Currently, my patients mother who is 32
years old with no underlying diseases is the sole breadwinner of the family.
SNAs maternal aunt suffered from nephrotic syndrome as a child but recovered by
adolescence. Another maternal aunt had cervical cancer but is now under full remission.
SNAs paternal uncle is a known case of SLE with renal involvement. There is no other
record of hereditary diseases such as coronary heart disease and asthma in the family.
Mother, 31

FATHER, 32

SNA, 13

Sister, 10

Brother, 8

Key
Male
Female

1st generation
Deceased
2nd generation

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Sister , 5

Social History
My patients father is deceased making NSAs the mother the sole breadwinner of the family.
Her highest level of education was Form 2. She has her own food stall and earns around 300
ringgit per day but her business is constantly interrupted by her daughters frequent
admission because she will choose to take care of her rather than go to work when her
daughter develops symptoms.
However, the family is currently coping reasonably well financially because NSAs father had
life insurance which they claimed and also because PERKESO gives them a certain amount
each month. However, my patients mother worries about furthering her childrens education
after they finish high school.
SNAs father used to smoke heavily but did not consume alcohol. NSAs mother has never
smoked nor consumed alcohol.
The patient stays with her nuclear family at Payu Jaras in a quarters house. There are 5
residents in the house. There are no windows in the house so it is a very stuffy and dusty
environment. They have 17 cats that wander in and out of the house. They do not live in a
dengue prone area. The other children are currently left at home to fend for themselves while
the mother is away because the mother feels they are old enough to look after each other. She
prepares their meals in advance before going to her daughter in the hospital and returns home
by the evening to look after them.
The mother reports that the patient performs well at school and gets along well with her
siblings and peers. There is no report of peer pressure or sibling rivalry. My patient has been
in the first class of her school since Standard 3 and performed reasonably well in her
UPSR(3As2Bs) but she feels that she could have done better if she did not have to be
frequent absent from school (due to her frequent admissions). She plays sports often with her
friends from school and is active in extracurricular activities.

Physical Examination
General examination

On inspection, the patient is sitting comfortably on the bed talking to her mother.
The patient is alert and responsive.
There is marked facial puffiness and prominent periorbital oedema seen
It is difficult to assess the patients build and thus nutritional status due to anarsarca.
However, I feel her nutrional status is only somewhat adequate and this is confirmed
by his weight and height centile which is plotted to be between 50th and 25th

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percentile and below the 5th percentile respectively (refer below). Her height is not up
to the normal standard though her weight falls in the normal range for her age.
The patient is not in any apparent pain respiratory distress.
There is an identification tag on her right wrist.
There is a measuring cup for the patients urine but it is not filled at this point of time
There is also an input output chart which shows the patient is not oliguric but there is
currently positive balance.
There is evidence of buffalo hump, acne, bruises, hirsutism or striae that can be seen.
There is no butterfly rash or alopecia seen.

Anthropometric Measurements
Height : 140.1cm
Dry Weight : 40.9 kg
Current weight: 49 kg
BMI : 20.8 kg/m2
A centile growth chart for her age and gender was plotted, as shown below. She weighed
40.9kg, which was plotted to be between 50th and 25th percentile, while her height was
measured to be 140.1 cm, which was plotted to be below the 5th percentile. Her BMI was
20.8kg/m2 which is plotted to be between the 75th and 50th percentile.

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On examination of the hands,

The palms are warm and pink


There is no pallor in palm
Capillary refilling time is less than 2 second
No leukonychia or koilonychias
No finger clubbing,
No peripheral cyanosis,
No signs of infective endocarditis like Oslers nodes, splinter haemorrhages, Janeway
lesions
Pulse was 80 beats per minute good volume regular rhythm (a point against
hypovolemic shock).

Examination of the face

There is obvious facial puffiness and periorbital oedema


No presence of conjunctival pallor.
No yellowish discolouration of the sclera.
No butterfly rash is seen
There are no mouth ulcers seen
Tongue is not coated and she looks well hydrated
Oral hygiene is good
There is an inflammation of the throat or the tonsils
No central cyanosis
No halitosis was noted.

Examination of the neck


Both the carotid pulses were regularly regular and of good pulse volume. There is no swelling
in the region of the thyroid. There was no cervical lymphadenopathy.

Examination of the lower limbs

There was bilateral pedal edema up to the level of upper thigh


No scars suggestive of impetigo or other infections
No ulcers or gangrene
No signs of limb ischaemia such as cold extremities, loss of hair, shiny skin,
pigmentation

Examination of the lymph nodes

no lymph nodes enlargements are noted

Vital Signs

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Blood pressure
Pulse rate
Respiratory rate
Temperature

: 108/80 mm Hg
: 80 beats per minute
: 20 breaths per minute
: 37 C

All her vital signs are within the normal range for her age. The pulse rate is regularly regular
and of good volume. There is no radio-radio or radio-femoral delay. The vital signs do not
suggest the presence of hypovolemic shock.
Assessment of dehydration
The patient is able to pass urine regularly. There are no sunken eyes. The mucus membrane is
moist as the tongue appears to be well hydrated. The patient does not experience increased
thirst. Examination of vital signs do not reveal any sign of dehydration such as hypotension,
tachypnea, weak and tready pulse. Hence, I would like to commit that my patient SNA is well
hydrated.

Respiratory examination
With how grossly edematous NSA appeared, I was concerned with the possibility of
pulmonary edema but this was ruled out on examination.
Inspection: Chest wall of the patient moves symmetrically. Usage of accessory
muscle to breath is not noted. No visible pulsation or heave over the chest wall. The
chest wall is ellipsoidal in shape. There is no pectus excavatum or pectus carinatum.
Palpation: No deviation of tracheal position. Chest wall expansion is symmetrical.
Vocal tactile fremitus was normal.
Percussion: Percussion reveals a resonant note at all lung fields except in the region
of cardiac and hepatic dullness.
Auscultation: Breathing sound is normal. Intensity of breathing sound equal on both
sides indicating air entry is equal on both sides. There is no wheeze, rhonchi, stridor,
crackles or pleural rub noted. Vocal resonance is normal.

Cardiovascular examination
Inspection: No significant findings. No elevated jugular venous pressure.
Hepatojugular reflex was negative. No visible pulsation or heave over the chest wall.
No sign of precordial bulge. Apex beat is not visible.
Palpation: Apex beat is not displaced ie it is at the 5th intercostal space at just medial
to the midclavicular line. No palpable thrill is felt. Left parasternal heave is not noted.
No tenderness is felt at right hypochondrium upon deep palpation.
Auscultation: S1 and S2 heart sound can be heard clearly with normal intensity. S3
and S4 heart sound cannot be heard. There is no murmur.

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Gastrointestinal examination
Inspection: The abdomen was distended symmetrically. The umbilicus was centrally
placed and inverted. There is a hypopigmmented area at the left lumbar area and a
mole just medial to it. There is another mile just below the umbilicus. All regions of
the abdomen moved equally with respiration and there were no rashes, distended
veins, surgical scars, visible peristalsis, or visible pulsations seen.
Palpation: The abdomen was soft and non-tender on superficial palpation, while on
deep palpation; the liver and spleen were not palpable. Both the kidneys were not
palpable. There were no other masses felt.
Percussion: Shifting dullness and fluid thrill were both noted to be positive,
indicating the presence of ascites. Liver span was 9 cm which was normal for her age.
Splenomegaly not elicited as there was resonance in Traubes space. Renal punch was
unremarkable.
Auscultation: Bowel sound is normal at about 6 per minute. There is no aortic or
renal bruit. No venous hum could be auscultated.
Inspection of external genitalia, hernia orifices and a per rectal examination was not
performed to protect my patients modesty. However the patient clains there was labial
swelling.

Nervous System Examination


Patient was fully conscious and orientated to time, place and person.
His higher functions (Language, and memory) were intact.
Cranial Nerves: All cranial nerves were normal.
Motor system: Normal
Sensory system: Normal

Summary
My patient is a 13 year old female child who presented with sudden periorbital swelling
which progressed to facial puffiness, then, abdominal and labial swelling, then swelling of the
limbs associated with 4+ proteinuria; the symptoms were preceded by a one week history of
fever and diarrhoea. She was diagnosed with nephrotic syndrome at the age of 10 but has no
features suggestive of secondary causes of nephrotic syndrome such as SLE and currently she
has frequent relapses and is steroid dependent.
Positive physical findings include prominent periorbital oedema and facial puffiness, pedal
oedema up to level of upper thigh, a distended abdomen, positive shifting dullness and fluid
thrill.

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Provisional Diagnosis
Steroid Dependent
relapses

Nephrotic

Syndrome

with

frequent

Favourable points that point towards the diagnosis of nephrotic syndrome are:

There is edema which initially develops around the eyes then spread progressively
downwards to the rest of the face, the abdomen, the genitals, then the arms and legs.
Due the edema, there may be associated increase in weight which is seen in my
patient(a 8.1 increase in body weight since the onset of edema)
The presence of ascites as evidenced by positive shifting dullness and fluid thrill
The presence of pitting pedal edema
Urine dipstick was 4+ for protein on admission
Cloudy appearance of urine
The fact that she is a known case of nephrotic syndrome

However, there are also points against this diagnosis, namely:

There was no report of frothy urine


The patient was 10 years old when she was initially diagnosed which is rare as a vast
majority of patients with nephrotic syndrome have minimal change disease which is
typical in a younger age group(3-5 years old) however this can be explained as a
possible case of a secondary nephrotic syndrome particularly of concern in my patient
because of the fact of my patients age, gender and family history, systemic lupus
nephritis causing nephrotic syndrome. However, she exhibits no sign of this disease.

Points for the diagnosis of Steroid Dependent Nephrotic Syndrome with frequent relapses

The patient has had many relapses(more than relapses 4 in a 12 month period) which
fits the criteria for frequent relapser
She has had more than 2 consecutive relapses occurring during a steroid taper which
fits the criteria of a steroid dependent nephrottic syndrome

Differential diagnosis
Steroid Resistant Nephrotic Syndrome
Favourable points that point to nephrotic syndrome are:

There is edema which initially developed around the eyes then spread progressively
downwards to the rest of the face, the abdomen, the genitals, then the arms and legs
which is typical of nephrotic syndrome.
Due the edema, there may be associated increase in weight which is seen in my
patient(a 8.1 increase in body weight since the onset of edema)
The presence of ascites as evidenced by positive shifting dullness and fluid thrill
The presence of pitting pedal edema

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Urine dipstick was 4+ for protein on admission


Cloudy appearance of urine
The fact that she is a known case of nephrotic syndrome

However, there are also points against the diagnosis of nephrotic syndrome, namely:

There was no report of frothy urine


The patient was 10 years old when she was initially diagnosed which is rare as a vast
majority of patients with nephrotic syndrome have minimal change disease which is
typical in a younger age group (3-5 years old) however this can be explained as a
possible case of a secondary nephrotic syndrome particularly of concern in my patient
because of the fact of my patients age, gender and family history, systemic lupus
nephritis causing nephrotic syndrome. However, she exhibits no sign of this disease.

Points against steroid resistant nephrotic syndrome:

My patient NSA has always been responsive to steroid therapy that is there will be a
response to the initial 4 weeks treatment with prednisolone at 60mg/m2/day which
will not happen if she is steroid resistant.
She will usually have a relapse once she is tapering down the steroids which is more
indicative of being steroid dependent.

Systemic Lupus Erythematosus (Lupus Nephritis)


Points in favour of this diagnosis are:

A new onset kidney disease can be a presenting feature of childhood onset systemic
lupus erythematosus.
SLE is more prevalent in females; more than 90% of cases occur in women
There is edema which initially develops around the eyes then spread progressively
downwards to the rest of the face, the abdomen, the genitals, then the arms and legs.
Due the edema, there is an increase in weight which is seen in my patient(a 8.1
increase in body weight since the onset of edema)
The presence of ascites as evidenced by positive shifting dullness and fluid thrill
The presence of pitting pedal edema
Urine dipstick was positive for proteinuria on admission ive s posit
Familial histor

Points against the disease are:

Patient does not have any other signs or symptoms suggestive of this diagnosis such
as fatigue, joint pain, malar rash, and fever
Absence of hematuria
Absence of oliguria
My patient is normotensive
Edema is not usually as severe as it is in a child with nephrotic syndrome

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Proteinuria was 4+. In nephritic syndrome, children usually have a lesser degree of
proteinuria.

Post-Streptococcal Glomerulonephritis
Points in favour of this diagnosis are:

There is edema which initially develops around the eyes then spread progressively
downwards to the rest of the face, the abdomen, the genitals, then the arms and legs.
Due the edema, there may be associated increase in weight which is seen in my
patient
The presence of ascites as evidenced by positive shifting dullness and fluid thrill
The presence of pitting pedal edema
Presence of proteinuria
Many of the episodes of swelling are usually preceded by a upper respiratory tract
infection
It is commonest in older children (6-10 years of age)

Points against the diagnosis are:

Absence of hematuria
Absence of oliguria
My patient is normotensive
Lack of impetigo scars (Group A B-hemolytic Streptococcus-commonest cause of
post-streptococcal glomerulonephritis)
Edema is not usually as severe as it is in a child with nephrotic syndrome
Proteinuria was 4+. In nephritic syndrome, children usually have a lesser degree of
proteinuria.

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Investigation
Bloods
Full Blood Count
In nephrotic syndrome, full blood count is usually normal except in instances where there
maybe thrombocytosis and an increased haematocrit due to dehydration. Presence of
leukocytosis could be suggestive of glomerulonephritis. As my patients history has me
suspicious of systemic lupus arthritis I would like to assess for hematological changes of SLE
such as anemia, leukopenia and thrombocytopenia.
Hemoglobin
White blood cell
Hematocrit
MCV
MCH
MCHC
RDW
Platelet

Value
12.3
13.85
40
79.3
29.6
360
14.2
381

Normal range
(11.0-16.0)g/dl
(4.0-11.0)109/l
(40-50)%
(76.0-96.0)fl
(27.0-33.0)pg
(320-360)g/l
(11-16)%
(150-450)109/l

Interpretation
Normal
Increased
Normal
Normal
Normal
Normal
Normal
Normal

Evaluation: There is an increase in total white blood cell which may be explained by the fact
my patient had a history of having acute gastroenteritis before developing periorbital
swelling. However, it is important to keep in mind that this can be indicative of a possible
glomerulonephritis. Otherwise, all the parameters are normal.

Liver Function Test


This test is done to evaluate protein levels particularly the albumin level which will be
markedly reduced, typically <25g/L.
Total Protein
Globulin
Albumin /
Globulin Ratio
Total Bilirubin
Alanine
Transaminase
(SGPT)
Albumin
Alkaline
Phosphatase

Value
30.0 g/L
34 g/L
0.9

Normal range
(64-83)g/L
(28-34)g/L
0.8-2.0

Interpretation
Low
High
Normal

3.8 mol/L
9 U/L

(3-21)umol/L
(0-55)U/L

Normal
Normal

6 g/L
135 U/L

(35-50)g/L
(40-150)U/L

Low
Normal

Evaluation: There is hypoalbuminemia that is serum albumin is <25g/L which is one of the
four diagnostic criteria of nephrotic syndrome. There is also reduced total protein which is

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probably attributable to the low serum albumin. The other parameters are within the normal
range.

Renal Function Test


To assess kidney function and to detect any abnormalities or possible kidney failure which
(although highly unlikely in this case), may be a differential diagnosis for anarsarca and also
to detect any electrolyte abnormalities.
This test can also rule out glomerulonephritis by confirming the absence of azotemia as well
as lupus nephritis which presents with increased urea and creatinine.
Urea
Sodium
Potassium
Chloride
Creatinine

Value
4.5
139
4.4
110
34.4

Normal range
(3.5-6.5)mmol/l
(135-145)mmol/l
(3.5-5.0)mmol/l
(98-107)mmol/L
(60-120)umol/l

Interpretation
Normal
Normal
Normal
High
Low

Evaluation: There is increased chloride which may be due to dehydration. There is also a low
creatinine which may be due to the fact my patient is currently in a state of hypoproteinemia.
The other parameters are normal. There is no azotemia or increased creatinine which is a
point against lupus nephritis and glomerulonephritis.

Serum Complement
This test is done to exclude post-streptococcal glomerulonephritis (low C3 normal C4) and
SLE (low C3 and C4).
C3
C4
Evaluation: Serum C3 and C4 levels are normal

Value
1.06 g/L
0.24 g/L

Anti-streptolysin O Titer
A high ASOT level signifies recent streptococcal infection which is suggestive of poststreptococcal glomerulonephritis.
Value
ASOT
100 units/mL
Evaluation: ASOT is negative(<200unit/mL). Post streptococcal glomerulonephritis in
unlikely.

Antinuclear Antibody/ anti-asDNA


The history my patient presents with that is her age at initial presentation, her gender and her
positive family history has me suspicious of SLE which could either cause secondary
nephrotic syndrome or lupus nephritis. Thus, I would like to screen for SLE using this test.
Assessment: This was done by Hospital Sungai Buloh earlier this year in September during a
previous admission for one of her relapses.

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Date:10/9/2016- ANA is negative


The result was negative at the time, however I would like to do the test again to rule out the
possibility of a false negative.

Body Fluids
Urinalysis/UFEME
This is used to detect proteinuria. This is to rule out glomerulonephritis as the presence of
RBC casts is almost pathognomonic of glomerulonephritis. This test can also detect
microscopic hematuria which will be useful for diagnosis nephritic syndrome.
Results: Dipstick done in the revealed protein within the range of 2+ to 4+ for 3 consecutive
days which supports the diagnosis of nephrotic syndrome.

Urine protein: creatinine ratio


This is to obtain evidence of proteinuria. A single spot urine collection is much easier to
obtain than a 24 hour urine sample though in this case my patient is old enough to be able to
do so.
Evalution: The urine protein: creatinine ratio was in the nephrotic range of proteinuria i.e
>200 mg/mmol

24 hour urine protein


This is to obtain evidence of proteinuria.
Evalution: Proteinuria of >40 mg/m2/day was recorded with supports the diagnosis of
nephrotic syndrome.

Additional investigations

Other laboratory tests associated with the presence of inflammation, such as


erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) may also be
used to evaluate a person for SLE.
A lipid profile can be done as nephrotic syndrome presents with
hypercholesterolaemia. This test was not done in my patient during her current
admission.
Renal biopsy would be diagnostic of a primary or idiopathic nephrotic syndrome but
it is mainly indicated when one is proven to be steroid resistant which is not the case
in my patient.

Working Diagnosis

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Steroid Dependent Nephrotic Syndrome with frequent relapses was chosen as the working
diagnosis because it most closely fits the description given in the history and the findings on
physical examination that is the edema that generally begins around the eyes and face before
progressing downwards among other things. In addition, the investigations also support the
diagnosis as there is hypoalbuminemia and proteinuria. My patients fulfills the diagnostic
criteria of nephrotic syndrome because there is a triad of edema, hypoalbuminemia (< 25 g/L)
and proteinuria(>40mg/m2/day). Furthermore, after thorough history taking, physical
examination and investigation, other causes like nephritic syndrome can be ruled out as can
other possible cause of generalized swelling such as congestive cardiac failure, liver failure,
renal failure or rare entities like Alports syndrome.

Management
General Management
1. Patient is admitted in ward 8C for monitoring and further investigations to confirm
this is a relapse rather than a different disease entirely and to exclude causes of
secondary nephrotic syndrome.
2. A normal protein diet with adequate calories is recommended. A low salt diet is
advocated when child has edema.
3. No fluid restriction was done as it is not recommended except in chronic edematous
states.
4. Ventilate on room air
5. Strict input-output(IO) charting and nephrotic charting with blood pressure
monitoring 2-hourly.
6. Daily Urine FEME and weight measurement are ordered
7. Patient was advised on how to do a 24 hour urine collection.

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8. Patients mother is counseled once again about prednisolone treatment, indication of


steroid treatment, benefits and risks of steroid treatment on patients current illness.
Mother is also counseled regarding the need of daily UFEME.
9. Penicillin V 500 mg BD (> 12 years) is recommended at diagnosis and during
relapses, particularly in the presence of gross edema.
10. Reinduction of oral prednisolone
11. Patients general condition and vital signs are monitored every 4 hours to assess
haemodynamic status and to rule out complications like
a. Hypovolaemia
b. Hypervolaemia
c. Thromboembolism
d. Peritonitis
Ideally, I would also like to counsel the mother parents to educate them about the importance
of following the steroid treatment regimen given by the doctor as there has been history of
not tapering the dose because I am concerned about an acute adrenal crisis and offering
options for monetary support as there has been times when the mother could not afford to buy
the urine dipsticks.
In addition, because the patient is short for her age (Height is below the 5th centile), I am
considering that my patient may be exhibiting steroid toxicity. I would like to continue
evaluation to rule out this possibility and if it is confirmed I would like to consider alternate
treatment.

Patient Progress Report


24/11/16
Day 1 of admission
Day 8 of illness

Progress: My patient appeared grossly edematous with prominent periorbital edema, facial
puffiness, labial swelling, clinical ascites and pedal edema but however was otherwise well
and comfortable at rest. There is no sign of shortness of breath, abdominal pain, tachycardia,
cool peripheries or other signs of complications. Currently, my patient is no longer febrile but
still has diarrhea(loose stool 3-4 times a day). Urine diptick was 4+.
Medications prescribed:

Patient is started with oral prednisolone 75mg OD (60mg/m2/day)


Prophylactic antibiotic is given: Oral penicillin V 500mg BD

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Patient was given ORS sachets and taught how to dilute them.

25/11/16
Day 2 of admission
Day 9 of illness

Progress: Patient is not yet improving clinically; periorbital edema, facial puffiness, labial
swelling, ascites and pedal edema has not improved and in fact is worsening. Urine dipstick
maintained at 4+. However, NSA is alert, cheerful and active. Patients vital signs are stable;
she is normotensive, afebrile, no tachycardic or tachypnic. The decision is made to add on
furosemide to reduce the edema although diuretics(according to the Paediatric Protocols For
Malaysian Hospitals) is not necessary in steroid responsive nephrotic syndrome. The patient
is to be monitored closely for signs of hypovolemia during the use of furosemide. Patient
reports frequency of defecation has reduced.
Medications prescribed:

Oral prednisolone 75mg OD (60mg/m2/day)


Prophylactic antibiotic is given: Oral penicillin V 500mg BD
Additional ORS sachets
IV furosemide 20mg/2ml

26/11/16
Day 3 of admission
Day 10 of illness

Despite adding furosemide, patient is still not improving clinically; the degree periorbital
edema, facial puffiness, labial swelling, ascites and pedal edema is unchanged and mother
noticed that patients urine output decreased markedly. However, patient still appears
comfortable, her vitals are stable and her diarrhea has resolved. It was decided to add human
albumin as well to reduce the edema.
The treatment of edema in patients with nephrotic syndrome is generally managed by dietary
sodium restriction and loop diuretics. However, edema does not improve in some patients
despite adequate sodium restriction and maximal dose of diuretics. In such patients,
combination of albumin and a loop diuretic may improve edema by diuresis and natriuresis.
The response to this combination of albumin and a diuretic has not been observed in all
studies.
Additionally, my patient was referred to a nephrologist in lieu of her frequent relapses.
Medications prescribed:

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Oral prednisolone 75mg OD (60mg/m2/day)

Prophylactic antibiotic is given: Oral penicillin V 500mg BD

IV furosemide 20mg/2ml

Human albumin 20% injection 100ml

27/11/16-30/11/16
Day 4-7 of admission
Day 11-14 of illness

Patient was switched to ward 7A because she was relatively stable despite showing little
improvement. The albumin infusion was continued over a period of 4 days and subsequently,
the patients condition improved; her periorbital edema and facial puffiness decreased greatly,
ascites was still clinically evident by shifting dullness and fluid thrill but pedal edema only
was up to the level of the knee. Urine output has increased. During this 4 day period, oral
spironolactone was added to counteract the potassium-losing effect of furosemide (a loop
diuretic) as spironolactone is a potassium-sparing diuretic. Also, IV furosemide was switched
to oral(syrup) furosemide.
Medications prescribed:

Tablet prednisolone 75mg OD (60mg/m2/day)

Prophylactic antibiotic is given: Oral penicillin V 500mg BD

Syrup furosemide 1mg/ml (25mg TDS)

Human albumin 20% injection 100ml

Syrup spironolactone 1mg/ml(25mg TDS)

1/12/16
Day 8 of admission
Day 15 of illness

After the albumin infusion, patients condition continues to improve. Periorbital edema and
facial puffiness decreased greatly, ascites was still clinically evident by shifting dullness but
fluid thrill was negative by day 7 of admission and pedal edema only was up to the level of
the knee. Her vitals were normal and there were no signs of complications. Urine dipstick is
now 2+ and current weight is 45.5kg.
Medications prescribed:

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Tablet prednisolone 75mg OD (60mg/m2/day)

Prophylactic antibiotic is given: Oral penicillin V 500mg BD

Syrup furosemide 1mg/ml (25mg TDS)

Human albumin 20% injection 100ml

Syrup spironolactone 1mg/ml(25mg TDS)

2/12/16(Present Day)
Day 9 of admission
Day 16 of illness

My patients condition continues to improve. Periorbital edema and facial puffiness


decreased, ascites and pedal edema have decreased. Her vitals were normal and there were no
signs of complications. Urine dipstick is now 2+ and current weight is 44.9 kg.
Medications prescribed:

Tablet prednisolone 75mg OD (60mg/m2/day)

Prophylactic antibiotic is given: Oral penicillin V 500mg BD

Syrup furosemide 1mg/ml (25mg TDS)

Human albumin 20% injection 100ml

Syrup spironolactone 1mg/ml(25mg TDS)

At this point of time, my patient cannot be discharged yet. However upon discharge special
considerations to be given are:

To do home urine albumin monitoring: once daily dipstix testing of the first morning
urine specimen. The patient is advised to consult the doctor if albuminuria 2+ for 3
consecutive days, or 3 out of 7 days. In this, I would also like to try to provide the
mother to means of monetary support if at all possible.
The child is also advised to consult the doctor should he/she become oedematous
regardless of the urine dipstix result.
Children on systemic corticosteroids or other immunosuppressive agents should be
advised and cautioned about contact with chickenpox and measles, and if exposed
should be treated like any immunocompromised child who has come into contact with
these diseases.
Regarding immunization, while the child is on corticosteroid treatment and within 6
weeks after its cessation, only killed vaccines may safely be administered to the child

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and live vaccines can be given only 6 weeks after cessation of corticosteroid therapy.
However, it is important to advocate that both pneumococcal and varicella zoster
vaccine be administered to all children with nephrotic syndrome. If possible, give
when the child is in remission.
My patient should also be counseled about acute adrenal crisis

Evidence-Based Medicine
1)How effective are steroids in the treatment of frequently
relapsing nephrotic syndrome? Do alternative treatments
have a better success rate?
Up to 80% of children with idiopathic nephrotic syndrome respond to corticosteroids, with a
complete remission occurring within 30 days. Approximately one third of these patients are
cured after the course of corticosteroids. Another 10-20% of patients, experience relapses
several months after stopping the treatment and a cure takes place after three or four episodes,
which respond to a standard course of corticosteroids. However, up to 40 to 50% of patients
experience frequent relapses either as soon as steroid therapy is stopped (frequent relapsers)
or when the dosage of steroids is decreased (steroid dependent). (1)
There are many factors may cause the relapse of primary nephrotic syndrome, including too
short steroid treatment period, rapid tapering off the dose of prednisone, infections, etc. The
most important factor is too short prednisone treatment period. A study by Wang et al adopted
prolonged prednisone treatment plus tripterysium glucosides therapy(a traditional Chinese
medication); results showed that children during the last three months of prednisolone
therapy adding tripterysiun glucosides helped to strengthen the treatment and reduce the rerelapse.(2) However, studies by Takeda et al that cyclophosphamide-controlled patients have
more stable long-term remission compared with tripterysium glucosides group. (3) However
it is of note that tripterysium glucosides has less side effects compared to cyclophosphamide.
The first second line drug is usually cyclophosphamide and indeed it is shown that this drug
may be of particular use in children with frequent relapses as in a study by Shohet et al, a
group of patients received cyclophosphamide for 56 days in a single daily dose in order to
prolong the length of remission and it was shown that the percentage of patients who
continued in remission at the end of the 1st, 2nd and 5th years was greater in the frequentrelapser group. (5) Another treatment suggestion is pulse intravenous cyclophosphamide
therapy in frequently relapsing nephrotic syndrome, however there it could not be proven to
have a greater efficacy than the conventional oral steroid treatment.(4, 10) Some physicians
advocate the effectiveness of alkylating agents in the treatment of frequent relapsers, in a
certain patient who had over 100 relapses a short cycle of chlorambucil (0.2mg/kg/day for 8
weeks) was the drug that brought about a lasting complete remission.(11)

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Finally, a study by B. Cammas et al showed that long-term efficacy of cyclophosphamide in


steroid-responsive nephrotic syndrome is disappointing.(6) Yet another study supported the
superiority of steroids as a first line of treatment in patients with nephrotic syndrome be they
frequent relapses or not by reaffirming that that though levamisole may be considered an
alternative for cyclosporin as a first second-line agent for patients it could not replace
steroids.(7)

2) Does being a frequently relapsing patient mean that they


have a worse prognosis?
The longterm prognosis of idiopathic nephrotic syndrome is favourable. It has been
suggested that the short duration of proteinuria (due to the rapid effect of corticosteroids) and
its high selectivity could explain the general absence of chronic renal insufficiency in this
entity. In fact a case study on a patient with over one hundred relapses showed that despite his
many relapses he still retained normal renal function. (11)
In the long term, the adverse effects of the treatments of nephrotic syndrome remain the main
concerns.(1) For example, in this patient with ove 100 relapses, though he had a completely
nomal renal profile, he suffered from severe arterial hypertension which resulted in cerebral
haemorrhage.(11) This could very well have been a side of long-term corticosteroid
treatment. Effects of long term corticosteroid use is seen common particularly in severe
steroid-responsive nephrotic syndrome; commonly reported side effects are short stature(9)
and mental retardation(8).

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References
1. Niaudet P. Long-Term Outcome of Children with Steroid-Sensitive Idiopathic Nephrotic
Syndrome. Clinical Journal of the American Society of Nephrology. 2009;4(10):1547-1548.
2. Wang Y, Liu A, Dai Y, Yang C, Tang H. The treatment of relapsing primary nephrotic
syndrome in children. Journal of Zhejiang University SCIENCE. 2005;6B(7):682-685.
3. Takeda A, Takimoto H, Mizusawa Y, Simoda M. Prediction of subsequent relapse in
children with steroid-sensitive nephrotic syndrome. Pediatric Nephrology. 2001;16(11):888893.
4. Jones B. Cyclophosphamide Pulse Therapy in Frequently Relapsing Nephrotic Syndrome.
Nephron. 2008;63(4):472-472.
5. Shohet I, Meyerovitch J, Aladiem M, Boichis H. Cyclophosphamide in treatment of
minimal change nephrotic syndrome. European Journal of Pediatrics. 1988;147(3):239-241.
6. Cammas B, Harambat J, Bertholet-Thomas A, Bouissou F, Morin D, Guigonis V et al.
Long-term effects of cyclophosphamide therapy in steroid-dependent or frequently relapsing
idiopathic nephrotic syndrome. Nephrology Dialysis Transplantation. 2010;26(1):178-184.
7. Ekambaram S, Mahalingam V, Nageswaran P, Udani A, Geminiganesan S, Priyadarshini S.
Efficacy of levamisole in children with frequently relapsing and steroid-dependent nephrotic
syndrome. Indian Pediatrics. 2014;51(5):371-373.
8. Simmonds J, Grundy N, Trompeter R, Tullus K. Long-term steroid treatment and growth: a
study in steroid-dependent nephrotic syndrome. Archives of Disease in Childhood.
2010;95(2):146-149.
9. Emma F, Sesto A, Rizzoni G. Long-term linear growth of children with severe steroidresponsive nephrotic syndrome. Pediatric Nephrology. 2003;18(8):783-788.
10. Gulati S. Pulse cyclophosphamide therapy in frequently relapsing nephrotic syndrome.
Nephrology Dialysis Transplantation. 2001;16(10):2013-2017.

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11. Carreno A. The patient with over 100 relapses of minimal change nephrotic syndrome:
prolonged complete remission after chlorambucil treatment. Nephrology Dialysis
Transplantation. 2000;15(6):922-a-923.
12. Haji Muhammad Ismail, H., Ng, H. and Thomas, T. (2013). Paediatric Protocols For
Malaysian Hospitals. 3rd ed. Kuala Lumpur: Kementerian Kesihatan Malaysia.

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