FACULTY OF MEDICINE AND HEALTH SCIENCES

UNIVERSITY PUTRA MALAYSIA

2ND CASE WRITE-UP

NEPHROTIC SYNDROME

MUHAMMAD FARIEZUAN BIN ABDUL HAMID

172004
4th YEAR MEDICAL STUDENT
PEDIATRIC POSTING

SUPERVISOR:
PROF INTAN HAKIMAH
Patient Identification

Name : Muhammad Darwisy

R/N :
Age : 2 years 8 Month
Gender : Boy
Address : Mukah
Race : Malay
Informant : Mother
Date of admission :
Date of clerking :

Chief Complaint
Muhammad Darwisy, a 2-year-8-month old Malay boy, who was diagnosed with
nephrotic syndrome, was referred to Hospital Kuala Lumpur for renal biopsy.

History of Presenting Illness

The history was taken from her mother on day 1 of admission., the child was
apparently until 6 months prior to admission (December 2016) when he started to complaint
of scrotal swelling noticed by her mother after bathing him. The child doesnt complaint of
pain or discomfort and no skin changes noted by his mother. 2 weeks after that, the scrotal
swelling still not resolve and the child start to develop periorbital swelling which is more
prominent after woke up in the morning and slowly reduced in the evening. However, the
child still can open the eyes and no redness, itchiness, and skin changes noted by his mother
and no history of anaphylactic.
The scrotal and periorbital swelling progressively increase in size and her mother
brought her child to Hospital Mukah to seek for the treatment. He was then been diagnosed as
nephrotic syndrome as the result of initial investigation is suggestive of nephrotic syndrome,
in which I assume as hypobilirubinemia, nephrotic range proteinuria, and
hypercholesterolemia. In first hospitalization, his mother denied any oligouria, hematuria or
dysuria. He was started on corticosteroid therapy (oral prednisolone) and discharged after 5
days of stay and then was referred to Hospital Sibu for continuation of care. However, his
parents requested to continue follow up with the private pediatrician at the private hospital in
Sibu. He is compliant to medication as his mother is the one who handling his medication
schedule.
He was continued on high dose of prednisolone (20 mg OM and 15mg ON) from 10 th
December until 8th January 2017. He achieved remission on day 12 of induction of
prednisolone and then started to tapering down the dose since 9 th January 2017 (40mg/m2/day
EOD). However, on March 2017, patient started to develop relapsed, in which he started to
develop symptoms such as periorbital and lower limb swelling. Dr in charged then increase
the dose of prednisolone and gave albumin dose once as he develop poor urine output and
oedematous state.
On this current admission, he was first presented at Hospital Mukah on 1 st May 2017
with one week history of increasing facial puffiness, periorbital swelling, increase abdominat
distention, bilateral upper and lower limbs swelling and worsening scrotal oedema. As the
swelling become worsen, his weight also increased from 17.7kg to 20.8kg in 10 days
duration. He also had reduced urine output, in which his mother stated that there was reduced
in diaper changing duration. He also started to develop symptoms o upper respiratory
infection, which were cough and runny nose during the hospitalization. He was then referred
to Hospital Sibu for further management.
In Hospital Sibu, Dr-in-charged continued his induction dose prednisolone 20mg BD
and also start with prophylaxis penicillin. As he had reduced urine output, he was then given
2 doses of IV human albumin 10% 200ml with IV frusemide 10mg midway. Later, he was
then referred to Paediatric Nephrologist HKL for renal biopsy and further management.

Systemic review.
Neurological system : No episode of fit or loss of consciousness, headache, no altered
mental status or change in behaviour. No different in gait or
sudden limbs weakness noted.
Hematological system : No history of increase bleeding tendency, no gum or nose
bleed, not easily bruising. No dark colour stool.
Gastrointestinal system : No diarrhea, constipation and vomiting.
Respiratory system : No pleuritic pain, wheeze and cough.
Rheumatological system : No joint swelling.
Dermatological system : No rash

Past Medical And Surgical History

He had no known medical illness namely heart disease, liver disease, vasculitis,
connective tissue disease, malignancy or immunological disease. He never undergo any
surgery before.

Medications and Allergies

He had no known food or drug allergy.

Birth History
Antenatally
Uneventful

Intrapartumly
She was delivered at 39 weeks via vertex delivery with birth weight 3.06 kg at Hospital
Mukah. No fetal and maternal complications. He was crying spontaneously after delivered.

Postpartumly
He had jaundice for one weeks and was treat with phototherapy. No history of ICU
admission. No feeding, bowel and urinary problems.

Nutritional History
He was exclusively breastfed for 6 months and was start weaning after that with introduction
of porridge. Currently, he had adult diet with 3 meals daily which comprised of rice, fish or
chicken and vegetables.

Immunization History

His immunization was completed up to age. No additional immunizations were given. Last
dose given was at 21 months old.
Immunization Age (months)
0 1 2 3 4 5 6 9 12 18 21
BCG
Hepatitis B
DPaT
Polio
Hib
Measles
MMR
JE

Developmental History

For the gross motor, patient could turn over at 6 months. He can sit unsupported at 8 months.
He crawls, stand and walk at 1year old. He runs at 2 years old. Currently, he is able to jump
and kick the ball.

For the fine motor and vision, he reaching and transferring object at 6 months. He stopped
mouthing at 9 months. He scribbled at 2 years old. Currently, he is able to draw a circle.

For hearing and speech, he was babbling at 7 months, speaking with 2 single words at 18
months. Currently, he can form 3 words phase.

For social and understanding, he responds and turns to his name at one year old.

Therefore, the developmental milestone was normal and corresponded to age.

Family History

There was no consanguinity between them. All family members are healthy and there was no
renal disease, diabetes, developmental delay, connective tissue disease or malignancy running
in her family.
Social History
Lives in single-storey house in Mukah. Both of his parents are teachers. During office hour,
he and his brother will be taken care by his uncle, who is his fathers younger brother. No pet
at home. His father is a smoker. Smoke 2 cigarettes per day.

Summary
Muhammad Darwisy, a 2-year-8-month old Malay boy, who was diagnosed with
nephrotic syndrome, currently with nephrotic syndrome symptoms, which is increasing facial
puffiness, periorbital swelling, increase abdominat distention, bilateral upper and lower limbs
swelling and worsening scrotal oedema for one week duration, was referred to Hospital Kuala
Lumpur for renal biopsy.

PHYSICAL EXAMINATION

Vital signs
Pulse rate : 108 beats per minute (normal)
Blood pressure : 104/ 69 mmHg (normal)
Respiratory rate : 20 breaths per minute (not tachpneic)

Temperature : 37 C ( afebrile)

Growth parameters

Weight : 19.2 kg (>95th centile)

Height: 86 cm (>10th centile)

General examination
Patient was playing comfortable on the bed. He looked well and not in pain. He was
pink and the hydrational status was good. There was periorbital swelling and facial puffiness
noted. No dysmorphic features seen.

Peripheral Examination
On examination of the hand, it was warm. There was no sign of clubbing, cyanosis or
pallor noted. There was no stigmata of infective endocarditis namely splinter haemorrhages,
Oslers nodes and Janeway lesion. There was also no sign or chronic liver disease such as
leuconychia, palmar erythema and Dupuytrens contracture. Scratch marks were not seen
over her arm. Capillary refilling time was less than 2 seconds. Pitting oedema up to knee
level ans also sacral oedema.

Examination of Head and Neck

Inspection of the face revealed no pallor, jaundice or central cyanosis. Oral hygiene
and hydration was good. The throat was not injected, the tonsil was not enlarged and not
hyperemic Otherwise, there was no palpable cervical lymph nodes.
Abdominal system
On inspection, patients abdomen was distended and moving with respiration. The
umbilicus was centrally located and inverted. There was no obvious mass, skin changes,
pigmentation and surgical scar on his abdomen. No visible peristalsis and dilated vein was
noted. Hernia orficies were intact. On palpation, the abdomen was soft and non-tender. The
liver and spleen was not palpable, the kidneys were also not ballotable. On percussion,
shifting dullness was positive and fluid thrills also positive. On auscultation, bowel sound
was present and normal. No renal bruit.

Respiratory system
Inspection of the chest revealed no scars, spider naevi, gynecomastia and no
deformity such as pectus carinatum, excavatum, or Harisson sulcus. There was no sign of
hyperinflation and recession noted on both sides. Both sides of the chest moved
symmetrically with respiration. On palpation, trachea was centrally located. Chest expansion
and vocal fremitus were normal. On percussion, it was resonant on both sides. On
auscultation, the intensity of the breath sound was normal bilaterally, there was no added
sound such as rhonchi and crepitation heard bilaterally.

Cardiovascular system
On inspection on the precordium, there was no gross deformity on the chest, dilated
veins, visible pulsation, and surgical scar. Her apex beat was palpable at the point of 4th
intercostals space and midclavicular line. There was no palpable thrill and parasternal heave.
On auscultation, the apex beat was heard. First and second heart sound were heard over all
the four area the mitral, tricuspic, aortic and pulmonary valves. There was no added heart
sound and murmur was not detected.

Nervous system examination.

On inspection of the face, there was no dysmorphic features seen. Overall size
and relative proportions of head, trunk and limbs were appropriate for his age. There was no
abnormal posture such as torticolis, scoliosis and kyphosis seen. There was no feature
suggestive of neurocutaneous syndrome such as caf au lait macules (flat,light brown areas of
skin) or adenoma sebaceum..

Central nervous system

Cranial Nerves Examination
CN2 : The visual acuity and visual field were normal, bilateral
pupillary reflex were present ( direct and consensual). The
accommodation also was elicited. However, I did not
perform the fundoscopic examination.
CN3,4,6 : There was no opthalmoplegia and diplopia.
CN5 : Motor: the messeter and temporalis muscle tone was good,
he was able to open mouth against resistance which showed
that both pterygoids muscle were intact.jaw jerk was present.
Sensory: Sensation over three division of trigeminal nerve
namely ophthalmic, maxillary and mandibular were intact.
The corneal reflex(not done).
CN7 : There was no facial asymmetry or loss of nasolabial fold.
He was able to close her eyes tightly (orbicularis oculi), he
had the wrinkled over the forehead upon looking up and he
was able to close her mouth tightly (orbicularis oris)
CN8 : Rinnes and Webers test was not performed in this patient.
CN11 : Sternocleidomastoid and trapezius muscles were intact as
there is no head tilt and drooping of the shoulder
CN9,CN10 : Uvula and palate were centrally located, gag reflex (not
CN12 done)
: Tongue was centrally located, not protruding there was no
tongue fasciculation or wasting.
Peripheral nervous system
On the examination of both upper and lower limb. He had normal gait, there was no
fasciculation, no involuntary movement, no scars, no ulcers and no neurocutaneous stigmata
noted on inspection. Muscle bulk appeared to be normal. Coordination and sensation was
intact.

Power: For the both upper and lower limb, both were 5/5 bilaterally.
Tone: Bilateral upper and lower limbs tone were normal.
Reflexes : All reflexes ( deep tendon reflex) namely biceps, triceps and supinator
reflex were normal for bilaterally. The plantar reflex ware down going for both right
and left plantar.

Provisional diagnosis
Steroid Resistance Nephrotic syndrome
Points for:
1. Age group 1-10 years old, this patient is 4 years old
2. Periorbital, facial and bilateral forearm and leg swelling
3. Reduced urine output
4. Facial puffiness
5. Ascites

Point against:
1. No shortness of breath indicating absence of pleural effusion
2. No frothy urine
3. Physical findings showed no sign of hypovolaemia.

Differential Diagnosis

Post-streptococcal acute glomerulonephritis (AGN)

Points for:
1. Oedema
2. History of upper respiratory tract infection ( might be post streptococcal
infection)

Points against:
1. No hematuria
2. No decrease urine output
3. No history of upper respiratory tract infection ( might be post streptococcal
infection)
4. No enlarged tonsils

Liver disease
Points for:
1. Oedema
 2. Ascites

Points against:
1. No jaundice
2. No hematemesis
3. No hepatomegaly

Heart failure
Points for:
1. Oedema
2. Reduced affect tolerance

Points against
1. No history of previous heart diseases
2. No palpitation
3. No orthopnea
4. No paroxysmal nocturnal dyspnea
5. No shortness of breath
6. No hepatomegaly
7. No cardiovascular findng suggestive of heart failure ( tachypenic ,
tachycardia, cardiomegaly, hepatomegaly)

Investigations
1. Full Blood Count

Result Normal range

WBC 12.8 x109/l (5.0 - 13.0)
Hb 12.2 g/dl (11.5 15.5)
Plt 393x109/l (150 - 400)
PCV 0.35 L/L (0.40 - 0.54)

All parameter are normal.

2. Urinalysis

Test Result Reference

Color yellow
Blood 3+
Bilirubin Neg
Urobilinogen Normal
Ketone Neg
Protein/ albumin 4+
Nitrite Neg
Urine glucose Neg 0.00-0.00
pH 6.0 5.00-8.00
Specific gravity 1.020 1.00-1.03
Leucocytes Neg
Crystal (microscopic) Nil
Cast (microscopic) Nil
Leucocytes (microscopic) Nil 0.00-6.00
Erythrocytes (microscopic) 63 0.00-3.00

Result support diagnosis of nephrotic syndrome with present of protein in the urine > 1+
which indicate proteinuria.

4. Renal profile and liver function test

Test Result Reference
Urea 3.7 mmol/l (0.00-8.00)
Sodium 138 mmol/l (132.00-145.00)
Potassium 3.31mmol/l (3.10-5.10)
Chloride 127mmol/l (96.00-111.00)
Creatinine 31 mmol/l (0.00-88.00)
Total protein 44g/L (66.00-87.00)
Albumin 12 g/L (30.00-54.00)
Alkaline phosphatase 111 g/L (0.00-320.00)
Alanine transaminase 9 g/L (0.00-39.00
Total bilirubin 0 g/L (0.00-17.00)

Hypoalbuminaemia due to urinary losses of protein. Otherwise the patient was not
dehydrated and the other parameters indicate normal renal and liver function.

5. Lipid profile

Test Result Reference

Cholesterol 16.2mmol/l (0-5.2)
HDL cholesterol 1.1 mmol/l (0.9-2.0)
LDL cholesterol 13.6mmol/l (0-4.0)
Triglycerides 3.4 mmol/l (0-2.3)

The patient had hypercholesterolemia which was correlates inversely with serum albumin
suggestive of nephrotic syndrome.

6. Protein creatinine index

Urine protein Conc : 14.81 g/l
Urine creatinine Conc : 9.91 mmol/L
Protein/creatinine index : 1.50 g/mmol creat (< 0.03)
High protein creatinine index suggest nephrotic syndrome.

7. Serum complement (pending)

( SLE -low both C3 and C4 , post infectious glomerulonephritis- low C3, normal C4)

8. ASOT titre
ASOT<200mg/dL

Other relevant investigations:

24 hour urine protein
Antinuclear factor/ anti- dsDNA ( to exclude SLE)

Final diagnosis

Nephrotic syndrome

Management

After all the investigation being ordered, oral Penicillin V 125mg was commenced. In the
ward, the patient was put on nephrotic chart, daily urine dipstick, low salt diet and he was
encouraged orally. Besides, patient was put on I/O chart, monitor vital sign and blood
pressure (4 hourly). Daily weight monitoring was also done in this patient and fluid
restriction 500 ml/24 hourly. They also need to watch out for hypovalaemic sign (cold
peripheries, prolonged capillary refilling time, poor pulse volume normal or low blood
pressure)and and hypervolaemic sign (basal lung crepitation,rhonchi, hepatomegaly,
hypertension) .

Discussion

Nephrotic proteinuria in children defined as protein greater than 40 mg//m 2 / hour or U Pr/Cr
greater than 2.0. Proteinuria between these two levels is mildly or moderately elevated but is
not nephrotic whereas nephrotic syndrome is a clinical syndrome that characterized by
persistent heavy proteinuria which mainly albuminuria > 1g/m2/day, hypoproteinemia,
( albumin < 3.0 g/dL), hypercholesterolemia(> 250mg/dL) and oedema.
 The increased glomerular permeability to serum protein is due to alteration in glomerular
basement membrane protein and their normal negative charge that restricts filtration of serum
protein that lead to massive protenuria. This further leads to decline in serum protein
particularly albumin. Oedema resulted because of the diminished of the plasma oncotic
pressure that make the fluid from vascular compartment shifted to the interstitial
compartment. Elevated serum lipid ( cholesterol and triglycerides) is caused by the
hypoproteinemia that stimulates hepatic lipoprotein synthesis and diminished lipoprotein
metabolism.

As for this patient he had the clinical symptom and sign that suggestive of nephrotic
syndrome namely periorbital, facial and leg swelling with abdominal distension. Urinalysis
done on different day showed protenuria > 1+ which suggests persistent proteinuria that
require more investigation. So, albumin and cholesterol level was checked and both
demonstrate hypoalbuminemia with only 12g/dl and also hypercholesterolemia which further
support diagnosis of nephrotic syndrome. Penicillin was commenced to this patient however
the induction with oral prednisolone was still keep in view until the diagnosis is comfirmed.

Nephrotic syndrome can be idiopathic or secondary to systemic illness such as Henoch-

Schonlein purpura(HSP) and other vasculitides for example systemic lupus erythematous
(SLE), infection (malaria) or even allergen (bee sting or drug- penicillin, aspirin). For this
patient, all the systemic disease have been rule out. Eventhough the result of complement
levels was still pending but the absent of hematuria grossly and microscopically can help us
to rule out acute nephritic that usually cause by post- streptococcal infection.

For this patient, it is most likely to be primary/ idiopathic nephritic syndrome which was
the commonest type of nephrotic syndrome in children. So, diagnosis of idiopathic nephrotic
syndrome was established, about 85-90% of the children with nephrotic syndrome are
steroid-sensitive nephritic syndrome which usually these children does not proceed to renal
failure. It is commoner in boys, asian and usually precipitated by respiratory infection just
like my patient. Besides, some features would be age between 1-10 years, no macroscopic
hematuria, normal blood pressure, normal complement level and normal renal function.
Usually 80% percent of the children will achieve remission which defined as urine dipstix
trace or nil for 3 consecutive days). However, there are some group of children that are
steroid- resistant nephrotic syndrome which defined by failure to achieve remission after 4
weeks of induction of corticosteroid. These type of patients require further evaluation by
peadiatric nephrologist and renal biopsy is usually indicated.

Parents should be counseled on the possibility of relapse and usually 1/3 of the patient
will have the nephrotic syndrome resolve directly, another 1/3 will have infrequent relapse
and the other 1/3 may have frequent relapses and become steroids dependent. Daily home
urine monitoring must be done and parents should be told that they should consult a doctor if
albuminuria is 2 + or more for 2 consecutive days or 3 out of 7 days because it indicate
relapse. However, this children usually does not need admission unless there are gross
oedema and have any complication of nephritic syndrome namely hypovolemia that will
presented as abdominal pain and feeling faint, the low urinary sodium and high packed cell
volume of red blood cell are indication of hypovolaemia. This is important as the patient may
require intravenous albumin as there are risk to develop vascular thrombosis and shock in this
patient. The other complication is thrombosis which may affect the brain, lim and splancnic
circulation that cause by urinary losses of antithrombin and thrombocytosis that are
exacerbated by steroid therapy, increase synthesis of clotting factor and increase blood
viscosity because of raise haematocrit. Besides, the group of children that have relapse may
have high chance to develop infection which is spontaneous bacterial peritonitis and usually
vaccination with pneumococcal vaccine should be administer to all the patient with nephritic
syndrome. The parents must also advised on cautioned about contact with chicken pox and
also measles while the patient was on corticosteroid which is an immunosuppressant.

References
Nelson Essential of pediatrics, 6th edition, Saunders Elseviers
Illustrated Textbook of Paediatrics 4th edition, edited by Tom Lissauer & Graham
Clayden, Mosby Publication.
Paediatric protocols, 3rd edition.