CASE

PRESENTATION
 Team Members
ANUSHKA NATASHAH DEWI SARAVANAN 012020100158

KISHANTINE A/P SATIASEELAN @SURESH 012020100032

KUMAR

SERENA SHANTINI A/P RICHARD IRUTHIAM 012020100011

MUHAMMAD MUZAKKIR BIN MANSOR 012019040009

JESSALYNN A/P IRATHIARAJ @ PETER 012020100084

SOMESVAREN A/L SIVANATHAN 012020040061

 PATIENT PROFILE
NAME: VAAGINI A/P SHANMUGAMPRIYAN

REGISTRATION NUMBER: SA 0582251

AGE: 6 YEARS 9 MONTHS

DATE OF BIRTH: 5/6/2016

GENDER: FEMALE

RACE & RELIGION: INDIAN,HINDU.

DATE OF ADMISSION: 11/3/2023

WARD 12,HOSPITAL SHAH ALAM
 CHIEF COMPLAINT

1. Swelling of face,abdomen and legs-7 days

Sunday (4/3/2023)
2. Cough and runny nose-6 days
Monday(5/3/2023)
HISTORY OF PRESENTING ILLNESS
Vaagini A/P Shanmuganpriyam is a 6 year 9 month Indian girl with underlying
nephrotic syndrome history was presented to the hospital with chief complain
swelling of leg for 7 days and cough along with runny nose for 6 days prior to
admission.

Patient was previously well until the mother noticed swelling of the area
around the eyes on day 1 of illness (Sunday 4/3/2023). According to the
mother it was not painful to touch or had any discoloration. The patient had
suffered from the same condition before a year ago.

A urine dipstick was done by mother which revealed 2+. There were no other
family member with similar symptoms.
On day 2 of illness the patient developed cough and runny nose. The
cough occurred approximately 5 times a day. In the morning it started off
dry and around 4pm it become productive. The sputum was clear and non
foul smelling. It was not associated with abnormal sound. It was not
associated with shortness of breath. The patient face did not turn blue or
red during these episodes. It was not followed by vomiting
(non-posttussive). The cough was associated with runny nose. The nasal
discharge was clear, minimal in amount and watery consistency. Over the
counter cough medicine was given. The cough was relieved
approximately for the day and reappeared the next day. The mother
rubbed vapour rub in attempt to relief. The urine dipstick done in day 2
revealed 2+.
On day 3, 4 and 5 of illness, the condition of the patient remained the
same with urine dipstick protein reading of 2+. The same over the
counter cough medicine was given for temporary relief.

On day 6 of illness mother noticed the swelling has spread to the

patient leg and abdomen around 10pm which prompted her to bring
the patient to the emergency department of Hospital Shah Alam. At the
emergency department a urine dipstick protein showed 3+. The patient
was then admitted to the Paediatric ward level 12 on day 7 of illness
approximately 12.40am.
 SYSTEMIC REVIEW
GENERAL HEALTH CARDIOVASCULAR
● Sleep disturbed ● Ankle swelling present
● Loss of appetite ● No palpitations
● Increased weight ● No orthopnea
● Loss of energy ● No nocturnal breathlessness
● No chest pain
● No dyspnea
● No cyanosis

RESPIRATORY GASTROINTESTINAL
● No shortness of breath ● Abdominal pain present
● No wheezing ● Diarrhoea present
● No sputum production ● No dysphagia
● No chest pain related to ● No nausea and vomiting
respiration ● No heartburn
 SYSTEMIC REVIEW (CONT. )
URINARY ENDOCRINE
● Frothy urine present ● Swelling of eyes present
● Dark urine output ● No excessive thirst
● Reduced urine output ● No heat or cold intolerance
● No dysuria ● No change in sweating
● No hematuria ● No prominence of eyes

NERVOUS SYSTEM MUSCULOSKELETAL

● No headache ● Joint pain present
● No fits ● Muscle pain and weakness
● No faints or blackouts present
● No abnormal movements
● No visual disturbance
● No hearing loss
 HISTORY OF PAST ILLNESS
OCT 2022

On Oct 2022 patient presented with swelling of the face and legs for 4 days.
Upon visit to Shah Alam Hospital, a full blood count, renal profile and
urinalysis was done. Patient was positive for proteinuria and hypoalbuminemia.
The patient was diagnosed with nephrotic syndrome and admitted for 2
weeks. She was managed by having a proper protein diet with adequate
calories and given Penicillin V 250mg BD. She was also on a watch out for
hypovolemia and hypervolemia signs. She was then treated with Prednisone
60mg for 4 weeks followed by prednisone 40mg alternatively for 4 months.
The patient was compliant to the medication.
 BIRTH/LIFE HISTORY
ANTE-NATAL

Vaagini's mother did antenatal medical check up regularly at KK Taman

Medan. Tetanus injection was taken during pregnancy. Serological test for Hep
B, C, VDRL and HIV was done and she tested negative for all. The mother did
not have any fever with rashes during the 1st trimester. The mother had
anemia during pregnancy and was controlled with iron and folic acid. The
mother did not need transfusion for the anemia. Other than that, the mother
did not have any epilepsy, gestational diabetes mellitus or pregnancy induced
hypertension. The mother did not have any trauma during pregnancy or any
radiation exposure.
PERI-NATAL

Vaagini was born premature at 28 weeks via spontaneous vaginal delivery.

The mother was in labour for approximately 12 to 14 hours. After the labour,
the mother's hemoglobin level was low and blood transfusion was done as her
legs got swollen. After 2 weeks, the complication of the mother resolved.
Vaagini was kept in the NICU till term age reached.
POST-NATAL

Vaagini cried immediately after birth. Her birth weight was 2.74 kg. She also
passed meconium on the same day. She was kept in the NICU due to
prematurity and inflammation of the heart. She was screened for G6PD and
thyroid function test which later came out normal. Once term age was
reached, Vaagini was discharged and she was followed up monthly for check
up due to the inflammation of the heart. Thus, the problem resolved after
Vaagini reached 2 years old.
 FEEDING HISTORY
BREASTFEEDING AND COMPLIMENTARY FEEDING

Vaagini was breastfed for 1 ½ month and was then introduced to formula
milk.Vaagini was fully on formula milk at the age of 2 months till 3 years old.
The milk given was Dumex at a ratio of 2 : 2. The milk was given on demand
and Vaagini finished it within 2 to 3 minutes. Weaning started at 6 months old
and porridge was firstly introduced to her. There was no pre-lacteal feeding.
CURRENT DIET

Vaagini loves to eat rice and she normally eats 3 times per day in small
portions. Proteins such as egg, fish as well as vegetables are also taken. Her
current weight is 30 kg, height 130 cm and her BMI is 17.8 kg/m² which is at
risk of being overweight.
 IMMUNIZATION HISTORY

Vaagini's vaccination was completed up to age and the last vaccinations taken were
DTap, IPV and Hib. No covid vaccination was given. Vaagini didn't have any
complications after that.
 DEVELOPMENTAL HISTORY

Vaagini can dance and run just like other normal children her age. She can also
color and draw properly. She loves to sing songs. Also, she can cooperate with her
friends and follow the rules when playing games. In short, her developmental
milestones are up to her age.
 FAMILY HISTORY

Vaagini's mother, Mrs Gunaladi, 27 years old, married to Vaagini's father, Mr

Shanmugampriyan, 29 years old. Marriage is non-consanginous and there are
no history of miscarriage. They have 4 children and all of them are girls at the
age of 7, 6, 5 and 4. There is no similar medical condition like Vaagini in the
siblings as well as their parents' generation. However, both of the parents'
mother and father, have diabetes mellitus.
 ALLERGY HISTORY

Vaagini doesn't have any known drugs and foods allergies. Allergic to pollen or
cat's fur is also not known.
 SOCIO-ECONOMIC HISTORY
Both of her parents' educational level was SPM. Their income is roughly RM1500 to
RM3000. Vaagini's mother is a full-time housewife while her father works as a lorry
driver. Vaagini's father is a smoker and an occasional drinker. They live in a flat house
at Taman Desa Shah Alam. They have 2 rooms only and Vaagini shares a room with all
her siblings. The housing area was a dengue prone area evidenced by frequent
fogging, present flooding problems and sometimes water cut issues. Vaagini goes to
school at SJKT Vivekananda. She is active and performes well at school. Vaagini also
gets along well with her classmates. As this is the first relapse of nephrotic syndrome,
the parents can still manage her well, and always give full support to her.
 PHYSICAL EXAMINATION
GENERAL APPEARANCE VITAL SIGNS
● Position : The patient was lying down in ● Pulse rate : 116 beats per minute
supine position supported by 1 pillow. ● Respiratory rate : 22 breaths per
● Surroundings : IV cannula at dorsum part of minute
left hand ● Blood pressure : 110/64 mmHg
● Behaviour : Alert and active ● Temperature : 37.7 C
● Respiratory distress : No, evidenced by no ● SPO2 : 99% under Room Temp.
nasal flaring and no rapid breathing
● Nutritional and hydrational status:
Satisfactory, evidenced by no sunken eyes EYES
and moist lips ● Presence of periorbital oedema
● No conjuncttival pallor
HEAD ● No xanthelasma
● No jaundice
● Size and shape of her head is normal
● No ptosis
● No corneal arcus
PHYSICAL EXAMINATION (cont)…
EARS MOUTH
● Position of ears are normal ● Oral hygiene was good
● No ear discharge ● Lips were moist
● Tounge was not coated
● No central cyanosis
NOSE ● No ulcers
● Nasal septum appears normal ● No enlarged tonsils
● No nasal discahrge ● No dental caries

NECK BACK
● No neck swelling ● Sacral oedema present
● No lymphadenopathy ● No spine deformity
● No raise in Jugular Venous
Pressure (JVP)
● Carotid pulse was palpable
 PHYSICAL EXAMINATION (cont)…
HANDS SKIN
● Dry and warm to touch ● No presence of rash, bruises or
● Capillary refill time (CRT) <2 seconds hyperpigmentation
● No splinter hemorrhage ● No surgical scars
● No clubbing
● No palmar erythema
● No peripheral cyanosis
● No fine and flapping tremor
● No olser nodes or janeway lesions
LEG
● Presence of bilateral pedal
oedema till the upper thigh
GENITALIA ● No leg ulcers
● No swelling of labia majora ● No peripheral cyanosis
 ABDOMINAL EXAMINATION
PALPATION
- Abdomen was hard and non-tender
- No masses felt during superficial and
deep palpation AUSCULTATION
- Kidney is not ballotable - Normal bowel sound
- Liver and spleen were not palpable - No renal bruits

1 2 3 4
INSPECTION PERCUSSION
- Abdomen was distended - Tympanic over all 9 quadrants
- Umbilicus was slit-like - Positive shifting dullness
- All 9 quadrants of abdomen moved - Positive fluid thrill
with respiration
- There was no bruises, dilated veins,
rashes, lumps or surgical scars seen
 RESPIRATORY EXAMINATION
PALPATION
- Chest expansion was equal on both AUSCULTATION
side -Air entry was equal on both
- Trachea was centrally located, no lungs
deviation - Vesicular breath sound
- No chest wall tenderness - No added sound

1 2 3 4
INSPECTION PERCUSSION
- Chest move bilaterally and symmetrically - All lung fields was
with respiration resonance
- No chest deformities like pectus
carinatum and excavatum
- No usage of accessory muscles
- No chest recession
- No visible pulse or dilated veins
 CARDIOVASCULAR EXAMINATION

PALPATION
- Apex beat was palpable on the 5th
intercostal space medial to left
midclavicular line

1 2 3
INSPECTION AUSCULTATION
- No pericardium deformity - Normal heart sound S1 and S2
- No surgical scars or dilated veins were heard
- No visible pulsation over the pericardium - No other additional sounds or
- No pericordial bulging murmur heard
 ANTHROPOMETRY MEASUREMENT
WEIGHT WEIGHT-FOR-AGE
30 KG 95th percentile

HEIGHT HEIGHT-FOR-AGE
130 CM Above 95th percentile

HEAD
BMI-FOR-AGE
CIRCUMFERENCE
Between 85th and 90th
48CM
percentile (17.8kg/m²)
HEIGHT-
FOR- BMI-FOR-AGE
AGE

WEIGHT-
FOR-
AGE
 CASE SUMMARY
Vaagini A/P Shanmuganpriyam, a 6 year 9 month Indian girl with past
medical history of nephrotic syndrome presented to the Emergency
Department with swelling of face, abdomen and leg for 7 days, cough for 6
days and runny nose for 6 days prior to admission. Over the counter cough
syrup was given at home for temporary relief. Patient had myalgia and
arthralgia. Upon physical examination, patient had periorbital oedema,
distended abdomen with slit-like umbilicus and bilateral pedal edema. The
edema was pitting in nature. The patient was also positive for shifting
dullness and fluid thrill.
 PROVISIONAL DIAGNOSIS

First Relapse of Nephrotic Syndrome

● Past medical history of nephrotic syndrome
● Presence of periorbital oedema
● Presence of bilateral pitting oedema of the lower extremities
● Presence of abdominal distension
● Positive for proteinuria (dipstick test 3+)
 DIFFERENTIAL DIAGNOSIS
Points supporting Points against

● Presence of oedema in
the legs
● No increased blood
● Presence of periorbital
Post-infectious pressure (hypertension)
oedema
Glomerulonephritis ● No oliguria/anuria
● Positive for proteinuria
● No hematuria
(dipstick test)

● Positive for proteinuria ● No characteristic skin

Henoch Schonlein
(dipstick test) rash
Purpura
● Presence of arthralgia ● No abdominal pain
DIFFERENTIAL DIAGNOSIS (CONT. )
Points supporting Points against

● Presence of oedema in
● No anemia
abdomen
● No ridged or cracked nails
Kwashiorkor ● Presence of tiredness
● No dry or brittle hair that
● Presence of loss of
falls off easily
appetite

● Presence of periorbital
● Not associated with
oedema
shortness of breath
Angioedema ● Presence of abdominal
● No itchy rash
pain
● No lip oedema
 INVESTIGATIONS DONE
● FULL BLOOD COUNT
Parameters Value in patient Normal Range

White Blood Cells (WBC) 9.0 x 10^3/uL 5.0 - 15.0 x 10^9/L

Red Blood Cell (RBC) 5.1 x 10^6/uL 4.0 - 5.9 x 10^12/L

Haemoglobin (Hb) 11.9 g/dL 11.2 - 14.5 g/dL

● BLOOD UREA AND SERUM ELECTROLYTES

Parameters Value in patient Normal Range

Urea 3.0 mmol/L 1.8 - 6.4 mmol/L

Sodium 140 mmol/L 136 - 146 mmol/L

Potassium 3.4 mmol/L 3.4 - 4.5 mmol/L

Chloride 105 mmol/L 101-109 mmol/L

INVESTIGATIONS DONE (CONT. )
● CREATININE

Parameter Value in patient Normal Value

Creatinine 36 μmol/L 27 - 62 μmol/L

● C-REACTIVE PROTEIN
Parameter Value in patient Normal Value

CRP <0.50 mg/L <5 mg/L

INVESTIGATIONS (PROPOSED)
Tests Reasons

Kidney ultrasound to check for kidney

Imaging enlargement and Chest X-Ray to check for
pleural effusion

To test for hyperlipidemia (level of

Lipid profile cholesterol is inversely correlated to the
serum albumin concentration)

To exclude SLE
Antinuclear factor/ anti-dsDNA

To exclude SLE and post-infectious

Serum complement (C3,C4) levels
glomerulonephritis

To exclude post-streptococcal
ASOT titres
glomerulonephritis
 MANAGEMENT & TREATMENT
General management
● A normal protein diet with adequate calories is recommended.
● No added salt to the diet when child has oedema.
● Penicillin V 250 mg is recommended at diagnosis and during relapses, particularly in the
presence of gross oedema.
● Careful assessment of haemodynamic status. (Look for signs of hypovolemia or
hypervolemia)

Initial treatment
● Initial Prednisolone therapy of 60mg/m^2 per day for 4 weeks followed by alternate day
Prednisolone of 40mg/m^2 per day for 4 weeks, then taper over 4 weeks and stop.

Treatment of relapses
● The majority of children with nephrotic syndrome will relapse.
● Treatment of initial relapse include Prednisolone at dose od 60mg/m^2 per day (maximum
dose of 60mg/day) until remission, then followed by 40mg/m^2/EOD for 4 weeks then stop.
NEPHROTIC SYNDROME
 NEPHROTIC SYNDROME
● Nephrotic syndrome is a clinical manifestation of renal disease due
to glomerular injury
 ETIOLOGY
PRIMARY/IDIOPATHIC SECONDARY

•SLE
•Minimal change disease (> 80 %)
•HSP
•Focal segmental glomerulosclerosis (10-20
%) •Chronic infections (HBV, HCV, HIV, Malaria)

•Membranoproliferative glomerulonephritis •Diabetes

(5-15 %) •Amyloidosis
•Membranous nephropathy (< 5 %) •Malignancies
•Ig A nephropathy/others •CHF, constrictive pericarditis
•Renal vein thrombosis
 CLINICAL FEATURES

● Periorbital edema (particularly on waking)

● Sudden onset of dependent pitting edema
● Scrotal or vulval, leg and ankle edema
● Ascites, Abdominal distension
● Oliguria
● Breathlessness due to pleural effusions
PATHOPHYSIOLOGY
 MINIMAL CHANGE DISEASE
PATHOLOGY RISK FACTOR
● Type of glomerulonephritis: ● Recent infection:
podocyte in glomeruli damaged by immunization,immune
T cells cytokines stimulus,medication(NSAIDS)
● Foot processes of podocytes ● Hematologic malignancies(e.g
damaged, flattened(AKA Hodgkin’s lymphoma)
effacement) lose function as ● Most common nephrotic
barrier albumin syndrome in children
permeates,bigger proteins cannot
get through(selective proteinuria)
COMPLICATION
CAUSES
● Relatively benign, does not affect
● Unknown; T cells release
kidney function
cytokines, may cause effacement
of podocytes
 MINIMAL CHANGE DISEASE (CONT. )
DIAGNOSIS

LAB RESULTS
● Protein in urine > 3.5g/day

KIDNEY BIOPSY
● Corticosteroid resistant patients
● Light microscopy - glomeruli appears normal
● Electron microscopy - effacement of foot processes
● Immunofluorescence - negative (no immune complex deposition)

SIGNS AND SYMPTOMS TREATMENT

● Proteinuria, hypoalbuminemia, edema, ● PREDNISONE THERAPY
hyperlipidemia, lipiduria,hypercoagulability Excellent response more quickly in
● Onset more rapid (days to weeks) than children than adults
other nephrotic syndromes
FOCAL SEGMENTAL GLOMERULOSCLEROSIS
PATHOLOGY RISK FACTOR
● Histologic finding of glomerular damage, ● More common in black people
not distinct disease ● Morbid obesity
● Affects parts (segmental) of some (focal) ● Chronic kidney disease
glomeruli of nephron; damage, scarring-
proteinuria
● Foot processes of podocyte damaged SIGNS AND SYMPTOMS
plasma proteins, lipids permeate ● Proteinuria,hypoalbuminemia,ede
glomerular filter ma
● Proteins, lipids trapped-build up inside
glomeruli-hyalinosis-scar tissue COMPLICATION
(glomerulosclerosis) ● End stage renal failure

CAUSE
● Primary : unknown
● Secondary: results of underlying cause
● Genetic forms: FSGS 1-6
FOCAL SEGMENTAL GLOMERULOSCLEROSIS
DIAGNOSIS TREATMENT
● Blood pressure reduction-”ACE
LAB RESULTS inhibitor”
● Protein in urine> 3.5g/L ● Edema - diuretics
● Prednisone
KIDNEY BIOPSY: MOST DEFINITIVE
● Light microscopy: segmental
sclerosis,hyalinosis of glomeruli
● Electron microscope: effacement of foot
processes of podocyte
● Immunofluorescence: nonspecific focal
deposits IgM
 MEMBRANOUS NEPHROPATHY
PATHOLOGY RISK FACTOR
● Type of nephrotic syndrome; ● Dysregulation of complement
inflammation of glomerular basement system
membrane mesangium - decreased
kidney function, proteinuria
● Immune complex/complement deposits COMPLICATION
trigger immune reactions ● Chronic renal failure, hypertension

TYPES
● Appearance under light microscopy SIGNS AND SYMPTOMS
- Type I,II,III
- All three can present as (NEPHROTIC SYNDROME)
nephrotic,nephritic syndrome ● Proteinuria, peripheral edema,
- Immunofluorescence: immune foamy urine,
complex-mediated MPGN, hyperlipidemia,lipiduria
complement-mediated MPGN
(NEPHRITIC SYNDROME)
● Hematuria, oliguria (low urine
production), hypertension
NEPHROTIC SYNDROME
● A clinical syndrome of massive proteinuria,defined as oedema,proteinuria>1g/m2/day or
hypoalbuminemia <25g/L and hypercholesterolemia
● No admission needed unless grossly edematous
● 60 mg/m2/day until remission or 40mg/m2/every alternate morning for 4 weeks only

RELAPSE
● Urine protein >1g/m2/day or urine dipstick 2+ for 3 consecutive days
● No admission needed unless grossly edematous
● 60 mg/m2/day until remission or 40mg/m2/every alternate morning for 4 weeks only

FREQUENT RELAPSE
● 2 Relapse within 6 months of initial diagnosis or 4 relapse within any 12 month period
● 60 mg/m2/day until remission or 40mg/m2/every alternate morning for 4 weeks only
● Taper prednisolone every 2 weeks & keep on as low as on alternate days dose as
possible for 6 months
REMISSION
● Urine dipstick trace or nil for 3 consecutive days

STEROID DEPENDANT
● 2 Consecutive relapses occurring during steroid tapering or within 14 days of cessation
of steroid
● If the child is not steroid toxic, reinduced with steroid and maintain as low of alternate
day prednisolone
● If steroid toxic then consider cyclophosphamide therapy (2-3mg/kg/day) for 8-12 weeks
COMPLICATIONS OF NEPHROTIC SYNDROME
INFECTION
● Children in relapse have increased susceptibility to bacterial infection due to urinary loss if
immunoglobulins and propending factor B, defective cell-mediated immunity and oedema
acting as potential culture medium
● Spontaneous bacterial peritonitis is the most frequent type of infection. Streptococcus
pneumoniae is the most common organism

THROMBOSIS
● Increase the risk of thromboembolic event because of the increased prothrombotic factors
and decreased fibrinolytic factors

HYPERLIPIDEMIA
● Serum lipids (cholesterol and triglyceride) are elevated because of hypoalbuminemia
stimulate generalised hepatic protein synthesis including lipoproteins. Lipid catabolism is
diminished as a result of reduced plasma level of lipoprotein lipase
THANK YOU