3. Nephrotic Syndrome

1. MUHAMMAD ALI BIN ABDUL RAZAK
WAN AHMAD SYAZANI BIN MOHAMED
NADIAH MOHD NASIR

2. NAME: SUFIAH MAAT
REGISTRATION NUMBER: SB 00302319
D.O.B: 5
th
NOVEMBER 2009
GENDER : GIRL
AGE: 1YEAR 1 MONTH OLD
ETHNIC GROUP: CAMBODIAN
DATE AND TIME OF ADMISSION: 4
th
DISEMBER 2010
DATE OF DISCHARGE: -
WARD OF ADMISSION: WARD 8C, HSB
INFORMANT: FATHER
RELIABILITY: GOOD
ADDRESS: KG KUBU GAJAH, SG BULOH.

3. Sufiah, a 1 year old Cambodian girl was a
referred case from private clinic to Hospital
Sg Buloh due to generalize swelling of the
body especially around the eyes(periorbital)
and abdomen for further management.  

4. Previously well until 10 days prior to admission
Started to had fever.
10 days prior to admission  
Fever:
Father claim that the fever as low grade fever and it is on and
off.
no episode of seizure or convulsion
Her parents gave her Paracetamol syrup, fever subside but
reoccur afterward.

5. Swelling:
3 days before admission.
Periorbital swelling could be seen by her parents.
Started to cough on and off.
2 days before admission.
Abdomen was swelling as well as the periorbital area.
Went to private clinic.
Suspects that she had been bitten by insect that cause
allergic reaction and cause the swelling. The doctor gave
her anti-allergic drug ,cough medication and paracetamol
for her fever.

6. 1 day before admission
Swelling –worse
–more prominent
–more generalize to the whole body.
Another private clinic.
Examine her and also tests her urine. The result
shown that her urine had high level of protein, thus the
doctor referred this case to Hospital Sg Buloh for
further management.

7. At Hospital Sg. Buloh (Emergency Department),the
doctor rechecked her urine sample and gave her
Prednisolone 25mg OD
IV albumin 20% 5 ml/kg over 2 hours
IV frusemide 1 mg/kg
Syrup penicillin V 125mg BD
Then, she was admitted to the ward.

8. System Complaints
General No loss of appetite,no weight loss
Respiratory No shortness of breath
Cardiovascular No diaphoresis during feeding and no cyanosis.
GastrointestinalNo constipation, no diarrheoa and no vomiting
Hematologic No pallor, no bleeding, no bruises
Genitourinary Decrease amount of urine,dark colour
Ear, nose and throatNo ear and nose discharge
Central nervous
No loss of consciousness, no seizure and no abnormal
movement.
Musculoskeletal
No muscle weakness.
no gross deformity
Skin No rash

9. She had never been hospitalized before and no
surgical history.

10. Nil
ALLERGY HX
Nil

11. Her mother was healthy during pregnancy and
was not on medication. The mother went to
clinic regularly for follow up for her pregnancy.

12. She was born on 5
th
November 2009 at
Damansara Damai Clinic, full-term and by
normal spontaneous vaginal delivery. Her birth
weight was 2.7 kg and she was crying at birth.

13. No admission to the NICU.
No other complication such as fever and
neonate jaundice.

14. Exclusive breastfeeding: 4 month
At 5 month she already been introduced to
formula milk and semi-solid food.
The diet continues until today.

15. Completed the immunization up to date.
No complication such as rash or fever.

16. Up to her chronological age.
Gross motor: Able to walk with one hand held.
Fine motor: Neat pincer grip
Speech: She can talk 2-3 words with meaning.
Sosial: Shy and casting, could waves ‘bye bye’

17. Sofia Maat is the youngest children over 2 siblings.
Her sister is 4 years old and currently healthy.
Both her parents are alive and well. No family members that
had same problem like her.
Sister,4,
stays with
aunt, healthy
Sufiah Maat, 1 year old with fever
for 10 days and generalize
swelling 3 days prior to admission.
Mother
25,
healthy
Father
30,
healthy

18. She was active and happy at home. She stays in Kg Kubu
Gajah, Sg buloh in a one storey village house.
She lives with her father, mother, sibling and aunt with all basic
amenities.
Her mother is a housewife while her father work at night
market. Monthly income of the family is RM800.
The area of their house is not dengue prone area.

19. Due to her condition, she must stay in the hospital for
further monitoring.
This is her first admission to the Hospital so she quite
irritable
Her mother had to look after her in the hospital.
Leave her sister at home to be taken care by her aunt.
Economic status:
worried about the cost of her treatment
> monthly income only RM800
> Cambodians-might need to pay more compare to
Malaysian citizen.

21. General condition

Sufiah was sitting comfortably on her mother’s lap.
-She was conscious, alert and responsive to people.
-Not in pain
-°dysmorphism
-Her face looks puffy and swollen
-°abnormal movement seen
-Nutritional and hydration status was good
-branula attached on her left dorsum

22. Vital signs
Temperature : 36°C
Blood pressure : 92/52 mmHg
Pulse : 118 beat per minute, normal
volume, normal rhythm
Respiratory rate : 34 breathe per minute
Oxygen saturation : 100%
Impression : She is currently stable.

23. Anthropometry
Weight : 8.7 kg
Length : 71.0 cm
Head circumference: 46.1 cm
Impression: She is in 50
th
centile in all
anthropometry measurement
when checked on centile chart.

24. Examination for Hydration status
°sunken eyes
 tongue and mucous membranes in the oral
cavity were moist
 °loss of skin turgor.
 Capillary refill time was less than 2 seconds
 Impression: Her hydration status was good.

25. Examination of Face, Head & Neck, Limbs
Appearance : °dysmorphism, bilateral periorbital swelling, face
puffiness
Hands : Both hands slightly swollen
Pallor : °pallor
Cyanosis : °cyanosis
Oral cavity : Good oral hygiene, moist mucous membrane, °ulcer, pink
tongue
Eyes : °pallor, °jaundice, °discharge, °sunken eyes
ENT : °ear and nose discharge, °throat redness, °redness on her
tymphanic membrane
Shape of head : Normal head shape
Neck : °thyroid enlargement, °abnormal pulsation
Hair : °hair loss
Extremities : °cyanosis at nail bed, °finger clubbing for upper and lower
extremities, °palmar erythema, and capillary refill time is
less than two seconds, °koilonychias, °muscle wasting.
Oedema : There is bilateral leg pitting oedema up to midshin.
Impression : There was generalized oedema

26. Examination of back
°spinal deformities such as scoliosis, lordosis and kyphosis
°no tenderness
°sacral oedema
Impression: No abnormality detected
Examination of lymph nodes
°palpable lymph nodes in cervical, occipital, axillary and
inguinal areas
Impression: No abnormality detected

27. Developmental assessment
 Gross motor : Sufiah can stand up and walks with
support.
Fine motor : She can do a pincer grasp as she
picks up toys.
Social : She can hold bottle herself.
Language & hearing : Sufiah has started to say simple
words and response when
she was called.
Impression : Her development is corresponding
with her milestone.

28. Cardio-vascular system
On inspection, her chest moves symmetrically with
respiration. There was no chest wall deformity, no scar, no
dilated veins, no precordial bulge, no sign of respiratory
distress and no visible pulsation noted.
On palpation, apex beat was felt at 4
th
intercostals space,
mid-clavicular line. There was no left parasternal heaves and
no thrills at left sternal edge, pulmonary area and aortic
area.

On auscultation, normal 1
st
and 2
nd
heart sound was heard.
There was no additional heart sound or murmur.

Impression: No abnormal findings

29. Respiratory system
On inspection, the chest moves symmetrically with respiration on both
sides. There was no suprasternal, intercostals and subcostal recession. There
was no chest deformity and no scar seen. The chest was not hyperinflated.
On palpation, the trachea is centrally located and chest expansion was
symmetrical on both sides. The apex beat was located at 4
th
intercostals
space, mid-clavicular line. Normal vocal fremitus was noted
On percussion, both sides of her mid clavicular, mid axillary, and scapular
line segments of lungs were resonance. There was normal liver and cardiac
dullness.
On auscultation, the air entry was adequate on both sides of the lung.
Normal vesicular breath sound was heard. There were no added
sounds heard.
Impression: No abnormal findings

30. Abdominal examination
On inspection, her abdomen was symmetrically distended and
moves with respiration. The umbilicus was centrally located
and inverted. There was no abnormal scar, no dilated vein, no
visible pulsation and peristalsis noted.
On light palpitation, her abdomen was soft and non tender. On
deep palpation, there was no tenderness, no mass felt and no
hepatospleenomegaly. Both her kidneys were not ballotable

On percussion, there was positive shifting dullness and fluid
thrills.

On auscultation, normal bowel sound present with no renal
bruit.

Impression: Sufiah’s abdomen was distended with fluid.

31. Musculoskeletal system
°muscle wasting or hypertrophy on upper
and lower limbs
°no bony deformity
°signs of inflammation
normal movement of joint
Impression: No abnormal findings.

32. Nervous system
Higher function:
-Mental status: She was conscious and response to people. No
abnormal behaviour.
-Speech: She can say simple words.
Cranial nerves: cranial nerves were intact.
Motor function: Muscle bulk and muscle tone was normal. Muscle
power for all extremities grading 5/5. Biceps, triceps, supinator,
knee, and ankle reflexes were present. Plantar response was normal
with negative Babinski’s sign. The abdominal reflex was also normal.
Sensory functions:
A) Sensory: Normal sensation to touch, pain, temperature, vibration
and joint position sense.
B) Signs of meningeal irritation: No neck stiffness with negative
Brudzinski’s sign and Kernig’s sign.

33. Diagram of Body: Back & Front
Periorbital swelling and
face puffiness
Distended abdomen with
positive shifting dullness and
fluid thrills
Bilateral pitting oedema up
to mid shin

34. SUMMARY
Sufiah, a 13 months Cambodian girl was referred
to the hospital with complaints of generalized
swelling especially at her periorbital area and
abdomen which started 3 days prior to
admission. Her urine appeared cloudy, dark in
colour and little in amount. Physical examination
revealed generalized oedema with positive
shifting dullness and fluid thrills.

35. PROVISIONAL DIAGNOSIS
Nephrotic syndrome based on:
Presence of generalised oedema
Cloudy urine
Oligouria
Fluid thrills
Positive shifting dullness
Toddler age
Weight gain (8 kg- 8.6 kg)

36. DIFFERENTIAL DIAGNOSIS
Points to supportPoints to against
Acute
gromerulonephritis
-Generalized
oedema
-Dark urine
-Oligouria
-Fluid thrills
-Positive shifting
dullness
-Toddler age
Cardiac failure-Generalized
oedema
-Fluid thrills
-Positive shifting
dullness
-Dark urine
-Oligouria
-Hypertension
-Clubbing
-Crepitations

37. INVESTIGATION
General Investigations
full blood count
Impression: Platelet and white blood cell count were elevated
Result Normal range Remarks
WBC 22.0 4.5-13.5 x 10*9/LIncrease
Hb 12.4 11.5-14.5 g/dL Normal
Plt 880 150-4– x 10*3 uL Increase
Haematocrit 37.2 37-45% Normal

38. Renal profile
Impression: Low creatinine level
Result Normal range Remarks
Urea 3.6 1.7-6.4 mmol/L Normal
Sodium 134 135-150 mmol/L Normal
Potassium 4.6 3.5-5 mmol/L Normal
Chloride 98 98.0-107.0 mmol/L Normal
Creatinine 27.7 44-88 mmol/L Decrease

39. Liver function test
Impression: There was markedly increased in total
protein. This might be due to albumin infusion.
Result Normal
range
Remarks
Total protein 45.0 6.3 - 7.9 g/dLIncreased
Albumin 8.0 3.5 - 5.0 g/dLIncreased
Globulin 37.0 9 - 48 U/L Normal
Bilirubin 1.8 0.1 - 1.0
mg/dL
Increased
Alanine
transaminase
19 7 - 55 U/L Normal
Alanine
transferase
217 45 - 115 U/L Increased

40. Specific Investigations
Urine Full Examination Microscopy Elements (UFEME)
-protein: 3+
-blood: 3+
-nitrite,leukocyte, ketone: negative
Urine Protein Creatinine Index
-no result can be obtained from the medical record
Urine Culture and Sensitivity
-blood stain urine
-heavy mixed growth

41. Antistreptolysin O titre- to exclude post
streptococcus glomerulonephritis
Anti-nuclear factor to exclude SLE
Serum complement (C3 and C4) to exclude post
infectious glomerulonephritis and SLE.

42. FINAL DIAGNOSIS
Idiopathic Nephrotic Syndrome

44. On admission :
MEDICATION
4.Syrup penicillin V 125mg BD
5.Tablet prednisolone 25mg OD
6.IV albumin 20% 5 ml/kg over 2 hours
7.IV frusemide 1 mg/kg OD
Monitor Nephrotic Chart : Daily weight, Blood
Pressure, Urine protein, Fluid intake

46. Nephrotic Syndrome
Definition
A collection of signs associated with glomerular
disorders characterized by:
1)massive proteinuria (>3.5 g/day),
2)hypoalbuminemia (serum albumin levels <2.5
g/dL)
3)hyperlipidemia ( >250 mg/dL)
4) generalized edema

47. Classification
Nephrotic
syndrome
Primary/ IdiopathicSecondary
•Minimal change disease
•Membranous GN
•Focal segmental
glomerulosclerosis
•Membranoproliferative
GN
•IgA nephropathy
•Diabetes Mellitus
•Amyloidosis
•Systemic lupus
erythematosus
•Ingestion of drugs
(lithium,
penicillamine,”street
heroin”)
•Infections (malaria,
syphilis, hepatitis B, HIV)
•Malignancy ( carcinoma,
melanoma)
•Miscellaneous (bee-sting
allergy, hereditary
nephritis)
Congenital
Present during the first 6
months life
• Finnish type is an
autosomal recessive
disorder most common in
Scandinavian and due to
mutation in component
protein in the glomerulus
filtration slit.
• Diffuse mesangial
sclerosis
which is a heterogenous
group of abnormalities.

48. Inflammatory reaction
Derangement in capillary
walls of glomeruli
Increase permeability to
plasma protein
Proteinuria
Allows protein to
escape from plasma
into glomerular filtrate
Drop in plasma
colloid osmotic
pressure
Fluid escapes into
tissues
Edema
Pathophysiology of
Nephrotic
Syndrome

49. •Periorbital edema (earliest sign)
•Scrotal or vulval, leg, ankle edema
•Weight gain
•Abdominal pain (Ascites)
•Respiratory distress (Pleural effusion)
•Malaise
•Diarrhea
Nelson Essential Paediatrics Illustrated Textbook of Paediatrics
CLINICAL CLINICAL
MANIFESTATIONMANIFESTATION

50. 1.Urine protein – on test strips
2.FBC and ESR
3.Renal profile – urea, electrolyte, creatinine
4.Serum cholesterol
5.LFT - albumin
6.Complement level
7.Antistreptolysin O titre and throat swab
Nelson Essential Paediatrics Paediatric
Protocols
INVESTIGATIONS

51. Imaging Studies
Ultrasound
Pulsed doppler studies
Void cysturethrogram (VCUG)
IV pyelogram
MRI
CT
Diagnostic Studies
First morning specimen:Urine protein-to-creatinine ratio (normal :
<0.2)
Serum C3 complement level
RENAL BIOPSY : only indicated if a child does not respond to the
prednisolone therapy within 4 weeks.

52. MANAGEMENT
Bed rest
Diet – adequate calories ,normal protein diet with
salt restriction
Antibiotic – penicillin V BD during relapse
Fluid status :
assess for hemodynamic status. ( underfilling or
overfilling )
Diuretic therapy
Human albumin 25% parenterally with IV loop
diuretic
( frusemide ) to produce diuresis

53. 1.Cortocosteroid Therapy
Effective in inducing remission of NS
Remission – urine dipstick is trace or nil for 3
consecutive days
Relapse – urine albumin excretion > 40 mg /m²/hr OR
urine dipstix 2+ or > for 3 consecutive days
Frequent Relapses
- Two or more relapses within 6 months of initial
response or
- 4 or more relapses within any 12 month period
2.Cyclophosphamide therapy
indicated for child who show signs of steroid toxicity

54. Initially
-60 mg/m²/day for 4 weeks
Prednisolone 40 mg/m²/alternate
day for 4 weeks then taper at 25%
monthly over 4 month
1. Response
-Prednisolone 60 mg/m²/day till
remission
-40 mg/m²/alternate day for weeks
then stop
2. RELAPSE
Reinduce (2), then taper & keep
low dose alternate day
prednisolone 0.1-0.5 mg/kg/dose
for 6 month
3. Frequent relapse
Treat as (3) if not steroid toxic,
consider cyclophosphamide if
steroid toxic
4. Relapse while on prednisolone
2-3 mg/kg/day for 8-12
weeks (cumulative dose
168 mg/kg
5. Oral cyclophosphamide
-not steroid toxic: treat as 2 & 3
- If steroid toxic paeds nephro
6. Relapse post cyclophosphamide
No response
Renal biopsy

55. 1. Infection- bacteremia and peritonitis – patient with
relapse have high risk of infection with capsulated bacteria
esp pneumococus / E.coli
2. Side effect of steroid
3. Hypovolemia – result from use of diueresis or diarrhea
4. Hypercoagulable state with risk of tromboembolism
– d/t loss of protein -> urinary lose of antithombin,
thrombocytosis (steroid therapy) -> increase synthesis of
clotting factor and increased blood viscosity ->predispose to
thrombosis

56. THANK YOU

57. MINIMAL CHANGES DISEASES.
Relatively benign disorder.
Most frequent cause of NS in children(1-7 years).
Clinical features
Insidious development of NS.
No hypertension and preserved renal function.
Good prognosis.
CAUSES

58. NEPHRITIC SYNDROME
Nephritic syndrome is defined by:
hematuria (usually with dysmorphic RBCs), and
hypertension,
oliguria (400 mL/day of urine).
Uremia - due to retention of waste products
Azotemia (elevated blood nitrogen)
Or come with symptom of underlying problems
Triad of sinusitis, pulmonary infiltrates, and nephritis suggesting Wegener
granulomatosis
Nausea/vomiting, abdominal pain, and purpura observed with Henoch-
Schönlein purpura
Arthralgias associated with systemic lupus erythematosus (SLE)
Hemoptysis occurring with Goodpasture syndrome or idiopathic
progressive glomerulonephritis
Skin rashes observed with a hypersensitivity vasculitis or systemic lupus
erythematosus; also possibly due to the purpura that can occur in
hypersensitivity vasculitis, cryoglobulinemia, and Henoch-Schönlein
purpura

59. Causes of Nephritic Syndrome
Post streptococal AGN
Post infectious AGN
Henoch schonlein perpura
IgA nephropathy
SLE
Systemic vasculitis

60. Focal Segmental
Glomerulosclerosis
Lesion characterized histologically by sclerosis.
Pathogenesis is unknown.
Clinical course:
Little tendency for spontaneous remission of
idiopathic FSGS.
Poor respond to steroid therapy.
Bad prognosis.
CAUSES

61. Management
Nephrotic Nephritic
Normal protein diet
No added salt when edema
Penicillin V at diag. & during
relapse esp. with gross
edema
Diuretics is not necessary
when steroid responsive
Human albumin-in grossly
edematous
Hemodynamic status -
Check for sign of
hypervolamia or
hypovolaemia
Strict monitoring-luid intake,
urine output, daily weight, BP
chart
Fluid restriction during
oligouric phase
Diuretics
Look for complication of post
strep AGN – hypertensive
encephalopathy (usu. seizure),
pulm edema (lft.vent failure),
acute renal failure

62. Nephrotic vs nephritic
•Nephrotic syndrome:
•1. Massive proteinuria
2. Hypoalbuminemia
3. Edema
4. Hyperlipidemia/hyperlipiduria
•Nephritic syndrome:
•1. Hematuria
2. Oliguria
3. Azotemia
4. Hypertension

63. Side effects of steroids:
1.Increased body weight
2.Muscle wasting
3.Growth retardation in
children
4.Cutaneous striae
5.Hypertension
6.Increased susceptibility
to infections
7.Delayed wound healing
8.Hirsutism
9.Osteoporosis
10.Diabetes peptic ulcer
11.cataract