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3. Nephrotic Syndrome

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MUHAMMAD ALI BIN ABDUL RAZAK WAN AHMAD SYAZANI BIN MOHAMED NADIAH MOHD NASIR
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Page 1: 3. Nephrotic Syndrome

MUHAMMAD ALI BIN ABDUL RAZAKWAN AHMAD SYAZANI BIN MOHAMEDNADIAH MOHD NASIR

Page 2: 3. Nephrotic Syndrome

NAME: SUFIAH MAATREGISTRATION NUMBER: SB 00302319D.O.B: 5th NOVEMBER 2009GENDER : GIRLAGE: 1YEAR 1 MONTH OLDETHNIC GROUP: CAMBODIANDATE AND TIME OF ADMISSION: 4th DISEMBER 2010DATE OF DISCHARGE: -WARD OF ADMISSION: WARD 8C, HSBINFORMANT: FATHERRELIABILITY: GOODADDRESS: KG KUBU GAJAH, SG BULOH.

Page 3: 3. Nephrotic Syndrome

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.  

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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.

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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.

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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.

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At Hospital Sg. Buloh (Emergency Department),the doctor rechecked her urine sample and gave her

1. Prednisolone 25mg OD2. IV albumin 20% 5 ml/kg over 2 hours3. IV frusemide 1 mg/kg4. Syrup penicillin V 125mg BD

Then, she was admitted to the ward.

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System Complaints

General No loss of appetite,no weight loss

Respiratory No shortness of breath

Cardiovascular No diaphoresis during feeding and no cyanosis.

Gastrointestinal No constipation, no diarrheoa and no vomiting

Hematologic No pallor, no bleeding, no bruises

Genitourinary Decrease amount of urine,dark colour

Ear, nose and throat No ear and nose discharge

Central nervous No loss of consciousness, no seizure and no abnormal movement.

MusculoskeletalNo muscle weakness.no gross deformity

Skin No rash

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She had never been hospitalized before and no surgical history.

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Nil

ALLERGY HX Nil

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

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She was born on 5th 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.

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No admission to the NICU. No other complication such as fever

and neonate jaundice.

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Exclusive breastfeeding: 4 month At 5 month she already been

introduced to formula milk and semi-solid food.

The diet continues until today.

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Completed the immunization up to date.

No complication such as rash or fever.

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Up to her chronological age.

Gross motor: Able to walk with one hand held.

Fine motor: Neat pincer gripSpeech: She can talk 2-3 words with

meaning.Sosial: Shy and casting, could waves

‘bye bye’

Page 17: 3. Nephrotic Syndrome

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

Page 18: 3. Nephrotic Syndrome

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.

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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.

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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

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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.

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Anthropometry Weight : 8.7 kg Length : 71.0 cm Head circumference: 46.1 cm Impression : She is in 50th centile in

all anthropometry measurement

when checked on centile chart.

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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.

Page 25: 3. Nephrotic Syndrome

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

Page 26: 3. Nephrotic Syndrome

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

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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.

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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 4th 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 1st and 2nd heart sound

was heard. There was no additional heart sound or murmur.

  Impression: No abnormal findings

Page 29: 3. Nephrotic Syndrome

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 4th 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

Page 30: 3. Nephrotic Syndrome

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.

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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.

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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.

Page 33: 3. Nephrotic Syndrome

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

Page 34: 3. Nephrotic Syndrome

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.

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

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DIFFERENTIAL DIAGNOSIS

Points to support

Points 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

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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/L Increase

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

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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

Page 39: 3. Nephrotic Syndrome

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/dL

Increased

Albumin 8.0 3.5 - 5.0 g/dL

Increased

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

Page 40: 3. Nephrotic Syndrome

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

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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.

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FINAL DIAGNOSISIdiopathic Nephrotic Syndrome

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On admission :

MEDICATION

1. Syrup penicillin V 125mg BD

2. Tablet prednisolone 25mg OD

3. IV albumin 20% 5 ml/kg over 2 hours

4. IV frusemide 1 mg/kg OD

Monitor Nephrotic Chart : Daily weight, Blood Pressure, Urine protein, Fluid intake

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Page 46: 3. Nephrotic Syndrome

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

Page 47: 3. Nephrotic Syndrome

Classification Nephrotic

syndrome

Primary/ Idiopathic Secondary

•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.

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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

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Periorbital edema (earliest sign)Scrotal or vulval, leg, ankle edemaWeight gainAbdominal pain (Ascites)Respiratory distress (Pleural effusion)MalaiseDiarrhea

Nelson Essential Paediatrics Illustrated Textbook of Paediatrics

CLINICAL CLINICAL MANIFESTATIONMANIFESTATION

Page 50: 3. Nephrotic Syndrome

1. Urine protein – on test strips2. FBC and ESR3. Renal profile – urea, electrolyte, creatinine4. Serum cholesterol5. LFT - albumin6. Complement level7. Antistreptolysin O titre and throat swab

Nelson Essential Paediatrics Paediatric Protocols

INVESTIGATIONS

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Imaging Studies

UltrasoundPulsed doppler studiesVoid cysturethrogram (VCUG)IV pyelogramMRICT

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.

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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

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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

Page 54: 3. Nephrotic Syndrome

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

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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

Page 56: 3. Nephrotic Syndrome

THANK YOU

Page 57: 3. Nephrotic Syndrome

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

Page 58: 3. Nephrotic Syndrome

NEPHRITIC SYNDROMENephritic 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

Page 59: 3. Nephrotic Syndrome

Causes of Nephritic Syndrome

Post streptococal AGN Post infectious AGN Henoch schonlein perpura IgA nephropathy SLE Systemic vasculitis

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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

Page 61: 3. Nephrotic Syndrome

Management Nephrotic Nephritic Normal protein dietNo added salt when

edemaPenicillin 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

Page 62: 3. Nephrotic Syndrome

Nephrotic vs nephritic

• Nephrotic syndrome:• 1. Massive proteinuria

2. Hypoalbuminemia3. Edema4. Hyperlipidemia/hyperlipiduria

• Nephritic syndrome:• 1. Hematuria

2. Oliguria3. Azotemia4. Hypertension

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Side effects of steroids:1. Increased body weight2. Muscle wasting3. Growth retardation in

children4. Cutaneous striae5. Hypertension6. Increased susceptibility

to infections7. Delayed wound healing8. Hirsutism 9. Osteoporosis10. Diabetes peptic ulcer11. cataract


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