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CASE PRESENTATION

09/12/2010MUHAMMAD ALI BIN ABDUL RAZAK

WAN AHMAD SYAZANI BIN MOHAMED

NADIAH MOHD NASIR
http://www.uitm.edu.my/uitm/index.php?option=com_banners&task=click&bid=17


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DEMOGRAPHIC DETAILS

NAME: SUFIAH MAAT

REGISTRATION NUMBER: SB 00302319

D.O.B: 5th NOVEMBER 2009

GENDER : GIRL

AGE: 1YEAR 1 MONTH OLD

ETHNIC GROUP: CAMBODIAN

DATE AND TIME OF ADMISSION: 4thDISEMBER 2010

DATE OF DISCHARGE: -

WARD OF ADMISSION: WARD 8C, HSB

INFORMANT: FATHER

RELIABILITY: GOOD

ADDRESS: KG KUBU GAJAH, SG BULOH.


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PRESENTING COMPLAINT

Sufiah, a 1 year old Cambodian girl was a

referred case from private clinic to Hospital

Sg Buloh due to generalize swelling of thebody especially around the

eyes(periorbital) and abdomen for further

management.


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HISTORY OF PRESENTING COMPLAINT

Previously well until 10 days prior to admission

Started to had fever.

10 days prior to admissionFever:

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 gaveher anti-allergic drug ,cough medication and paracetamol

for her fever.


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CONTINUE

1 day before admission

Swellingworse

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, thusthe 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. Prednisolone25mg OD

2. IV albumin20% 5 ml/kg over 2 hours

3. IV frusemide1 mg/kg

4. Syrup penicillin V 125mg BD

Then, she was admitted to the ward.


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SYSTEMIC REVIEWSystem 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 nervousNo loss of consciousness, no seizure and no abnormal

movement.

MusculoskeletalNo muscle weakness.

no gross deformity

Skin No rash


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PAST MEDICAL/SURGICAL HX

She had never been hospitalized before and

no surgical history.


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DRUG HX

Nil

ALLERGY HX

Nil


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ANTENATAL HX

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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BIRTH HX

She was born on 5thNovember 2009 at

Damansara Damai Clinic, full-term and by

normal spontaneous vaginal delivery. Her

birth weight was 2.7 kg and she was cryingat birth.


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NEONATAL HX

No admission to the NICU.

No other complication such as fever and

neonate jaundice.


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FEEDING HX

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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IMMUNISATION HX

Completed the immunization up to date.

No complication such as rash or fever.


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DEVELOPMENTAL HX

Up to her chronological age.

Gross motor: Able to walk with one handheld.

Fine motor: Neat pincer grip

Speech: She can talk 2-3 words withmeaning.

Sosial: Shy and casting, could waves bye

bye


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FAMILY HX

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


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SOCIAL AND ENVIRONMENTAL HX

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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EFFECT OF ILLNESS ON PTS AND FAMILY

Due to her condition, she must stay in the hospital forfurther monitoring.

This is her first admission to the Hospital so she quiteirritable

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 toMalaysian citizen.


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PHYSICAL

EXAMINATION


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General condition

Sufiah was sitting comfortably on her mothers 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 : 36C

Blood pressure : 92/52 mmHg

Pulse : 118 beat per minute, normalvolume, 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 50thcentile 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.


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Examination of Face, Head & Neck, Limbs

Appearance : dysmorphism, bilateral periorbital swelling, facepuffiness

Hands : Both hands slightly swollen

Pallor : pallor

Cyanosis : cyanosis

Oral cavity : Good oral hygiene, moist mucous membrane, ulcer, pinktongue

Eyes : pallor, jaundice, discharge, sunken eyes

ENT : ear and nose discharge, throat redness, redness on hertymphanic 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 lowerextremities, 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


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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 andinguinal areas

Impression: No abnormality detected


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Developmental assessment

Gross motor : Sufiah can stand up and walks withsupport.

Fine motor : She can do a pincer grasp as shepicks up toys.

Social : She can hold bottle herself.

Language & hearing : Sufiah has started to say simplewords and response whenshe was called.

Impression : Her development is correspondingwith 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, nodilated veins, no precordial bulge, no sign of respiratorydistress and no visible pulsation noted.

On palpation, apex beat was felt at 4thintercostals space,

mid-clavicular line. There was no left parasternal heaves andno thrills at left sternal edge, pulmonary area and aorticarea.

On auscultation, normal 1stand 2ndheart sound was heard.

There was no additional heart sound or murmur.

Impression: No abnormal findings


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Respiratory system

On inspection, the chest moves symmetrically with respiration on bothsides. 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 wassymmetrical on both sides. The apex beat was located at 4thintercostalsspace, mid-clavicular line. Normal vocal fremitus was noted

On percussion,both sides of her mid clavicular, mid axillary, and scapularline segments of lungs were resonance. There was normal liver and cardiacdullness.

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


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Abdominal examination

On inspection, her abdomen was symmetrically distended and

moves with respiration. The umbilicus was centrally locatedand inverted. There was no abnormal scar, no dilated vein, novisible pulsation and peristalsis noted.

On light palpitation, her abdomen was soft and non tender. Ondeep palpation, there was no tenderness, no mass felt and nohepatospleenomegaly. Both her kidneys were not ballotable

On percussion, there was positive shifting dullness and fluidthrills.

On auscultation, normal bowel sound present with no renalbruit.

Impression: Sufiahsabdomen was distended with fluid.


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Musculoskeletal system

muscle wasting or hypertrophy on upperand 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. Noabnormal behaviour.

-Speech: She can say simple words.

Cranial nerves: cranial nerves were intact.

Motor function: Muscle bulk and muscle tone was normal. Musclepower for all extremities grading 5/5. Biceps, triceps, supinator,knee, and ankle reflexes were present. Plantar response was normalwith negative Babinskis sign. The abdominal reflex was also normal.

Sensory functions:

A) Sensory: Normal sensation to touch, pain, temperature, vibrationand joint position sense.

B) Signs of meningeal irritation: No neck stiffness with negativeBrudzinskis sign and Kernigs sign.


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Diagram of Body: Back & Front

Periorbital swelling andface puffiness

Distended abdomen withpositive shifting dullness and

fluid thrills

Bilateral pitting oedema up

to mid shin


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SUMMARY

Sufiah, a 13 months Cambodian girl was referred

to the hospital with complaints of generalized

swelling especially at her periorbital area andabdomen which started 3 days prior to

admission. Her urine appeared cloudy, dark incolour and little in amount. Physical examination

revealed generalized oedema with positiveshifting dullness and fluid thrills.


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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)


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DIFFERENTIAL DIAGNOSIS

Points to support Points to against

Acutegromerulonephritis

-Generalizedoedema-Dark urine

-Oligouria-Fluid thrills-Positive shiftingdullness

-Toddler age

Cardiac failure -Generalized

oedema-Fluid thrills-Positive shiftingdullness

-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 880 150-4x 10*3 uL Increase

Haematocrit 37.2 37-45% Normal


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Renal profile

Impression: Low creatinine level

Result Normal range RemarksUrea 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


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Liver function test

Impression: There was markedly increased in totalprotein. This might be due to albumin infusion.

Result Normalrange 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 NormalBilirubin 1.8 0.1 - 1.0

mg/dLIncreased

Alaninetransaminase

19 7 - 55 U/L Normal

Alaninetransferase

217 45 - 115 U/L Increased


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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 poststreptococcus glomerulonephritis

Anti-nuclear factor to exclude SLE

Serum complement (C3 and C4) to exclude post

infectious glomerulonephritis and SLE.


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FINAL DIAGNOSIS

Idiopathic Nephrotic Syndrome


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PRINCIPLE OF MANAGEMENT


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On admission :

MEDICATION1. 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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DISCUSSION


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ClassificationNephrotic

syndrome

Primary/ Idiopathic Secondary

Minimal change disease

Membranous GNFocal segmental

glomerulosclerosis

Membranoproliferative

GN

IgA nephropathy

Diabetes Mellitus

AmyloidosisSystemic 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 lifeFinnish type is an

autosomal recessive

disorder most common in

Scandinavian and due to

mutation in component

protein in the glomerulus

filtration slit.

Diffuse mesangialsclerosis

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 intotissues

Edema

Pathophysiology ofNephrotic

Syndrome


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Periorbital edema (earliest sign)

Scrotal or vulval, leg, ankle edema

Weight gainAbdominal pain (Ascites)

Respiratory distress (Pleural effusion)

Malaise

Diarrhea

Nelson Essential Paediatrics Illustrated Textbook of Paediatrics

CLINICAL

MANIFESTATION


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1. Urine proteinon test strips2. FBC and ESR

3. Renal profileurea, electrolyte, creatinine

4. Serum cholesterol

5. LFT - albumin6. Complement level

7. Antistreptolysin O titre and throat swab

Nelson Essential Paediatrics Paediatric

Protocols

INVESTIGATIONS


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Imaging Studies

Ultrasound

Pulsed doppler studiesVoid cysturethrogram (VCUG)

IV pyelogram

MRI

CT

Diagnostic Studies

First morning specimen :Urine protein-to-creatinine ratio (normal :


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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 loopdiuretic( frusemide ) to produce diuresis


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1. Cortocosteroid Therapy

Effective in inducing remissionof NS

Remissionurine dipstick is trace or nil for 3consecutive days

Relapseurine albumin excretion > 40 mg /m/hr ORurine dipstix 2+ or > for 3 consecutive days

Frequent Relapses

- Two or more relapses within 6 months of initialresponse or

- 4 or more relapses within any 12 month period

2. Cyclophosphamide therapy indicated for child who show signs of steroid toxicity


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Initially

-60 mg/m/day for 4 weeks

Prednisolone 40 mg/m/alternate

day for 4 weeks then taper at 25%

monthly over 4 month

1. Response

-Prednisolone 60 mg/m/day tillremission

-40 mg/m/alternate day for weeks

then stop

2. RELAPSE

Reinduce (2), then taper & keeplow 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 toxic4. Relapsewhile 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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THANK YOU


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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


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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-Schnlein 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 inhypersensitivity vasculitis, cryoglobulinemia, and Henoch-Schnleinpurpura


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Causes of Nephritic Syndrome

Post streptococal AGN

Post infectious AGN

Henoch schonlein perpura

IgA nephropathy

SLE

Systemic vasculitis


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Focal SegmentalGlomerulosclerosis Lesion characterized histologically by sclerosis.

Pathogenesis is unknown.

Clinical course:

Little tendency for spontaneous remission ofidiopathic FSGS.

Poor respond to steroid therapy.

Bad prognosis.

CAUSES


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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 ofhypervolamia orhypovolaemia

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


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Nephrotic vs nephritic

Nephrotic syndrome:

1. Massive proteinuria2. Hypoalbuminemia

3. Edema4. Hyperlipidemia/hyperlipiduria

Nephritic syndrome:

1. Hematuria

2. Oliguria3. Azotemia4. Hypertension


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Side effects of steroids:

1. Increased body weight

2. Muscle wasting

3. Growth retardation inchildren

4. Cutaneous striae

5. Hypertension

6. Increased susceptibilityto infections

7. Delayed wound healing

8. Hirsutism

9. Osteoporosis

10. Diabetes peptic ulcer

11. cataract

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