CASE PRESENTATION

09/12/2010
MUHAMMAD ALI BIN ABDUL RAZAK WAN AHMAD SYAZANI BIN MOHAMED NADIAH MOHD NASIR

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: 4th DISEMBER 2010 DATE OF DISCHARGE: WARD OF ADMISSION: WARD 8C, HSB INFORMANT: FATHER RELIABILITY: GOOD ADDRESS: KG KUBU GAJAH, SG BULOH.

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 the body especially around the eyes(periorbital) and abdomen for further management.

HISTORY OF PRESENTING COMPLAINT

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.

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.

CONTINUE
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.

1. 2.

3.
4.

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.

SYSTEMIC REVIEW
System
General Respiratory

Complaints
No loss of appetite,no weight loss No shortness of breath

Cardiovascular Gastrointestinal
Hematologic Genitourinary Ear, nose and throat

No diaphoresis during feeding and no cyanosis. No constipation, no diarrheoa and no vomiting

No pallor, no bleeding, no bruises Decrease amount of urine,dark colour No ear and nose discharge

Central nervous
Musculoskeletal Skin

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

No muscle weakness. no gross deformity No rash

PAST MEDICAL/SURGICAL HX

She had never been hospitalized before and no surgical history.

DRUG HX

Nil

ALLERGY HX

Nil

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.

BIRTH HX

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.

NEONATAL HX
No admission to the NICU. No other complication such as fever and neonate jaundice.

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.

IMMUNISATION HX
Completed the immunization up to date. No complication such as rash or fever.

DEVELOPMENTAL HX

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

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.
Mother 25, healthy Father 30, healthy

Sister,4, stays with aunt, healthy

Sufiah Maat, 1 year old with fever for 10 days and generalize swelling 3 days prior to admission.

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.

EFFECT OF ILLNESS ON PTS AND FAMILY

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.

PHYSICAL EXAMINATION

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

Vital signs
Temperature

: 36C 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.

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.

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.

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

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

Developmental assessment

Gross motor support. Fine motor picks up toys. Social Language & hearing

: Sufiah can stand up and walks with : She can do a pincer grasp as she : She can hold bottle herself. : Sufiah has started to say simple words and response when she was called. : Her development is corresponding with her milestone.

Impression

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

On palpation, apex beat was felt at 4th intercostals space,

On auscultation, normal 1st and 2nd heart sound was heard.

There was no additional heart sound or murmur.

Impression: No abnormal findings

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

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: Sufiahs abdomen was distended with fluid.

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.

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 Babinskis 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 Brudzinskis sign and Kernigs sign.

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

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.

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)

DIFFERENTIAL DIAGNOSIS
Points to support Acute -Generalized gromerulonephritis oedema -Dark urine -Oligouria -Fluid thrills -Positive shifting dullness Cardiac failure -Generalized oedema -Fluid thrills -Positive shifting dullness Points to against -Toddler age

-Dark urine -Oligouria -Hypertension -Clubbing -Crepitations

INVESTIGATION
General Investigations

full blood count

Result WBC Hb Plt Haematocrit 22.0 12.4 880 37.2 Normal range 4.5-13.5 x 10*9/L 11.5-14.5 g/dL 150-4 x 10*3 uL 37-45% Remarks Increase Normal Increase Normal

Impression: Platelet and white blood cell count were elevated

 Renal profile
Result
Urea Sodium Potassium 3.6 134 4.6

Normal range
1.7-6.4 mmol/L 135-150 mmol/L 3.5-5 mmol/L

Remarks
Normal Normal Normal

Chloride
Creatinine

98
27.7

98.0-107.0 mmol/L
44-88 mmol/L

Normal
Decrease

Impression: Low creatinine level

 Liver function test

Result Total protein Albumin Globulin Bilirubin Alanine transaminase Alanine transferase 45.0 8.0 37.0 1.8 19 217 Normal range 6.3 - 7.9 g/dL 3.5 - 5.0 g/dL 9 - 48 U/L 0.1 - 1.0 mg/dL 7 - 55 U/L 45 - 115 U/L Remarks Increased Increased Normal Increased Normal Increased

Impression: There was markedly increased in total

protein. This might be due to albumin infusion.

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

 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.

FINAL DIAGNOSIS
Idiopathic Nephrotic Syndrome

PRINCIPLE OF MANAGEMENT

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

DISCUSSION

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

Nephrotic syndrome
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.

Classification
Secondary
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)

Primary/ Idiopathic
Minimal change disease Membranous GN Focal segmental glomerulosclerosis Membranoproliferative GN IgA nephropathy

Inflammatory reaction
Derangement in capillary walls of glomeruli

Increase permeability to plasma protein

Pathophysiology of Nephrotic Syndrome

Allows protein to escape from plasma into glomerular filtrate

Proteinuria
Drop in plasma colloid osmotic pressure Fluid escapes into tissues

Edema

CLINICAL MANIFESTATION
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

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

Nelson Essential Paediatrics Paediatric Protocols

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.

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

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 /m/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

Initially -60 mg/m/day for 4 weeks

1. Response
Prednisolone 40 mg/m/alternate day for 4 weeks then taper at 25% monthly over 4 month 2. RELAPSE -Prednisolone 60 mg/m/day till remission -40 mg/m/alternate day for weeks then stop

No response Renal biopsy

6. Relapse post cyclophosphamide -not steroid toxic: treat as 2 & 3 - If steroid toxic paeds nephro

5. Oral cyclophosphamide 2-3 mg/kg/day for 8-12 weeks (cumulative dose 168 mg/kg

3. Frequent relapse
Reinduce (2), then taper & keep low dose alternate day prednisolone 0.1-0.5 mg/kg/dose for 6 month
4. Relapse while on prednisolone

Treat as (3) if not steroid toxic, consider cyclophosphamide if steroid toxic

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

THANK YOU

CAUSES
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.

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 HenochSchnlein 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-Schnlein purpura

Causes of Nephritic Syndrome

Post streptococal AGN Post infectious AGN

Henoch schonlein perpura

IgA nephropathy SLE

Systemic vasculitis

CAUSES
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.

Management
Nephrotic
Normal protein diet No added salt when edema Penicillin V at diag. & during

Nephritic
Strict monitoring-luid intake,

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

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

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

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