Sufiah, a 13-month-old Cambodian girl, was referred to the hospital with generalized swelling, especially around her eyes and abdomen. Physical examination revealed generalized edema with fluid in the abdomen. Laboratory tests showed protein in her urine and low albumin levels. She was diagnosed with idiopathic nephrotic syndrome based on her symptoms and test results.

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. 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. 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 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

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/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

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/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

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 Syrup penicillin V 125mg BD Tablet prednisolone 25mg OD IV albumin 20% 5 ml/kg over 2 hours 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: massive proteinuria (>3.5 g/day), hypoalbuminemia (serum albumin levels <2.5 g/dL) hyperlipidemia ( >250 mg/dL) 4) generalized edema

47. 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.

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 MANIFESTATION

50. 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 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. 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 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: hematuri a (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 A rthralgia s 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: Increased body weight Muscle wasting Growth retardation in children Cutaneous striae Hypertension Increased susceptibility to infections Delayed wound healing Hirsutism Osteoporosis Diabetes peptic ulcer cataract