Extern conference

1. Extern conference A 1-year-3-month-old boy presented with generalized edema for 1 month 20 December 2007

3. History A 1-year-3month-old boy • Chief complaint : Generalized edema for 1 month • Present history: • He was no underlying disease. • 1 month PTA, his mother noticed that her child had enlarged abdomen and edema of both feet and ankles. The patient’s body weight increased 4 kg in 2mo.

4. History - Normal urination and defecation, no gross hematuria • No previous history of fever, cough, sore throat and skin infection • No rash, no arthralgia • No dyspnea, no orthopnea • No chronic diarrhea

5. History Past history Caesarean section due to previous C/S birth weight 2616 gm, Apgar score 9,10 Family history No history of kidney disease in family Nutritional status Solid food 3 feeds Powdered milk 8 oz 3 feeds per day

6. History • Drugs and immunization • No history of drug allergy • No routine drug use • Immunization • Up to date • Developmental history • Say some words and walk

7. Physical Examination • Vital signs : T 36.9 C, PR140/min, BP 99/72 mmHg, RR 32/min • GA : 1 3/12-year-Thai boy, uncooperative, not pale, no jaundice, no dyspnea, no cyanosis, puffy eye lids, generalized pitting edema 1+ • Ht 80 cm (P50-75), BW 11 kg (P50-75),HC 46 cm (P25-50), Abdominal circumference 49 cm

8. Physical Examination • HEENT : no dental caries no injected pharynx and tonsils • Lungs : clear • CVS : normal S1 and S2, no murmur • Abdomen : soft, not tender, liver and spleen not palpable, no palpable mass, mild ascites, bowel sound active • KUB: CVA-not tender, bimanual palpation-negative • Genitalia: scrotal edema • Others: unremarkable

9. PROBLEM LIST • Generalized pitting edema for 1 month • Weight gain 4 kg in 2 months

10. DIFFERENTIAL DIAGNOSIS • Generalized edema • Nephrotic syndrome • Acute or chronic glomerulonephritis • Congestive heart failure • Protein losing enteropathy • Protein malnutrition

11. PROVISIONAL DIAGNOSIS • Nephrotic syndrome

12. Characteristic features of nephrotic syndrome • 1.Generalized pitting edema • 2.Hypoalbuminemia (≤2.5g/dl) • 3.Hypercholesterolemia(≥250g/dl) • 4.Heavy proteinuria (≥40 mg/m²/hr)

13. Question 1 “What is the initial investigation in this patient?”

14. INVESTIGATIONS AT INITIAL PRESENTATION • Complete blood count • Renal profiles, urinalysis and quantification for urinary protein excretion • Serum albumin • Lipid profiles

15. INVESTIGATIONS • CBC(11/12/50) • Hb 13.3 g/dl , Hct 39.9% • WBC 14070 /ul (N 32.2%, L 58.9%, M 4.8%, E 3.7%, B 0.4%) • Plt 428,000 /ul • Urinalysis(11/12/50) • pH 7.0, spgr.1.010 • protein 4+, sugar neg, • wbc 0-1/HP, rbc 1-2/HP

16. INVESTIGATIONS • Blood chemistry(11/12/50) • BUN 9 mg/dl, Cr 0.1 mg/dl • Cholesterol 366 mg/dl • Albumin 1.5 g/dl • Na 131 mmol/L • K 3.8 mmol/L • Cl 104 mmol/L • HCO₃ 24 mmol/L

17. INVESTIGATIONS • Urine 24 hrs • Volume 440 ml (1.67ml/kg/hr) • urine creatinine 31.3 mg/24hr • urine protein 607 mg/24hr(50.6mg/m²/hr)

18. Diagnosis • Nephrotic syndrome

19. Types of nephrotic syndrome • Primary nephrotic syndrome(90%) • Secondary nephrotic syndrome(10%)

20. Nephrotic syndrome • Primary nephrotic syndrome(90%) • Most common at age 2-6 yr (6 mo. patient has been reported) • Male : female = 2 : 1

21. Distribution of primary nephrotic syndrome * Adapted from a report of the International Study of Kidney Disease in Children

22. Nephrotic syndrome • Secondary nephrotic syndrome(10%) • Suspected in • Age>8 yrs • Hypertension • Hematuria • Renal dysfunction • Extrarenal symptoms (rash, arthralgia, fever)

23. Secondary nephrotic syndrome • Causes • Infections • Drugs • Immunologic or allergic disorders • Malignant disease • Glomerular hyperfiltration

24. In this patient • Primary nephrotic syndrome • More common (90%) • Lower age group • Male • No clinical suspection of secondary nephrotic syndrome

25. Management • General • Low salt, normal protein for age diet • Salt poor albumin/diuretic if indicated • Clear infection • Education • Immunization

26. Management • Specific • Prednisolone • Cyclophosphamide • Cyclosporin • Levamisole

27. Indications for renal biopsy • Pretreatment • Macroscopic hematuria • Persistent hypertension and microscopic hematuria • Renal failure not attributable to hypovolemia • Low plasma C3 • Onset age < 6 months

28. Before start steroid therapy • Complete physical examination • CXR, PPD skin test • Stool conc. for parasite • Dental exam

29. In this patient • Complete physical examination • CXR: no infiltration • PPD skin test: negative • Stool conc. for parasite: not found 3 days • Dental exam: no dental caries

30. CORTICOSTEROIDS 1 . Nephrotic Syndrome Initial Diagnosis Prednisolone 60 mg/m2/day (max 80/day) for 4 weeks Response No Response Prednisolone 40 mg/m2/48 hours for 4 weeks Renal Biopsy *Discontinue *Steroid taper at 25% monthly over 4 months 2. Relapse

31. Progression • Admit 11-14/12/50 • V/S stable • BW 11 – 11.1 kg • Balanced I/O

32. Discharge • 14/12/50 • Home med Prednisolone[5] 4 tab oral OD pc • Follow up OPD2weeks with urinalysis

33. Follow up 1 . Nephrotic Syndrome Prednisolone 60 mg/m2/day (max 80/day) for 4 weeks Response No Response Prednisolone 40 mg/m2/48 hours for 4 weeks Renal Biopsy *Discontinue *Steroid taper at 25% monthly over 4 months

34. INDICATIONS FOR RENAL BIOPSY • Steroid resistant • Steroid dependence • Frequent relapse

35. Definitions • REMISSION: • Urinary protein excretion < 4 mg/m2/hour • or urine dipstix nil/trace for 3 consecutive days. • RELAPSE: • Urinary protein excretion > 40 mg/m2/hour • or urine dipstix ++ or more for 3 consecutive days. • FREQUENT RELAPSES: • Two or more relapses within 6 months of initial response • or four or more relapses within any 12 month period.

36. Definitions • STEROID DEPENDENCE: • Two consecutive relapses occurring during the period of steroid taper or within 14 days of its cessation. • STEROID RESISTANCE: • Failure to achieve remission in spite of 4 weeks of standard prednisolone therapy

37. Question 2 ‘What will you advice the patient’s caregiver ?”

38. Prognosis • The prognosis depends on the cause of nephrotic syndrome. • It is usually good in children. • Minimal change disease responds very well to steroids and does not cause chronic renal failure. • Focal segmental glomerulosclerosis frequently lead to end stage renal disease.

39. Prognosis • Children who present with hematuria and hypertension are more likely to be steroid resistant • Poor patient response to steroid therapy seems to be the finding most predictive of a poor outcome • Frequent relapses are more common with young age of onset and in boys.

40. Patient Education • Nephrotic syndrome is a chronic illness characterized by relapses and remissions • Ensure normal activity and school attendance. • Infections are an important cause of morbidity and mortality

41. Patient Education • Live vaccines can be administered 6 weeks after cessation of corticosteroid therapy. • Peer support and psychological counseling are important.

43. THANK YOU