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Nephrotic Syndrome Prepared by Amjad Ali MS N, KMU Peshawar

Presentation on nephrotic syndrome

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  1. 1. Prepared by Amjad Ali MS N, KMU Peshawar
  2. 2. CASE SCENARIO A male child patient aged 6 yrs was admitted in pediatric ward with: Present complains: Hematuria burning maturation History of present illness: Subject developed dyspnea 2 days back and on the present day morning blood in urine was found. Past family history: Not significant (NO ONE HAD HEMATURIA)
  3. 3. Investigations 1. CUE (COMPLETE URINE EXAMINATION): urine proteins: 420 (0-8 mg/dl) creatinine: 39 (30-40 mg/dl) P/C ratio : 10.70 ( 40 mg/m2/hr First morning protein : creatinine ratio of > 2-3 : 1
  4. 5. PATHOPHYSIOLOGY
  5. 6. PATHOPHYSIOLOGY 1. Edema
  6. 7. Incidence ( pediatric ) 2 7 cases per 100,000 children per year Higher in underdeveloped countries ( South east Asia ) Occurs at all ages but is most prevalent in children between the ages 1.5-6 years. It affects more boys than girls, 2:1 ratio http://www.kidney.org/site/107/pdf/NephroticSyndro me.pdf
  7. 8. Etiology Genetic Secondary Idiopathic or Primary
  8. 9. Genetic causes Finnish type Congenital Nephrotic Syndrome Focal Segmental Glomerulosclerosis Diffuse Mesangial Sclerosis Denys-Drash Syndrome Nail Patella Syndrome Alport Syndrome Charcot-Marie-tooth disease Cockayne syndrome Laurence-Moon-Beidl-Bardet Syndrome Galloway-Mowat Syndrome
  9. 10. Secondary causes Congenital Oligomeganephronia Infectious Hepatitis (B,C) , HIV-1, Malaria, Syphilis, Toxoplasmosis Inflammatory Glomerulonephritis Immunological Castleman Disease, Kimura Disease, Bee sting, Food allergens Neoplastic Lymphoma, Leukemia Traumatic ( Drug induced ) Penicillamine, Gold, NSAIDS, Pamidronate, Mercury, Lithium.
  10. 11. Idiopathic Minimal Change disease ( >80 % ) Mesangial proliferation Focal segmental Glomerulosclerosis Membranous Nephropathy Membranoproliferative glomerulonephritis
  11. 12. Clinical Features Edema Mild to start with peri orbital puffiness, lower extremities Progression to generalized edema, ascites, pleural effusion, genital edema Decreased urine output Anorexia, Irritability, Abdominal pain and diarrhea Absence of Hypertension Gross hematuria
  12. 13. NO PEDAL EDEMA BUT FACIAL PUFFYNESS PRESENT
  13. 14. DIFFERENTIALS Protein losing enteropathy Hepatic failure Heart failure Acute/Chronic Glomerulonephritis Protein Malnutrition
  14. 15. Lab Investigations Urine Examination Complete Blood Count & Blood picture Renal parameters : Spot Urine Protein : Creatinine ratio Urinary protein excretion protein selectivity ratio Liver Function Test Renal Biopsy ???
  15. 16. Continue Urinalysis - 3+ to 4+ proteinuria Renal Function Spot UPC ratio > 2.0 UPE > 40 mg/m2/hr Serum Creatinine normal or elevated Serum albumin - < 2.5 gm/dl Serum Cholesterol/ TGA levels elevated Serum Complement levels Normal or low - Nelson Textbook of Pediatrics, Vol 2, 19th Edition, page 1804
  16. 17. Additional Tests C3 and antistreptolysin Chest X ray and tuberculin test Hepatitis B surface antigen Indications for Biopsy Age below 12 months Gross or persistent microscopic hematuria Low blood C3 Hypertension Impaired renal Function Failure of steroid therapy
  17. 18. Management (Initial Episode) High protein diet Salt moderation Treatment of infections If significant edema diuretics Aldosterone antagonist ( Fursemide, spironolactone ) Corticosteroid therapy with Prednisolone or prednisone ( 2mg/kg per day for 6 weeks followed by 1.5 mg/kg single morning dose on alternate days for 6 weeks )
  18. 19. Subsequent course Relapse Infrequent Relapsers : 3 or less relapses per year Frequent Relapsers : 4 or more relapses per year Steroid therapy Steroid dependant : relapse following dose reduction or discontinuation Steroid resistant : Partial or no response to initial treatment
  19. 20. Management of Relapse Parent Education Symptomatic therapy for infections in case of low grade proteinuria Persistent proteinuria ( 3 - 4+ ) Prednisolone ( 2mg/kg/day until protein is negative for 3 days ) 1.5 mg/kg on alternate days for 4 weeks ) Ghai Essential Pediatrics,8th edition, page 479.
  20. 21. Complications Edema Infections Thrombotic complications Hypovolaemia and Acute renal Failure Steroid Toxicity
  21. 22. Nursing management Goals of nursing management Reducing edema Nutrition Administering medications Skin care Infection prevention Promoting psychosocial growth Parental teaching
  22. 23. Nursing management Assessment Age, height and weight Vital signs Past hospital admissions Immunization status Edema Urinary output Presence of infections Dietary pattern Malnourishment
  23. 24. Nursing management Monitoring of Vital signs Intake output Urinary protein Diet Weight Abdominal girth
  24. 25. Nursing management Diet High protein diet 2-3 gm/kg/day Calories- 50- 70 kcal/kg/day No Added salt Protein rich food items Egg Milk Fish Chicken Paneer Spourts Pulses & legumes
  25. 26. FLUID MANAGEMENT Strict intake output monitoring Hypovolemic symptoms persistent tachycardia Hypotension abdominal pain Capillary refill >2sec urine Na