Presentation on nephrotic syndrome
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- 1. Prepared by Amjad Ali MS N, KMU Peshawar
- 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. 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
- 5. PATHOPHYSIOLOGY
- 6. PATHOPHYSIOLOGY 1. Edema
- 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
- 8. Etiology Genetic Secondary Idiopathic or Primary
- 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
- 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.
- 11. Idiopathic Minimal Change disease ( >80 % ) Mesangial
proliferation Focal segmental Glomerulosclerosis Membranous
Nephropathy Membranoproliferative glomerulonephritis
- 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
- 13. NO PEDAL EDEMA BUT FACIAL PUFFYNESS PRESENT
- 14. DIFFERENTIALS Protein losing enteropathy Hepatic failure
Heart failure Acute/Chronic Glomerulonephritis Protein
Malnutrition
- 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 ???
- 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
- 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
- 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 )
- 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
- 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.
- 21. Complications Edema Infections Thrombotic complications
Hypovolaemia and Acute renal Failure Steroid Toxicity
- 22. Nursing management Goals of nursing management Reducing
edema Nutrition Administering medications Skin care Infection
prevention Promoting psychosocial growth Parental teaching
- 23. Nursing management Assessment Age, height and weight Vital
signs Past hospital admissions Immunization status Edema Urinary
output Presence of infections Dietary pattern Malnourishment
- 24. Nursing management Monitoring of Vital signs Intake output
Urinary protein Diet Weight Abdominal girth
- 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
- 26. FLUID MANAGEMENT Strict intake output monitoring
Hypovolemic symptoms persistent tachycardia Hypotension abdominal
pain Capillary refill >2sec urine Na