Nephrotic syndrome 1


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  • Nephrotic syndrome 1

    1. 1. Nephrotic Syndrome..…(NS) Prepared by:NAZURAH
    2. 2. Nephrotic syndrome  Proteinuria Proteinuria >40mg/m2/hour or an early morning ur pr creatinine index >200mg/mmol Edema Hypoalbuminemia  Hypercholesterolemia
    3. 3. Classification: A-Primary Idiopathic NS (INS): majority Accounting for 90% of NS in child. mainly discussed. Unknown cause B-Secondary NS: Include post streptococcal glomerulonephritis and SLE
    4. 4. 1.The construction of the glomerular basement membrane has changed. 2.The loss of the negative charges on the GBM.
    5. 5. Pathophysiology: The Main Trigger Of primary Nephrotic Syndrome and Fundamental and highly important change of pathophysiology :Proteinuria
    6. 6. Pathogenesis of Proteinuria: Increase glomerular permeability for proteins due to loss of negative charged glycoprotein  Degree of protineuria: Mild less than 0.5g/m2/day  Moderate 0.5 – 2g/m2/day  Sever more than 2g/m2/day  Type of proteinuria: A-Selective proteinuria: where proteins of low molecular weight .such as albumin, are excreted more readily than protein of HMW  B-Non selective :  LMW+HMW are lost in urine
    7. 7. How many pathological types causes nephrotic syndrome?
    8. 8. Investigations: 1-Urine analysis:- A-Proteinuria : 3-4 + SELECTIVE. b-24 urine collection for protein >40mg/m2/hr for children c- volume: oliguria (during stage of edema formation) d-Microscopically:microscopic hematuria 20%, large number of hyaline cast
    9. 9. Investigations: 2-Blood:  A-serum protein: decrease >5.5gm/dL , Albumin levels are low ( < 2.5gm/dL).  B-Serum cholesterol and triglycerides: Cholesterol > 5.7mmol/L (220mg/dl).  C-- ESR↑ > 100mm/hr during activity phase .  3.Serum complemen: Vary with clinical type.  4.Renal function
    10. 10. Kidney Biopsy:-  Considered in:  1-Secondary N.S  2-Steroid resistant N.S  3- Gross Hematuria  4-Hypertension  5- Renal Impairment
    11. 11. Complications of NS:1-Infections:Infections is a major complication in children with NS. It frequently trigger relapses. Nephrotic pt are liable to infection because : A-loss of immunoglobins in urine. B-the edema fluid act as a culture medium. C-use immunosuppressive agents. D- malnutrition The common infection : URI, peritonitis, cellulitis and UTI may be seen. Organisms: encapsulated (Pneumococci, H.influenzae), Gram negative (e.g E.coli
    12. 12. Complication…..  2-Hypercoagulability (Thrombosis).  Hypercoagulability of the blood leading to venous or arterial thrombosis:  Hypercoagulability in Nephrotic syndrome caused by:   1-Higher concentration of I,II, V,VII,VIII,X and fibrinogen 2- Lower level of anticoagulant substance: antithrombin III  3-decrease fibrinolysis.  4-Higher blood viscosity   5- Increased platelet aggregation 6- Overaggressive diuresis
    13. 13.  3-ARF: pre-renal and renal  4- cardiovascular disease :-Hyperlipidemia, may be a risk factor for cardiovascular disease.  5-Hypovolemic shock  6-Others: growth retardation, malnutrition, adrenal cortical insufficiency 
    14. 14. Management of NS: General (non-specific ) Corticosteroid therapy
    15. 15. General therapy:Normal diet with adequate calories No added salt to the diet whn child has edema Avoiding infection: very important. Penicillin V is recommended at diagnosis and during relapses  Severe edema: Restricting fluid intake Human albumin (20-25%)- symptomatic grossly edematous together with IV frusemide(diurresis)
    16. 16. GENERAL ADVICE Home urine albumin monitoring (1st urine specimen) Consult doctors if 1)albuminuria >= 2+ for consecutives day or out 7 days. 2)edematous Immunisation on corticosteroid treatment and within 6 weeks (killed vacines) after 6 weeks cessation (live vaccine) pneumococcal vaccine
    17. 17. Corticosteroid—prednisone therapy:- REMISSION : Urine dipstick trace or nil for 3 consecutives days within 28 days. RELAPSE: Urine albumin excretion > 40mg /m2/hour or urine dipstick >= 2+ for 3 consecutives days FREQUENT RELAPSES : >= 2 Relapses within 6 month of initial diagnosis or >= 4 relapses within 12 month periods STEROID DEPENDENT NEPHROTIC SYNDROME : >= 2 Consecutives relapses occuring during steroid taper or within 14days of cessation of steroid
    18. 18. Side Effects With Long Term Use of Steroids “Steroid toxicity -Stunted growth Cataracts - Pseudotumor cerebri  hyperglycemia  myopathy  peptic ulcer  poor healing of wound. -Psycosis  Hirsutism -Osteoporosis  Thromboembolism - Cushingoid features -Adrenal gland suppression
    19. 19. Alternative agent: When can be used:  Steroid-dependent patients, frequent relapsers, and steroid- resistant patients.  Cyclophosphamide Pulse steroids  Cyclosporin A  Tacrolimus  Microphenolate
    20. 20. Treatment Cytotoxic drugs with corticosteroid: (for steroid dependent or steroid resistant) Cyclophosphamide (CTX): p.o. or intravenously Side effects: liver injury, inhibition of bone marrow, etc. Cyclosporine (for those failed responsing to combination of steroid and cytotoxic drugs) Dose: 5mg/kg/d, bid, p.o. Side effects: renal and liver toxic injury, expensive, etc.
    21. 21. Treatment Mycophenolate mofetil, MMF (for steroid dependent or steroid resistant) Dose:1.5-2g/d, bid, p.o. for 3-6 months, maintaining 0.5 year
    22. 22. THE END…. THANK YOU….