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<ul><li>1.Department of pediatrics The first affiliated hospital Sun Yat Sen University Sun Liangzhong( ) [email_address] Nephrotic Syndrome</li></ul> <p>2. Definition </p> <ul><li>Glomerular permeability </li></ul> <ul><li>Clinical features </li></ul> <ul><li><ul><li>Massive proteinuria </li></ul></li></ul> <ul><li><ul><li>Hypoproteinemia </li></ul></li></ul> <ul><li><ul><li>Hyperlipidemia </li></ul></li></ul> <ul><li><ul><li>Edema </li></ul></li></ul> <p>3. Classification </p> <ul><li>Primary/Idiopathic (90%) </li></ul> <ul><li>Secondary:</li></ul> <ul><li><ul><li>SLE, HBV, anaphylactoid purpura </li></ul></li></ul> <ul><li>Congenital/hereditary </li></ul> <p>4. IdiopathicNephrotic Syndrome </p> <ul><li>Etiology and Pathogenesis </li></ul> <ul><li><ul><li>Immunologic mechanisms </li></ul></li></ul> <ul><li><ul><li>T-lymphocyte Abnormalities </li></ul></li></ul> <ul><li><ul><li>Glomerular permeability factor </li></ul></li></ul> <p>5. a Basement membrane b Epithelial cell c Endothelial cell d Mesangial cellGlomerular filtration barrier 6. Glomerularfiltration barrier a Basement membrane b Epithelial cell c Endothelial cell d Mesangial cell 7. Glomerular filtration barrier </p> <ul><li><ul><li>Size-selective (aperture) barrier </li></ul></li></ul> <ul><li><ul><li>Charge-selective barrier </li></ul></li></ul> <p>8. aperture barrier Endothelium slit diaphragm 9. 10. Charge-selective barrier Endothelium 11. 12. 13. Nonselective proteinuria 14. </p> <ul><li>minimal change disease (MCD)</li></ul> <ul><li>mesangial proliferative glomerulonephritis (MsPGN)</li></ul> <ul><li>focal segmental glomerulosclerosis (FSGS)</li></ul> <ul><li>membranous nephrosis (MN) </li></ul> <ul><li>membranoproliferative glomerulonephritis (MPGN) </li></ul> <p>Pathological changes in INS 15. 16. 17. 18. Mild Moderate Mesangial proliferative glomerulonephritis 19. Focal segmental glomerulosclerosis (FSGS) 20. membranous nephrosis (MN) 21. Membranoprliferative glomerulonephritis 22. Pathological patterns of INS in children and adults 23. Pathophysiology Pathogenetic factor glomerular permeability massive proteinuria Hypoproteinemia lipoproteins synthesis hyperlipidemia Plasma oncotic pressure Intravascular volume RAA(aldosterone) ADH Water sodium retention Edema Fluid Interstitial space Lipoprotein lipase 24. Clinical manifestation </p> <ul><li>Epidemiology </li></ul> <ul><li><ul><li>Incidence, sex and age </li></ul></li></ul> <ul><li>Main symptoms and signs </li></ul> <ul><li>Edema ascites pleural effusion </li></ul> <ul><li><ul><li>Urine, hematuria </li></ul></li></ul> <ul><li><ul><li>Blood pressure </li></ul></li></ul> <ul><li><ul><li>Renal function</li></ul></li></ul> <ul><li><ul><li>Genaral situation </li></ul></li></ul> <p>25. 26. 27. 28. 29. </p> <ul><li>Urine </li></ul> <ul><li><ul><li>Urinalysis, 24h urinary protein excretion, urinary Pro/Cr </li></ul></li></ul> <ul><li>Serum </li></ul> <ul><li><ul><li>albumin, cholesterol, triglyceride </li></ul></li></ul> <ul><li><ul><li>IgG, IgA, IgM, C3 </li></ul></li></ul> <ul><li><ul><li>BUN, Cr</li></ul></li></ul> <ul><li><ul><li>sodium, potassium, calcium </li></ul></li></ul> <ul><li>Ultrasonography </li></ul> <ul><li>renal biopsy </li></ul> <p>Laboratory tests 30. Diagnosis and classifications </p> <ul><li><ul><li>24h urinary protein excretion 50/40mg/kg/d </li></ul></li></ul> <ul><li><ul><li>Serum albumin 25g/L </li></ul></li></ul> <ul><li><ul><li>Serum cholesterol 5.72mmol/L </li></ul></li></ul> <ul><li><ul><li>Edema </li></ul></li></ul> <p>31. </p> <ul><li>Hematuria</li></ul> <ul><li><ul><li>Urinary RBC10/HPF </li></ul></li></ul> <ul><li>Hypertension:</li></ul> <ul><li><ul><li>Preschool age child120/80mmHg </li></ul></li></ul> <ul><li><ul><li>School age child130/90mmHg </li></ul></li></ul> <ul><li>Renal function insufficient </li></ul> <ul><li>Hypocomplementemia </li></ul> <p>Simple type and Nephritic typeClinical types 32. Differential diagnosis </p> <ul><li>What are the related diseases? </li></ul> <ul><li>Edema caused by renal diseases </li></ul> <ul><li>Nephrotic syndrome </li></ul> <ul><li>Primary, secondary or congenital </li></ul> <ul><li>Simple type or nephritic type </li></ul> <p>33. Treatment General treatment </p> <ul><li>Rest </li></ul> <ul><li>Diet </li></ul> <ul><li><ul><li>Sodium and water </li></ul></li></ul> <ul><li><ul><li>Protein </li></ul></li></ul> <ul><li><ul><li>calcium and vitamin D </li></ul></li></ul> <ul><li>Diuresis </li></ul> <ul><li>Education of the family </li></ul> <p>34. </p> <ul><li>Steroid </li></ul> <ul><li><ul><li>Prednisone, methyl-prednisolone </li></ul></li></ul> <p>Treatment 35. </p> <ul><li>Corticosteroid therapy </li></ul> <ul><li>Scheme </li></ul> <ul><li><ul><li>Short course </li></ul></li></ul> <ul><li><ul><li>Mediate course </li></ul></li></ul> <ul><li><ul><li>Long course </li></ul></li></ul> <p>36. </p> <ul><li>Prednisone 1.5-2 mg/kg/d*6-8w</li></ul> <ul><li>Prednisone 2mg/kg qod*4w </li></ul> <ul><li>Prednisone dose (every 2-4w) </li></ul> <ul><li>Course of treatment </li></ul> <ul><li>6m9m </li></ul> <ul><li>Intermediatelong </li></ul> <p>37. </p> <ul><li>steroid responsive/sensitive </li></ul> <ul><li>steroid resistant / insensitive </li></ul> <ul><li>steroid dependent </li></ul> <ul><li>frequent relapse </li></ul> <p>classification on curative effects 38. </p> <ul><li><ul><li>Metabolic disturbance </li></ul></li></ul> <ul><li><ul><li>Hypertension </li></ul></li></ul> <ul><li><ul><li>Infection, peptic ulcer </li></ul></li></ul> <ul><li><ul><li>Euphoria, lunacy, induce epilepsy, insomnia </li></ul></li></ul> <ul><li><ul><li>Osteoporosis, growth retardation </li></ul></li></ul> <ul><li><ul><li>Cataract </li></ul></li></ul> <ul><li><ul><li>abuse syndrome and adrenal insufficiency </li></ul></li></ul> <p>Side effects of corticosteroid 39. </p> <ul><li><ul><li>frequent relapse </li></ul></li></ul> <ul><li><ul><li>steroid dependent </li></ul></li></ul> <ul><li><ul><li>steroid resistant </li></ul></li></ul> <ul><li><ul><li>unable to tolerate steroid treatment </li></ul></li></ul> <p>Indications for Alternative agents 40. Alternative agents for INS </p> <ul><li>Cyclophosphamide (CTX) </li></ul> <ul><li>Cyclosporine (CsA) and tacrolimus (FK506)</li></ul> <ul><li>Mycophenolate, MMF . </li></ul> <ul><li>Angiotensin-converting enzyme inhibitors (ACEI) and angiotensin II blockers </li></ul> <p>41. Cyclophosphamide (CTX) </p> <ul><li>Cyclophosphamide prolongs the duration of remission and reduces the number of relapses in children withfrequently relapsingandsteroid-dependent nephrotic syndrome .</li></ul> <p>42. </p> <ul><li><ul><li>Leukopenia </li></ul></li></ul> <ul><li><ul><li>Alopecia </li></ul></li></ul> <ul><li><ul><li>Hepatic function disorder </li></ul></li></ul> <ul><li><ul><li>Hemorrhagiccystitis </li></ul></li></ul> <ul><li><ul><li>Sterility </li></ul></li></ul> <ul><li><ul><li>Disseminated varicella infection </li></ul></li></ul> <ul><li><ul><li>Inappropriate ADH secretion </li></ul></li></ul> <p>Side effects ofCTX 43. Cyclosporine and tacrolimus </p> <ul><li>Cyclosporine (36 mg/kg/24 hr divided q 12 hr)</li></ul> <ul><li>Tacrolimus(0.15 mg/kg/24 hr divided q 12 hr)</li></ul> <ul><li>Both are also effective in maintaining prolonged remissions in children with nephrotic syndrome and are useful as steroid-sparing agents.</li></ul> <p>44. Side effects ofCSA and FK506 </p> <ul><li>Hypertension </li></ul> <ul><li>Nephrotoxicity </li></ul> <ul><li>Hirsutism </li></ul> <ul><li>gingival hyperplasia</li></ul> <p>45. Mycophenolate and ACEI </p> <ul><li>Mycophenolatemay maintain remission in children with steroid-dependent or frequently relapsing nephrotic syndrome.</li></ul> <ul><li>ACEI and angiotensin II blockersmay be helpful as adjunct therapy to reduce proteinuria in steroid-resistant patients.</li></ul> <p>46. Others </p> <ul><li>Anticoagulant therapy </li></ul> <ul><li><ul><li>Heparin, Persantine </li></ul></li></ul> <ul><li>Immunologic regulators </li></ul> <ul><li>Chinese medicine </li></ul> <p>47. Complications </p> <ul><li>Infection </li></ul> <ul><li>Electrolyte disorder, Hypovolemia </li></ul> <ul><li>Hypercoagulability and thrombosis </li></ul> <ul><li>Acute renal failure </li></ul> <ul><li>Renal tubular function disorder </li></ul> <p>48. </p> <ul><li>Infection </li></ul> <ul><li>Manifestations </li></ul> <ul><li><ul><li>URI,spontaneous peritonitis , tuberculosis, cellulitis, urinary tract infection </li></ul></li></ul> <ul><li>Cause </li></ul> <ul><li><ul><li>Immunoglobulin and complement factor </li></ul></li></ul> <ul><li><ul><li>protein malnutrition,edema, </li></ul></li></ul> <ul><li><ul><li>immunosuppressive therapy </li></ul></li></ul> <ul><li>Management and Prophylaxis </li></ul> <ul><li><ul><li>high index of suspicion, prompt evaluation </li></ul></li></ul> <ul><li><ul><li>early initiation of therapy </li></ul></li></ul> <ul><li><ul><li>polyvalent neumococcal vaccine </li></ul></li></ul> <p>49. </p> <ul><li>Manifestations </li></ul> <ul><li><ul><li>Hyponatremia, hypokalemia, Hypocalcemia </li></ul></li></ul> <ul><li><ul><li>Hypovolemic shock </li></ul></li></ul> <ul><li>Cause </li></ul> <ul><li><ul><li>salt intake restriction </li></ul></li></ul> <ul><li><ul><li>diuretic treatment </li></ul></li></ul> <ul><li><ul><li>vomit, diarrhea, intestinalreabsorbtion </li></ul></li></ul> <ul><li><ul><li>Loss of calcium binding protein</li></ul></li></ul> <ul><li>Prophylaxis</li></ul> <ul><li><ul><li>Avoiding aggressive diuretic therapy </li></ul></li></ul> <ul><li><ul><li>Inappropriate salt intake restriction </li></ul></li></ul> <p>E lectrolytes disorder and hypovolemia 50. </p> <ul><li>Hypercoagulability and thrombosis </li></ul> <ul><li>Manifestations </li></ul> <ul><li><ul><li>thrombosis within kidney, extremities, brain andlung </li></ul></li></ul> <ul><li>Cause </li></ul> <ul><li><ul><li>coagulation factors, , , , , platelet aggregation, antithrombin </li></ul></li></ul> <ul><li><ul><li>Hyperlipidemia, diuretic and steroid therapy </li></ul></li></ul> <ul><li>prophylaxis </li></ul> <ul><li><ul><li>Avoiding puncture of deep veins </li></ul></li></ul> <ul><li><ul><li>Prophylactic anticoagulation drugs </li></ul></li></ul> <p>51. 52. 53. </p> <ul><li>Manifestations </li></ul> <ul><li><ul><li>Oliguria or anuria hypertension </li></ul></li></ul> <ul><li><ul><li>Elevated serum Cr and BUN levels </li></ul></li></ul> <ul><li>Cause </li></ul> <ul><li><ul><li>Intravascular blood volume </li></ul></li></ul> <ul><li><ul><li>Obstruction, crescent formation </li></ul></li></ul> <ul><li><ul><li>Acute interstitial nephritis, drugs </li></ul></li></ul> <ul><li>Prophylaxis </li></ul> <ul><li><ul><li>Avoiding use of renal toxic drugs </li></ul></li></ul> <ul><li><ul><li>Avoiding aggressive diuretic therapy </li></ul></li></ul> <p>Acute renal failure 54. </p> <ul><li>Renal tubular function disorder </li></ul> <ul><li>Manifestations </li></ul> <ul><li><ul><li>polyuria, nocturia, Glucosuria, </li></ul></li></ul> <ul><li><ul><li>aminoaciduria, Fanconi syndrome </li></ul></li></ul> <ul><li>Cause </li></ul> <ul><li><ul><li>Progress of the glomerular disease </li></ul></li></ul> <ul><li><ul><li>Persistent massive proteinuria </li></ul></li></ul> <ul><li>prophylaxis </li></ul> <ul><li><ul><li>avoiding excessive albumin transfusion </li></ul></li></ul> <p>55. Prognosis </p> <ul><li>Relapse and resolve </li></ul> <ul><li>Prognosis is depend on pathologic patterns </li></ul> <p>56. Thank You </p>

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