Nephrotic Syndrome. Department of pediatrics The first affiliated hospital Sun Yat Sen University. Yue Zhihui ( 岳智慧 )

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1 Nephrotic Syndrome Department of pediatrics The first affiliated hospital Sun Yat Sen University Yue Zhihui ( 岳智慧 ) yuezhihui810@yahoo.com.cn

2 Contents Definition Pathophysiology Clinical manifestation & laboratory test Complication Treatment

3 Definition Glomerular permeability Clinical features Massive proteinuria Hypoproteinemia Hyperlipidemia Edema

4 Classification Primary/Idiopathic (90%) Secondary: SLE, HBV, anaphylactoid purpura Congenital

5 Primary Nephrotic Syndrome Etiology Pathogenesis

6 Glomerular filtration barrier Size-selective (aperture) barrier Charge-selective barrier

7 Glomerular filtration barrier

8 aperture barrier slit diaphragm Endothelium

9 Charge-selective barrier Endothelium

10 电荷屏障 Endothelium

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14 Nonselective proteinuria

15 Pathogenetic factor Pathophysiology glomerular permeability hyperlipidemia massive proteinuria Hypoproteinemia Plasma oncotic pressure Fluid Lipoprotein lipase Lipoproteins synthesis Intravascular volume RAA(aldosterone) ADH Interstitial space Edema Water&sodium retention

16 Pathological changes in INS minimal change disease (MCD) mesangial proliferative glomerulonephritis (MsPGN) focal segmental glomerulosclerosis (FSGS) membranous nephrosis (MN) membranoproliferative glomerulonephritis (MPGN)

17 Pathological patterns of INS in children and adults Children Adults 9% 10% 4% 8% 3% 66% 33% 2% 33% 22% 2% 8% MCNS MsPGN FSGS MN MPGN Others

18 MCD

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21 Focal segmental glomerulosclerosis (FSGS)

22 Clinical manifestation Epidemiology Incidence, sex and age Main symptoms and signs Genaral situation Edema ascites pleural effusion Urine, hematuria Blood pressure Renal function

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29 Clinical types Simple type and Nephritic type hematuria urinary RBC 10/HPF hypertension: preschool age child 120/80mmHg school age child 130/90mmHg renal function insufficient hypocomplementemia

30 Complications Infection Electrolyte disorder, Hypovolemia Hypercoagulability and thrombosis Acute renal failure Renal tubular function disorder

31 Infection Manifestations URI, spontaneous peritonitis, tuberculosis, cellulitis, urinary tract infection Cause Immunoglobulin and complement factor protein malnutrition, edema, immunosuppressive therapy Management and Prophylaxis high index of suspicion, prompt evaluation early initiation of therapy polyvalent neumococcal vaccine

32 Electrolytes disorder and hypovolemia Manifestations Hyponatremia, hypokalemia, Hypocalcemia Hypovolemic shock Cause salt intake restriction diuretic treatment vomit, diarrhea, intestinal reabsorbtion Loss of calcium binding protein Prophylaxis Avoiding aggressive diuretic therapy Inappropriate salt intake restriction

33 Hypercoagulability and thrombosis Manifestations thrombosis within kidney, extremities, brain and lung Cause coagulation factorsⅡ, Ⅴ, Ⅶ, Ⅷ, Ⅹ, platelet aggregation, antithrombin Ⅲ Hyperlipidemia, diuretic and steroid therapy prophylaxis Avoiding puncture of deep veins Prophylactic anticoagulation drugs

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36 Acute renal failure Manifestations Oliguria or anuria,hypertension Elevated serum Cr and BUN levels Cause Intravascular blood volume Obstruction, crescent formation Acute interstitial nephritis, drugs Prophylaxis Avoiding use of renal toxic drugs Avoiding aggressive diuretic therapy

37 Renal tubular function disorder Manifestations polyuria, nocturia, Glucosuria, aminoaciduria, Fanconi syndrome Cause Progress of the glomerular disease Persistent massive proteinuria prophylaxis avoiding excessive albumin transfusion

38 Laboratory tests Urine Urinalysis, 24h urinary protein excretion, urinary Pro/Cr Serum albumin, cholesterol, triglyceride IgG, IgA, IgM, C3 BUN, Cr sodium, potassium, calcium Ultrasonography renal biopsy

39 Diagnosis sequence Nephrotic syndrome? Primary, secondary or congenital? Simple type or nephritic type?

40 Treatment General treatment Rest Diet sodium, protein, calcium and vitamin D Prevention and cure of infection Diuresis Education of the family

41 Anticoagulant therapy Heparin, Persantine Steroid Prednisone, prednisolone Immunosupressive drugs CTX, CsA, FK506 Others ACEI, Immunologic regulators, Chinese medicine

42 Corticosteroid therapy Scheme Short range Intermediate range Long range

43 Prednisone mg/kg/d*6-8w Prednisone 2mg/kg qod*4w Prednisone dose (every 2-4w) Course of treatment 6m 9m Intermediate long

44 classification on curative effects steroid responsive steroid resistant / insensitive steroid dependent frequent relapse

45 Indications for cytotoxic drugs frequent relapse steroid dependent steroid resistant unable to tolerate steroid treatment

46 Side effects of corticosteroid Metabolic disturbance Hypertension Infection, peptic ulcer Euphoria, lunacy, induce epilepsy, insomnia Osteoporosis, growth retardation Cataract abuse syndrome and adrenal insufficiency

47 Side effects of CTX Leukopenia Alopecia Hepatic function disorder Hemorrhagic cystitis Sterility Disseminated varicella infection Inappropriate ADH secretion

48 Prognosis Relapse and resolve Prognosis is depend on pathologic patterns

49 Case presentation A 5 years old male child was admitted to our hospital for progressed edema and foamy urine for 1 week. 3 days before edema appearance, he suffered from fever, cough and running nose. On examination patient had generalized anasarca. Vitals were normal. Systemic examination revealed ascites and pleural effusion. Biochemical investigations showed serum cholesterol8.7mmol/l, total serum proteins 43 g/l, Albumin 16 g/l, Globulins 27 g/l. Urine proteinwas +++, RBC +++,electrolytes and urea were normal. Diagnosis? Differential diagnosis? Treatment?

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