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Nephrotic syndrome (NS) 肾病综合征

Nephrotic syndrome (NS) 肾病综合征. Department of Pediatrics Soochow University Affiliated Children’s Hospital. Concept. Heavy proteinuria Hypoproteinaemia Oedema Hyperlipidaemia. Physiology. Heavy proteinuria---reasons. Injured glomerular filtration barrier. 足 突 细 胞. 足 突 细 胞 的 超 微

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Nephrotic syndrome (NS) 肾病综合征

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  1. Nephrotic syndrome (NS)肾病综合征 Department of Pediatrics Soochow University Affiliated Children’s Hospital

  2. Concept • Heavy proteinuria • Hypoproteinaemia • Oedema • Hyperlipidaemia

  3. Physiology

  4. Heavy proteinuria---reasons Injured glomerular filtration barrier

  5. 足 突 细 胞

  6. 足 突 细 胞 的 超 微 机 构

  7. 肾小球滤过膜通透性与分之大小的关系

  8. Heavy proteinuria--results ? Prolonged glomerular leakage of protein leads to hypoalbuminaemia ,that is associated with loss of fluid into the extracellular space, Reduction in circulating volume stimulates renal retention of salt and water, manifested as oedema.

  9. Hypoalbuminaemia-reasons? • Urinary losses of albumin are the major factor in the development of hypoalbuminaemia  • possible role of catabolism of reabsorbed albumin in the tubule

  10. Hyperlipidemia: • Due to Increased Hepatic synthesis • Decreased Large molecular Lipidoprotein depletion • high LDL, TG, cholesterol and low HDL level cause cardiovascular diseases • At same time it also lead to glomerular sclerosis and tubulointerstitial fibrosis

  11. Oedema—reasons? • Reduced oncotic pressure • Low blood volume • Increased Na and water absorption • Increased RAS activity • Decreased GFR

  12. GFR

  13. Others physiologic changes • Reduced immunity : low IgG • High coagulative status : thrombi • Lost of Microelements : Ca, Zn, Fe, Copper • Vitamin D deficiency • Hormone: T3, T4

  14. Pathology • Idiopathic NS 76.4% ( Nil disease, Lipoid Nephrosis, Minimal change disease, MCD ) • Focal Segmental Glomerular sclerosis (FSGS) 6.9% • Mesangial Capillary proliferative GN ( Membranous Proliferative GN, MPGN)7.5% • Mesangial GN (MsPGN)2.3% • Membranous GN (MN) 1.5%

  15. Minimal change NS

  16. Normal Minimal change NS

  17. FSGS

  18. MsPGN

  19. MN

  20. MN

  21. MpGN

  22. Diagnosis (1) • Clinical :congenital , primary or secondary simple or nephritis NS • Pathologic: minimal changes and non minimal changes • Therapeutic : steroid sensitive , steroid resistance , steroid dependence and frequency relapse NS

  23. Diagnosis(2) • Congenital NS • Primary NS 90% • Secondary NS 10%: HSP; SLE, IgAN, vasculitis, Wilson’s disease, tumor disease,

  24. Diagnosis (3) Simple NS : Nephritis NS : 3 highs and 1 low 1 high and 1 low with any one item below Haematuria : no >10/HP within 2 weeks Hypertension: no Yes BUN & SCr: normal Persistence Increased : C3 : normal Low

  25. Treatment general therapy 1. Have a rest : don’t need enforced bed rest other than severe oedema and hypertension 2. Infections: prevention, prophylaxis therapy; vigor treatment; 3. vaccine? 4.Diuretics: combined potassium keeper

  26. Diet: 1.low salt and water diet need only in severe oedema and hypertension, medium protein input (1.5-2g/Kg) is suggested 2. high protein feeding merely led to increased spilling of albumin into the urine without an increase in plasma albumin concentrations (Kaysen et al. 1989). 3. low protein diets may lead to negative protein balance in the long term

  27. Treatment (2) Specific treatment Steroid : prednisolone: first selection dexmethasone methylprednisone Principle: dose must be up to 2 mg/Kg.d maximum, 60mg/d; taper slowly; medium-long term maintenance

  28. Steroid---term • Short term therapy : 8 weeks: • Medium term therapy: 6 months • Long term therapy: >9 months

  29. Therapeutic judgment • Steroid Sensitive NS (SSNS) • Steroid Partial Sensitive NS • Steroid Resistant NS (SRNS) • Steroid Dependent NS (SDNS) • Frequent Relapse NS

  30. Side effects Short terms: predisposing infections electrolyte unbalance Lon terms : cohn’s syndrome bone malnutrition diabetic cardiovascular: eyes problem:cataract psychological disease:

  31. Cytotoxic drugs Indications : steroid resistant NS steroid dependent NS frequently relapse NS severe pathologic change NS: FSGS

  32. Cyclophosphamide • Mechanism: DNA mitosis level • Side effects: gonad damage • Administration: Oral , Intravenous pulse

  33. others • Chlorambucil : mitosis level • Azathiopurine : synthesis level • Cyclosporine or FK 506 : activation T cells level • Mycophenolic acid : synthesis level • VCR: synthesis level

  34. Anti-coagulations Anti—coagulation factor :heparin Anti—platelet accumulation : dipyridamole Fibrin split drugs : urokinase

  35. Immune regulation drugs • Levamisole • Intravenous Immunoglobulin • Chinese herbs

  36. Prognostic outcome • Simple nephrotic syndrome: good: minimal change nephrotic syndrome infections and malnutrition • Nephritis nephrotic syndrome: poor: nonminimal change nephrotic syndrome, renal malfunction , hypertension, low complement

  37. Homework • The management of urinary tract infections • The investigations of a child with proteinuria

  38. Thanks

  39. You should have an understanding of : • The investigation of a child with haematuria • The basic symptoms and signs suggestive of renal failure, and have a working overview of its management.

  40. Haematuria

  41. Concept • Urine 10 ml 1500rpm spin for 10 minutes . 0.2ml precipitation under microscope • Pathological: More than 3 red cells per high field under microscope • As a result of disease : kidneys and lower urinary tract, as well as coagulopathies, drugs and exercise

  42. Glomerular causes of haematuria • Glomerularonephritis • Recurrent haematuria : IgA nephropathy • HUS: • HSP: • SLE:

  43. Non-glomerular • Polycystic diseases • Congenital anomalies • Infection • Trauma • Tumors • Calculi : hypercalciuria • Nut cracker syndrome

  44. Non renal causes • Haematological : Coagulopathies; thrombocytopenia; renal vein thrombosis; sickle cell diseases • Non-haematological: exercise; Drugs

  45. Glomerulonephritis(GN): Poststreptococcal GN • Is the most common form of Glomerulonephritis and caused by certain strains of streptococci (group A -hemolytic streptococcal infection • It affect young children greater than 3 yrs of age • The incidence is declining due to reduced infection rate and prophylactic antibiotics • Sex: More boys than girls ,about ratio of 2: 1

  46. Pathology(1)

  47. Pathology(2) • The glomerular tuft shows an increased number of cells and appears to fill up the Bowman’s space: mesangial , endothelial, and epithelial cells proliferate ( crescent GN) • There is infiltration of cells from the circulation: Polymorphonuclear leukocyte; Monocytes and T lymphocytes • Immune deposits in the glomerular basement membrane and mesangium can be seen by immunofluorescence microscopy. • Electron-dense subepithelial deposits (humps) are typical of acute poststreptococcal glomerulonephritis

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