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Nephrotic Syndrome

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Dang Thanh Tuan
Dang Thanh Tuan
Health & Medicine
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Nephrotic Syndrome In Children 肾 病 综 合 征 华中科技大学同济医学院 附属同济医院儿科教研室 刘 铜 林
Idiopathic nephrotic syndrome (INS) Simple nephrosis ， nephr i tic nephrosis Minimal change nephropathy (MCN) Non-minimal change nephropathy (non-MCN) Massive (heavy ， excessive) proteinuria Hypo-proteinemia ， hypo-albuminemia Hyper-lipidemia ， hyper-cholesterolemia Pitting edema ， non- pitting edema Anasarca ， ascites ， pleural effusion Corticosteroid ， prednisone ， methylprednisolone Key words
Definition Classification Etiology Pathology Pathophysiology Clinical Manifestation Complication Laboratory Data Diagnosis Treatment Main Contents
Male patient, 4 years old, Complaint of : edema , oliguria and proteinuria for 7 days.
Nephrotic syndrome (NS) results from increased permeability of glomerular basement membrane ( GBM ) to plasma protein. It is a syndrome characterized by massive proteinuria, hypo-albuminemia, hyper-cholesterolemia ， Hypercoagulable state and pitting edema. (4-increase, 1-decrease). Definition
Nephrotic Criteria ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Nephr i tic Criteria ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],肾炎型 NS (Nephritic-type NS)
Clinical Classification of NS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Etiology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Secondary NS : DIAMOND ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Pathology
Pathology: Minimal Change Nephropathy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
MCN: normal glomerulus in LM
 
MCN: fusion of foot process of epithelial in EM
MsPGN: Mesangial proliferation and expansion IgG and C3 deposits in mesangial
2.INS 的发病机制： 肾小球毛细血管滤过屏障结构 电荷屏障与分子屏障 INS 的病因与发病机制
Pathophysiology : pathogenesis of proteinuria
Pathophysiology : pathogenesis of proteinuria ,[object Object],[object Object],[object Object]
Pathophysiology : pathogenesis of proteinuria ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Pathophysiology : pathogenesis of proteinuria ,[object Object],[object Object],[object Object],[object Object],[object Object],MCN may be associated with a primary disorder of T–cell lymphocyte function.
Pathophysiology : pathogenesis of proteinuria ,[object Object],[object Object]
Pathophysiology : pathogenesis of hypoalbuminemia ,[object Object],[object Object],[object Object]
Pathophysiology : pathogenesis of hyperlipidemia ,[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Pathophysiology : pathogenesis of Hypercoagulable state
Pathophysiology : pathogenesis of edema ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
INS 的病理生理 致病因素 高脂血症 ↓ ↑ 肾小球损伤 脂蛋白合成↑ ↓ ↑ 通透性↑— 大量蛋白尿 — 低蛋白血症 ↓ 低血容量 低血浆胶体渗透压 ↓ ADH ↑ 、 RAAS ↑ 、心钠素↓ 体液进入间质或体腔 ↓ 肾炎型 INS 时 水钠潴留 GFR ↓ 凹陷性水肿
Clinical Manifestation ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Ascites and abdomen distention Edema in scrotum and penis
Clinical Manifestation Edema in scrotum and penis
Clinical Manifestation Pitting edema and abdomen distention
Complications ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Complications ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Laboratory Data ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Laboratory Data ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Diagnosis and differential diagnosis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],The pathway of diagnosis ：
Treatment ,[object Object],[object Object],[object Object],[object Object],[object Object]
General therapy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Anticoagulation therapy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Corticosteroid—prednisone therapy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Induction phase and maintanence phase
Response to Steroid therapy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],According to response to prednisone therapy
(1) 激素敏感型 INS (steroid-responsive NS) ： 以口服泼尼松足量治疗≤ 8 周，尿蛋白转阴者 (2) 激素耐药型 INS (steroid-resistant NS) ： 以口服泼尼松足量治疗满 8 周，尿蛋白仍阳性者 (3) 激素依赖型 INS (steroid-dependent NS) ： 对泼尼松敏感，但减量或停药 1 个月内复发，且重复 2 次以上者。 (4) 复发（ relapse ） 与频复发（ frequent relapse ） ： 尿蛋白由阴性转为阳性，持续在 2 周以上者为复发。 病程中半年内复发≥ 2 次， 1 年内复发≥ 3 次者为频复发 INS 的治疗方法 根据激素疗效对 INS 分型 :
Immunosuppressive agent therapy ,[object Object],[object Object],[object Object],[object Object],Indication:
Immunosuppressive agent therapy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Pulse therapy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
1.What is nephrotic syndrome ？ 2.What are the main clinical types of INS ? 3.What is the diagnostic criteria of INS ？ 4.What are the pathological types of INS ? 5.What are the common complications of INS ？ 6.How do you treat INS (general principles) ？ 7.How do you evaluate the response of steroid therapy ？ Questions

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Nephrotic Syndrome

  • 1. Nephrotic Syndrome In Children 肾 病 综 合 征 华中科技大学同济医学院 附属同济医院儿科教研室 刘 铜 林
  • 2. Idiopathic nephrotic syndrome (INS) Simple nephrosis ， nephr i tic nephrosis Minimal change nephropathy (MCN) Non-minimal change nephropathy (non-MCN) Massive (heavy ， excessive) proteinuria Hypo-proteinemia ， hypo-albuminemia Hyper-lipidemia ， hyper-cholesterolemia Pitting edema ， non- pitting edema Anasarca ， ascites ， pleural effusion Corticosteroid ， prednisone ， methylprednisolone Key words
  • 3. Definition Classification Etiology Pathology Pathophysiology Clinical Manifestation Complication Laboratory Data Diagnosis Treatment Main Contents
  • 4. Male patient, 4 years old, Complaint of : edema , oliguria and proteinuria for 7 days.
  • 5. Nephrotic syndrome (NS) results from increased permeability of glomerular basement membrane ( GBM ) to plasma protein. It is a syndrome characterized by massive proteinuria, hypo-albuminemia, hyper-cholesterolemia ， Hypercoagulable state and pitting edema. (4-increase, 1-decrease). Definition
  • 6.
  • 7.
  • 8.
  • 9.
  • 10.
  • 11.
  • 12.
  • 13.
  • 14. MCN: normal glomerulus in LM
  • 15.  
  • 16. MCN: fusion of foot process of epithelial in EM
  • 17. MsPGN: Mesangial proliferation and expansion IgG and C3 deposits in mesangial
  • 18. 2.INS 的发病机制： 肾小球毛细血管滤过屏障结构 电荷屏障与分子屏障 INS 的病因与发病机制
  • 19. Pathophysiology : pathogenesis of proteinuria
  • 20.
  • 21.
  • 22.
  • 23.
  • 24.
  • 25.
  • 26.
  • 27.
  • 28. INS 的病理生理 致病因素 高脂血症 ↓ ↑ 肾小球损伤 脂蛋白合成↑ ↓ ↑ 通透性↑— 大量蛋白尿 — 低蛋白血症 ↓ 低血容量 低血浆胶体渗透压 ↓ ADH ↑ 、 RAAS ↑ 、心钠素↓ 体液进入间质或体腔 ↓ 肾炎型 INS 时 水钠潴留 GFR ↓ 凹陷性水肿
  • 29.
  • 30. Ascites and abdomen distention Edema in scrotum and penis
  • 31. Clinical Manifestation Edema in scrotum and penis
  • 32. Clinical Manifestation Pitting edema and abdomen distention
  • 33.
  • 34.
  • 35.
  • 36.
  • 37.
  • 38.
  • 39.
  • 40.
  • 41.
  • 42.
  • 43. (1) 激素敏感型 INS (steroid-responsive NS) ： 以口服泼尼松足量治疗≤ 8 周，尿蛋白转阴者 (2) 激素耐药型 INS (steroid-resistant NS) ： 以口服泼尼松足量治疗满 8 周，尿蛋白仍阳性者 (3) 激素依赖型 INS (steroid-dependent NS) ： 对泼尼松敏感，但减量或停药 1 个月内复发，且重复 2 次以上者。 (4) 复发（ relapse ） 与频复发（ frequent relapse ） ： 尿蛋白由阴性转为阳性，持续在 2 周以上者为复发。 病程中半年内复发≥ 2 次， 1 年内复发≥ 3 次者为频复发 INS 的治疗方法 根据激素疗效对 INS 分型 :
  • 44.
  • 45.
  • 46.
  • 47. 1.What is nephrotic syndrome ？ 2.What are the main clinical types of INS ? 3.What is the diagnostic criteria of INS ？ 4.What are the pathological types of INS ? 5.What are the common complications of INS ？ 6.How do you treat INS (general principles) ？ 7.How do you evaluate the response of steroid therapy ？ Questions