Nephrotic Syndrome

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

Nephrotic Syndrome

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Nephrotic Syndrome