儿科泌尿系统NS2英文教学课件

1、 Nephrotic Syndrome(NS) 第1页，共70页。 Definition NS is an accumulation of symptoms and signs and is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia. 第2页，共70页。 The vast majority patients (90% of cases) with NS of childhood are primary. Definition In children under age 5 years the

2、 disease usually takes the form of idiopathic (primary) NS of childhood. 第3页，共70页。 Conditions Of Attack Second only to acute nephri- tisIncidence age: at all ages, but most commonly between 35 years of age第4页，共70页。 Type 1. Clinical type Simple type NS ; Nephritic NS 2. Response to steroid therapy (P

3、274)第5页，共70页。 Type The initial response to cortico- steroids is a guide to prognosis Steroid-dependent NS Steroid-responsive NS Steroid-resistant NS 第6页，共70页。 Type Total effect Partial effect Non-effect第7页，共70页。 Type 3. Pathologic type (P282) Minimal change disease, MCD 80% of all NS, little change

4、on light microscopy,第8页，共70页。 Type but electron microscopy shows effacement of foot processes (podocytes) of epithelial cells 第9页，共70页。 Type Other forms (Non-MCNS) MSPGN, FSGN, MGN, MPGN, etc. 第10页，共70页。第11页，共70页。第12页，共70页。第13页，共70页。第14页，共70页。第15页，共70页。 Pathogenesis The primary disorder is an increa

5、se in glomerular permeability to plasma proteinsloss of proteins, mainly albumin in urine第16页，共70页。 Pathogenesis Filtrated barrier Charge barrier Molecular barrier The loss of the negative charges on the GBM第17页，共70页。 Pathogenesis The underlying pathogenesis is unknown, but evidence strongly support

6、s the impor- tance of immune mechanisms (P281) 第18页，共70页。 Pathophysiology 1. Proteinuria: fundamental and highly important change of pathophysiology第19页，共70页。 Pathophysiology 2. Hypoproteinemia: mainly albumin 3. Edema: nephrotic edema (pitting edema)第20页，共70页。 Pathophysiology Hypoproteinemiaplasma

7、oncotic pressure, result in a shift of fluid from intervas- cular to extravascular com- partment edema 第21页，共70页。 Pathophysiology Plasma volumeactivates the reninangiotensinaldo- sterone system, also ADH secretion Na and water reabsorption第22页，共70页。 Pathophysiology 4.Hyperlipidemia (Hyper- cholester

8、olemia): Ch,TC, LDL-ch, VLDL-ch第23页，共70页。 Pathophysiology Caused by: 1. Hypoproteinemia stimulates liver protein synthesis including lipoproteins; 2. Lipid catabolism(lipoprotein lipases lost in urine?) 第24页，共70页。 Clinical Manifestations Peak age: 25 years Boys:girls = 3.7:1 1. Main manifestations E

9、dema (varying degrees) is the most common symptom, +/- weight gain第25页，共70页。 Clinical Manifestations Edema of periorbital /face, pitting edema in lower limbs, perineum anasarca evident Perhaps oliguria are noticed Ascites, pleural effusion Frothy urine 第26页，共70页。Clinical Manifestations 2. General sy

10、mptoms: pallid, anorexia, fatigue, diarrhea, abdominal pain第27页，共70页。 Laboratory Exam 1. Urinary protein: 2+4+ 24 h total urinary protein 50 mg/kg/d or 0.1g/m2/d ( the most are selective proteinuria )第28页，共70页。 Laboratory Exam UP/Ucr(mg/mg)3.5 (normal 0.2) May occur RBC (15%), granular and red cell

11、casts 第29页，共70页。 Laboratory Exam 2. Total serum protein, 30 g/L Albumin levels are low , often25 g/L 第30页，共70页。 Laboratory Exam 3. Serum lipids cholesterol(CH) 5.7mmol/L triglycerides(TC) LDL, VLDL第31页，共70页。 Laboratory Exam 4. ESR100 mm/h 5. Serum protein electrophoresis Albumen, 2-G,-G, A/G inversi

12、on 6. Serum Ca+ 第32页，共70页。 Laboratory Exam 7. Serum complement: vary with clinical type 8. Renal function BUN & serum Cr第33页，共70页。 Complications 1. Infections Acute infection is a major complication in children with NS. It frequently trigger relapses第34页，共70页。 Complications Often precipitated by vir

13、al infections Site: respiratory tract(URI), skin, urinary tract and acute primary peritonitis第35页，共70页。 Complications Due to: immunity lower (urinary loss of IgG, etc.) severe edemamalcirculation protein malnutrition use steroid therapy第36页，共70页。 Complications 2. Electrolyte disturbances 2.1. Hypona

14、tremia 2.2. Hypokalemia 2.3. Hypocalcemia第37页，共70页。 Complications3. Thromboembolic phenomena ( Hypercoagulability ): Potential arterial & venous thrombosis, e.g. renal vein thrombosis第38页，共70页。 Complications Due to: urinary loss of antithrombin, hepatic fibrinogen synthesis, platelet aggregationetc.

15、 第39页，共70页。 Complications 4. Hypovolemia (Hypovolemic shook) 5. Acute renal failure 6. Stunting第40页，共70页。 Diagnosis 1. Diagnostic standard (P273) Four characteristics Excluding other renal disease (second NS)第41页，共70页。 Diagnosis2. Clinical type: Simple type NS Nephritic type NS 第42页，共70页。 Management

16、1. General measures1.1. Rest1.2. Diet Hypertension and edema: low salt diet (2 g Na/ day) or salt-free diet第43页，共70页。 Management Generally do not restrict oral fluid intake Severe edema: restrict fluid intake and use diuretics 第44页，共70页。 Management Increase proteins properly 1.52 g/kg/d While underg

17、oing steroid treatment: give VitD 400 iu/d (or Rocaltrol) and calcium第45页，共70页。 Management 1.3. Prevent infection 1.4. Diuretics Not requires diuretics usually HCT 25 mg/kg/d第46页，共70页。 Management Antisterone 35 mg/kg/d Triamterene Attention: Hypovolemia, electrolyte disturbances and embolism第47页，共70

18、页。 Management Apparent edema Give low molecular dextran 1015 ml/kg/time;+Dopamine 23g/kg/min) and Regitine 10 mg +Lasix 2 mg/kg第48页，共70页。 Management 2. Corticosteroid therapy mainstay of treatment 2.1. Short-course therapy: Prednisone 2 mg/kg/d or 60 mg/m2/d (Max. 60 mg/d), 第49页，共70页。 Management in

19、3 or 4 divided doses for 4 wmaintenance treatment: Prednisone 1.52 mg/kg, every- other- day, given as a single, morning dose, for 4 w 第50页，共70页。 Management Total course of therapy: 8 w 2.2. Middle-course & long- course therapy Induction of remission 第51页，共70页。 Management Prednisone 2 mg/kg/d (Max.60

20、 mg/d) for 4 w until the urinary protein falls to trace/ (8w) 第52页，共70页。 Management After maintenance treatment Prednisone 2 mg/kg , single dose for every-other-day4 w tapered gradually (2.55 mg/ 24 wk) discontinued 第53页，共70页。 Management Total course of treatment Middle: 6 m Long: 912 m Estimate of

21、curative effect第54页，共70页。 Management 3. Treatment of relapse and frequently relapse 3.1. Extend the course of corti- costeroid 3.2. Change preparation第55页，共70页。 Management3.3. Immunosuppressive agents (Cytotoxic agents) CTX (Cyclophosphamide) 23 mg/kg/d for 812 w Total amount: 200 mg/kg 第56页，共70页。 M

22、anagement Side effects: leukopenia, trichomadesis, nausea, vomiting, hemorrhagic cystitis and fertility第57页，共70页。 Management CB (Chlorambucil) 0.2 mg /kg for 8 w Total amount: 10 mg/kg 6-MP, VCR, MMF第58页，共70页。 Management 4. Impulsive therapy 4.1. Methylprednisolone (MP) 1530 mg/kg(1g/d+10% GS 100 200 ml, iv drip第59页，共70页。 Management (within 12 h) , qd/qod, 3times /one course, if necessary give another 12 courses after 1 2 wprednisone第60页，共70页。 Management 4.2.CTX 1015 mg/kg or 0.50.75 /m2 + NS or 5%GNS100 200ml, iv drip (12 h), every 2w for 68 times,第61页，共70页。 Management total amount 1