Nephrotic Syndrome (NS)

Definition NS is an accumulation of symptoms and signs and is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia.

Type 1.Clinical type Simple NS ; Nephritic NS 2.Response to steroid therapy

3. Pathologic type Minimal change disease, MCD: 80% of patients.

Pathophysiology 1.Proteinuria: Fundamental and highly important change of pathophysiology.

2.Hypoproteinemia (mainly albumin) 3.Edema: Nephrotic edema (pitting edema)

Hypoproteinemia plasma oncotic pressure is diminished, result in a shift of fluid from the vascular to the interstitial compartment and plasma

volume↓ → the activation of the renin–angiotensin–aldo- sterone system → tubular sodium chloride reabsorp- tion↑.

4. Hyperlipidemia (Hyper- cholesterolemia)

فیزیوپاتولوژی تخریب غشائ مویرگ های گلومرولی کاهش پروتئین پلاسما کاهش فشاراونکوتیک ادم عمومی مایعات ازعروق وارد فضای خارج سلولی می شوند فعال شدن سیستم رنین آنژیوتانسین احتباس سدیم هیپوآلبومینمی تحریک تولید لیپوپروتئین هیپر لیپیدمی ادم

Laboratory Exam 1.Urinary protein: 2 + ～ hr total urinary protein > 0.1g/kg. ( The most are selective proteinuria. )

May occur granular and red cell casts. 2.Total serum protein↓, ＜ 30g/L. Albumin levels are low ( ＜ 20g/L).

3.Serum cholesterol and triglycerides: Cholesterol ＞ 5.7mmol/L (220mg/dl). 4. ESR↑ ＞ 100mm/hr.

5.Serum proteins electro- phoresis : Albumin↓, α 2 -G↑,γ-G↓, A/G inversion. 6.Renal function

Complications 1.Infections Infections is a major compli- cation in children with NS.

Site: Respiratory tract, skin, urinary tract and acute pri- mary peritonitis.

Causes: Immunity lower, severe edema→malcirculation, protein malnutrition, and use hormone and immunosuppre- ssive agents.

2.Electrolyte disturbances (1) Hypernatremia (2) Hypokalemia (3) Hypocalcemia

3.Thromboembolic phenomena ( Hypercoagulability ) Renal vein thrombosis 4.Hypovolemic shook 5.Acute renal failure (prerenal)

Diagnosis 1.Diagnostic standard ●Four characteristics. ●Excluding other renal disease (second nephrosis).

2.Clinical type Simple NS or Nephritic NS. Treatment 1.General measures 1.1 Rest

1.2 Diet Hypertension and edema: Low salt diet (<2gNa/ day) or salt-free diet. Severe edema: Restricting fluid intake.

Increase proteins properly: 2g/(kg·day) While undergoing the corti- costeroid treatment: Give VitD 500~1000iu/day (or Rocaltrol) and calcium.

1.3 Prevent infection 1.4 Diuretics Not requires diuretics usually.

Attention: Volume depletion, disorder of electrolyte and embolism.

Apparent edema: Give low molecular dextran Dopamine 2~3ug/(kg·min) and/or Regitine 10mg +Lasix 1~2mg/kg].

2.Corticosteroid therapy

3. Treatment of relapse and recurrence 3.1 Extend the course of corti- costeroid 3.2 Immunosuppressive agents (Cytotoxic agents):

① CTX (Cytoxan) 2mg/(kg·day) for 8~12wk. Total amount: 250mg/kg Side effects: nausea, vomiting, WBC↓, trichomadesis, hemo- rrhagic cystitis and the damage of sexual glands.

② CB (Chlorambucil) 0.2mg/kg for 8wk. Total amount : 10mg/kg ③ VCR & Levamisole

4.Impulsive therapy (1) Methylprednisolone (MP) 15~30mg/kg(<1g/day+10% GS 100~ 250ml, iv drip (within 1~2hr), 3 times/one course. If

(2) CTX 0.5~0.75mg/m 2 + NS/GS iv drip (1hr), give liquid 2,000ml /(m 2.d). Every one mo for 6~8 times.

(3) Anticoagulants Heparin Persantin 5mg/(kg·day ） for 6mo.

4.Alleviar proteinuria Angiotensin converting en- zyme inhibitions (ACEI) : Captopril, Enalapril and Benazepril.