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Nephrotic Syndrome (NS)
Definition NS is an accumulation of  symptoms and signs and is characterized by proteinuria, hypoproteinemia,  edema, and  hyperlipidemia. In children under age 5 years the disease usually takes the form of idiopathic (primary) NS of childhood
 
 
Pathogenesis   The primary disorder is an increase in glomerular permea- bility to plasma proteins.   *Foot processes of the visceral epithelium of the GBM. 1. The construction of the glomerular basement membrane has changed.  2. The loss of the negative charges on the GBM.   * The underlying pathogenesis is unknown, but evidence strongly supports the importance of immune mechanisms.
Pathophysiology   1. Proteinuria: Fundamental and highly important change of pathophysiology. 2. Hypoproteinemia (mainly albumin)  3. Edema: Nephrotic edema  (pitting edema) 4.   Hyperlipidemia (Hypercholesterolemia)   Ch↑, TG↑, LDL-ch↑,VLDL-ch↑.
  Clinical Manifestations There is a male preponderance of 2:1.   1. Main manifestations: Edema (varying degrees) is the common symptom. Periorbital swelling and perhaps oliguria are noticed->increasing edema->anasarca evident.  2. General symptoms:  Pallid, anorexia, fatigue,abdominal pain, diarrhea.
  Laboratory Exam 1. Urinary protein: 2 + ~ 4 +   24hr 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 electrophoresis :Albumin↓, α 2 -G↑,γ-G↓,A/G inversion. 6. Serum complemen: Vary with clinical type. 7. Renal function:
 
Complications  ,[object Object],[object Object]
Complications ,[object Object],[object Object]
Complications ,[object Object],[object Object],[object Object]
Principle of therapy (1) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Principle of therapy (2) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
General measures (supportive) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Specific treatment of underlying cause   ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Dietary recommendations   ,[object Object]
Dietary recommendations ,[object Object],[object Object],[object Object],[object Object],[object Object]
Prognosis ,[object Object]

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Nephrotic Syndrome

  • 2. Definition NS is an accumulation of symptoms and signs and is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia. In children under age 5 years the disease usually takes the form of idiopathic (primary) NS of childhood
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  • 5. Pathogenesis The primary disorder is an increase in glomerular permea- bility to plasma proteins. *Foot processes of the visceral epithelium of the GBM. 1. The construction of the glomerular basement membrane has changed. 2. The loss of the negative charges on the GBM. * The underlying pathogenesis is unknown, but evidence strongly supports the importance of immune mechanisms.
  • 6. Pathophysiology 1. Proteinuria: Fundamental and highly important change of pathophysiology. 2. Hypoproteinemia (mainly albumin) 3. Edema: Nephrotic edema (pitting edema) 4.   Hyperlipidemia (Hypercholesterolemia) Ch↑, TG↑, LDL-ch↑,VLDL-ch↑.
  • 7. Clinical Manifestations There is a male preponderance of 2:1. 1. Main manifestations: Edema (varying degrees) is the common symptom. Periorbital swelling and perhaps oliguria are noticed->increasing edema->anasarca evident. 2. General symptoms: Pallid, anorexia, fatigue,abdominal pain, diarrhea.
  • 8. Laboratory Exam 1. Urinary protein: 2 + ~ 4 + 24hr 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.
  • 9. 5. Serum proteins electrophoresis :Albumin↓, α 2 -G↑,γ-G↓,A/G inversion. 6. Serum complemen: Vary with clinical type. 7. Renal function:
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