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Glomerular Diseases  in Children Lecturer Xin Yue TIANJIN MEDICAL UNIVERSITY  PEDIATRIC DPT.  GENERAL HOSPITAL
Nephrotic syndrome (NS)
Introduction The nephrotic syndrome (NS) is characterized by Edema Hypercholesterolemia  (greater than 5.72  mmol/L) Hypoalbuminemia (less than   30 g/L ) Severe proteinuria (greater than  0.05/kg/24hr)
Secondary  nephrosis Primary nephrotic syndrome(90%) Types of nephrotic syndrome Congenital nephrosis Simple  nephrosis Nephritic  nephrosis
Etiology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Pathology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],Minimal-change Disease
 
 
Normal Foot Processes Effacement of Foot Processes
 
 
 
MGN: Mildly Thickened Capillary Loops
 
 
 
Pathophysiology ,[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Proteinuria(1)
[object Object],[object Object],[object Object],[object Object],[object Object],Proteinuria(2)
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],glomerular filter membrane
The glomerular capillary wall: Charge  selective filter The glomerular capillary wall: Size selective filter
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Hypoproteinemia(1)
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Hypoproteinemia(2)
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Edema(1) Traditional  opinions:
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Edema(2) Recent  opinions:
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Hyperlipidemia
Clinical manifestations ,[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],Edema
 
 
 
 
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Classification
[object Object],[object Object],[object Object],[object Object],[object Object],Simple nephrosis
[object Object],[object Object],[object Object],[object Object],[object Object],Nephritic nephrosis
Differential between simple and nephritic nephrosis Typical features Simple nephrosis   Nephritic nephrosis Age   2~7 yr, peak age is 3 yr  School age Edema   Severe (ascites)   Mild or moderate Hyperlipidemia   ↑↑   ↑ Proteinuria   + + + ~ + + + +  ++ ~ +++ >50~100mg/kg/24hr   <50mg/kg/24hr  Serum albumin   ↓↓   Normal/slightly  ↓ Hematuria   RBC < 10/HP   RBC > 10/HP   Blood pressure   Normal   Raised Complement C 3   Normal  Normal/low Renal function   Normal  Abnormal Histological type  MCNS  FSGS, MPGN Prognosis   well  Bad
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],[object Object],[object Object],Infections
[object Object],[object Object],[object Object],[object Object],Hypovolemia
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Electrolyte disturbances
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Hypercoagulability states and thrombosis
[object Object],[object Object],Acute renal failure
Laboratory findings ,[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],[object Object],[object Object],[object Object]
Diagnosis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Differential diagnosis ,[object Object]
Treatment(1) ,[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],Activity
[object Object],[object Object],[object Object],[object Object],Diet
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Diuretic therapy
[object Object],[object Object],[object Object],Treatment of complications
Treatment(2) ,[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Glucocorticoid therapy
Medium-long term prednisone therapy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Short term prednisone therapy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],NS types classified  by response to steroid therapy(1)
[object Object],[object Object],[object Object],[object Object],NS types classified  by response to steroid therapy(2)
Adverse effects of long term corticosteroid treatment   ,[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],Cytotoxic agent therapy
[object Object],[object Object],[object Object],[object Object],Pulse therapy
Prognosis(1) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],Prognosis(2)
Indications of renal biopsy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Keys to be remembered ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

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Nephrosis 2009,

  • 1. Glomerular Diseases in Children Lecturer Xin Yue TIANJIN MEDICAL UNIVERSITY PEDIATRIC DPT. GENERAL HOSPITAL
  • 3. Introduction The nephrotic syndrome (NS) is characterized by Edema Hypercholesterolemia (greater than 5.72 mmol/L) Hypoalbuminemia (less than 30 g/L ) Severe proteinuria (greater than 0.05/kg/24hr)
  • 4. Secondary nephrosis Primary nephrotic syndrome(90%) Types of nephrotic syndrome Congenital nephrosis Simple nephrosis Nephritic nephrosis
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  • 10. Normal Foot Processes Effacement of Foot Processes
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  • 14. MGN: Mildly Thickened Capillary Loops
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  • 22. The glomerular capillary wall: Charge selective filter The glomerular capillary wall: Size selective filter
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  • 37. Differential between simple and nephritic nephrosis Typical features Simple nephrosis Nephritic nephrosis Age 2~7 yr, peak age is 3 yr School age Edema Severe (ascites) Mild or moderate Hyperlipidemia ↑↑ ↑ Proteinuria + + + ~ + + + + ++ ~ +++ >50~100mg/kg/24hr <50mg/kg/24hr Serum albumin ↓↓ Normal/slightly ↓ Hematuria RBC < 10/HP RBC > 10/HP Blood pressure Normal Raised Complement C 3 Normal Normal/low Renal function Normal Abnormal Histological type MCNS FSGS, MPGN Prognosis well Bad
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