Download - Nephrotic Syndrome
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Nephrotic Syndrome
• Etiology1. Idiopathic nephrotic syndrome (90%)– Minimal change disease (85%)– Focal segmental glomerulosclerosis (10%)– Mesangial proliferation (5%)
2. Glomerulonephritis; Membranous nephropathy; and membranoproliferative glomerulonephritis (10%)
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Idiopathic Nephrotic Syndrome
• Approximately 90% of children with nephrotic syndrome have idiopathic nephrotic syndrome
• Male:female ratio (2:1) • Peak incidence: 2 – 6 yr• The initial episode and subsequent relapses
may follow minor infections and, occasionally, reactions to insect bites, bee stings, or poison ivy.
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Idiopathic Nephrotic SyndromeMinimal Change Disease
Mesangial Proliferation
Focal segmental
glomerulosclerosis
Light Microscopy
Normal or minimal increase in mesangial cells and matrix
Diffuse increase in mesangial cells and matrix
Mesangial proliferation and segmental scarring
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Idiopathic Nephrotic SyndromeMinimal Change Disease
Mesangial Proliferation
Focal segmental
glomerulosclerosis
Immunofluorescence
Negative Trace to 1+ mesangial IgM and/or IgA staining
IgM and C3 staining in the areas of segmental sclerosis
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Idiopathic Nephrotic SyndromeMinimal Change Disease
Mesangial Proliferation
Focal segmental
glomerulosclerosis
Electron Microscopy
Effacement of epithelial foot processes
Increased numbers of mesangial cells and matrix; effacement of the epithelial cell foot processes
Segmental scarring of the glomerular tuft with obliteration of the glomerular capillary lumen
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Idiopathic Nephrotic SyndromeMinimal Change Disease
Mesangial Proliferation
Focal segmental
glomerulosclerosis
Steroid response
>95% 50% 20%
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Characteristics
• Proteinuria– >3.5 g/24 hr in adults– >40 mg/m2/hr in children– Spot urine protein to creatinine ratio >2.0
• Hypoalbuminemia– <2.5 g/dl
• Edema• Hyperlipidemia
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Proteinuria
• Results from increased permeability of glomerular basement membrane (GBM) to plasma protein
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Degrees of proteinuria• Mild less than
0.5g/m2/day• Moderate 0.5 –
2g/m2/day• Severe more than
2g/m2/day
Types of proteinuria• Selective proteinuria:
where proteins of low molecular weight .such as albumin, are excreted more readily than protein of HMW
• Non selective :• LMW+HMW are lost
in urine
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Hypoalbuminemia
• Due to hyperproteinuria– Mainly albumin
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Edema
• plasma oncotic pressure transudation of fluid from the intravascular compartment to the interstitial space
• intravascular volume renal perfusion (activates RAAS) tubular reabsorption of sodium water retention
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Hyperlipidemia
• Hypoalbuminemia stimulates generalized hepatic protein synthesis
• Diminished catabolism of lipids
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Complications
• Infection (major)– Bacterial peritonitis – most frequent– Sepsis– Pneumonia– Cellulitis– UTI
• Commonly caused by S. pneumoniae and E.coli
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Complications
• All children with nephrotic syndrome should receive polyvalent pneumococcal vaccine (if not previously immunized), ideally administered when the child is in remission and off of daily prednisone therapy.
• Nonimmune nephrotic children in relapse exposed to varicella should receive varicella zoster immune globulin (VZIG) within 72 hr of exposure.
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Complications
• Thromboembolic events– increased prothrombotic factors (fibrinogen,
thrombocytosis, hemoconcentration, relative immobilization)
– decreased fibrinolytic factors (urinary losses of antithrombin ill, proteins C and S)
• Prophylaxis is not indicated
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Prognosis
• Steroid-responsive nephrotic syndrome– Repeated relapses– Decrease in frequency as the child grows older
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Prognosis
• Children who respond to steroids rapidly and those who have no relapses during the first 6 mo after diagnosis tend to follow an infrequently relapsing course
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Prognosis
• Steroid-resistant nephrotic syndrome– most often caused by focal segmental
glomerulosclerosis– Generally have a much poorer prognosis
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Prognosis
• Recurrent nephrotic syndrome develops in 30-50% of transplant recipients with focal segmental glomerulosclerosis.
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Prevention
• Cannot be totally prevented– Usually follows minor infections, reactions to
insect bites, bee stings, or poison ivy
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BioPsychoSocial
• It is important to indicate to the family that:1. The child with steroid-responsive nephrotic
syndrome will not develop chronic renal failure2. The disease is generally not hereditary, and 3. The child (in the absence of prolonged
cyclophosphamide therapy) will remain fertile.
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BioPsychoSocial
• To minimize the psychological effects of the condition, the physician should emphasize that the child should be considered normal when in remission and may have unrestricted diet and activity, without the need for urine testing for protein.
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BioPsychoSocial
• Affected children may attend school and participate in physical activities as tolerated.