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• 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
Proteinuria
• Results from increased permeability of glomerular basement membrane (GBM) to plasma protein
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
Hypoalbuminemia
• Due to hyperproteinuria– Mainly albumin
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
Hyperlipidemia
• Hypoalbuminemia stimulates generalized hepatic protein synthesis
• Diminished catabolism of lipids
Complications
• Infection (major)– Bacterial peritonitis – most frequent– Sepsis– Pneumonia– Cellulitis– UTI
• Commonly caused by S. pneumoniae and E.coli
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.
– decreased fibrinolytic factors (urinary losses of antithrombin ill, proteins C and S)
• Prophylaxis is not indicated
Prognosis
• Steroid-responsive nephrotic syndrome– Repeated relapses– Decrease in frequency as the child grows older
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
Prognosis
• Steroid-resistant nephrotic syndrome– most often caused by focal segmental
glomerulosclerosis– Generally have a much poorer prognosis
Prognosis
• Recurrent nephrotic syndrome develops in 30-50% of transplant recipients with focal segmental glomerulosclerosis.
Prevention
• Cannot be totally prevented– Usually follows minor infections, reactions to
insect bites, bee stings, or poison ivy
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.
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.
BioPsychoSocial
• Affected children may attend school and participate in physical activities as tolerated.