Nephrottic

• What are the causes of Glomerular diseasegenerally

  Numerous primary (kidney disease alone) and secondary (due to systemic autoimmune disorders, vasculitis, or infection) disorders that produce glomerular disease
• Findings suggestive of glomerular disease


  • Proteinuria
  • Hematuria (microscopic or macroscopic)
  • Nephrotic syndrome
  • Arterial hypertension
  • Kidney insufficiency
• Acute nephritic syndrome


  Associated with active urine sediment with red cells, white cells, cellular casts, a variable degree of proteinuria, and, often, elevated blood pressure and a rising serum creatinine. Histologic examination demonstrates inflammation.
• Isolated nephrotic syndrome


  Presents with nephrotic-range proteinuria and, usually, an inactive urine sediment with few cells or casts. Most often, it is secondary to an idiopathic nephrotic syndrome with a rapid onset and peripheral edema.
• Macroscopic hematuria


  Recurrent episodes commonly seen in patients with IgA nephropathy or Alport syndrome. Microscopic hematuria may be observed in patients with IgA nephropathy, Alport syndrome, thin basement membrane syndrome, or poststreptococcal glomerulonephritis.
• Rapidly progressive glomerulonephritis


  Typically presents with heavy proteinuria, active urine sediment, and kidney failure that does not resolve spontaneously. The kidney biopsy shows extensive cellular crescents in most glomeruli.
• Chronic glomerulonephritis


  Associated with proteinuria, microscopic hematuria, hypertension, and, often, kidney function impairment.
• Urinalysis findings indicative of glomerular bleeding and glomerulonephritis


  • Red cell casts
  • Dysmorphic red blood cells
  • Proteinuria
• Nephrotic-range proteinuria


  Urinary protein excretion greater than 50 mg/kg per day or 40 mg/m2 per hour
• Glomerular filtration rate (GFR)


  Estimated using the Schwartz formula based on serum creatinine, age, height, and sex
• Serologic testing for glomerulonephritis


  • Antistreptolysin, antihyaluronidase, antistreptokinase, antinicotinamide-adenine dinucleotidase, and anti-DNase B antibodies (to screen for recent streptococcal infection)
  • Antinuclear antibodies (ANA) and anti-double-stranded deoxyribonucleic acid (dsDNA) antibodies (to screen for systemic lupus erythematosus)
  • Complement studies including C3, C4, and CH50 (to assess complement activation)
• Antistreptolysin


  Antibody that indicates a recent streptococcal infection
• Antihyaluronidase


  Antibody that indicates a recent streptococcal infection
• Antistreptokinase


  Antibody that indicates a recent streptococcal infection
• Antinicotinamide-adenine dinucleotidase


  Antibody that indicates a recent streptococcal infection
• Anti-DNase B antibodies


  Antibody that indicates a recent streptococcal infection
• Positive serology indicates a recent streptococcal infection and fulfills a criterion for the diagnosis of poststreptococcal glomerulonephritis
• Antinuclear antibodies (ANA)


  Elevated titer fulfills one of the classification criteria for systemic lupus erythematosus
• Anti-double-stranded deoxyribonucleic acid (dsDNA) antibodies


  Elevated titer fulfills one of the classification criteria for systemic lupus erythematosus
• Complement component 3 (C3)

  Low levels due to activation of the alternative pathway are associated with poststreptococcal and C3 glomerulopathies
• Complement component 4 (C4)

  Low levels due to activation of the classical pathway are seen in lupus nephritis and immune complex-mediated membranoproliferative glomerulonephritis
• Total hemolytic complement (CH50)


  Low levels are seen in poststreptococcal and C3 glomerulopathies
• C3 levels typically return to normal within 4-6 weeks in poststreptococcal glomerulopathy, but remain persistently low in C3 glomerulopathies
• Antineutrophil cytoplasmic antibodies (ANCA)


  Positive test is seen in most patients with granulomatosis with polyangiitis or microscopic polyangiitis
• IgA levels


  May be elevated in IgA nephropathy or IgA vasculitis
• A promising assay that screens for antibodies to galactose-deficient IgA1 may be helpful in diagnosing IgA nephropathy
• Serologic testing for Epstein-Barr virus or hepatitis B and C may be performed
• Kidney biopsy is performed to confirm a diagnosis, determine the extent of kidney injury, and/or predict renal outcome
• In rapidly progressive glomerulonephritis, early diagnosis with kidney biopsy and serologic testing and early initiation of appropriate therapy are essential to minimize irreversible kidney injury
• Transient proteinuria may occur during exercise or fever, and repeat testing is necessary to see if it is persistent
• Orthostatic proteinuria is a benign condition that needs no further evaluation
• Persistent isolated nonnephrotic proteinuria requires further evaluation as these patients are at-risk for glomerular disease
• Glomerular disease presentations

  • Proteinuria
  • Hematuria
  • Nephrotic syndrome
  • Arterial hypertension
  • Kidney insufficiency
• Glomerular disease patterns


  • Nephritic pattern (acute nephritis, rapidly progressive glomerulonephritis, chronic glomerulonephritis)
  • Isolated nephrotic syndrome
  • Macroscopic hematuria
• Children with acute nephritis typically have edema, hypertension, reddish-brown to brown-colored urine, rising serum creatinine, active urinary sediment, and variable proteinuria
• Children with isolated nephrotic syndrome may have anasarca with ascites, nephrotic-range proteinuria, and inactive urinary sediment
• Some children may have components of both nephritic and nephrotic syndrome
• Patients with secondary causes of glomerular disease often have nonrenal symptoms and signs suggestive of a specific underlying systemic disease
• Persistent isolated proteinuria or hematuria is often caused by etiologies other than glomerular disease, many of which are benign
• Further evaluation for nephritic syndrome pattern includes serologic testing, complement studies, and kidney biopsy