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Treatments for Nephronophthisis 2

Treatment List for Nephronophthisis 2

The list of treatments mentioned in various sources for Nephronophthisis 2 includes the following list. Always seek professional medical advice about any treatment or change in treatment plans.

  • Manage water and electrolyte balance. Once kidney failure occurs, dialysis or a kidney transplant is necessary for survival
  • Treatment of nephronopthisis is symptomatic as no therapy exists to prevent the progression to end stage renal failure. Management includes:
    • Aggressive management of high blood pressure - helps slow progression of renal disease. ACE (Angiotensin converting enzyme) inhibitors and ARB's (Angiotensin II receptor blockers) are considered first line therapy
    • Avoidance of medications that may contribute to further renal damage - NSAID's, IV contrast
    • Smoking cessation - people who continue to smoke progress to end stage renal failure sooner
    • Aggressive management of urinary tract infections with appropriate antibiotics
    • Treatment with growth hormone for children with renal impairment
    • Dietary management
      • Protein management dependant upon urinary protein loss and degree of renal impairment. Protein intake needs to be adequate for growth
      • Sodium management as required. May need to be restricted, or supplemented if salt wasting occurs
      • Adequate hydration
      • Phosphate restriction
      • Potassium restriction as necessary
    • Management of chronic renal failure:
      • Erythropoietin agonists - for management of anaemia associated with chronic renal failure
      • Phosphate binders - for management of hyperphosphatemia in chronic renal failure
      • Calcium supplements - for hypocalcaemia
      • Calcitriol and other Vitamin D supplements - for hypocalcaemia and hyperparathyroidism associated with chronic renal failure
      • Sodium bicarbonate - for acid-base disturbance
      • Diuretics - may be used in situation of volume overload
      • Renal dialysis - used when there are manifestations of uraemia and the GFR is < 10mL/min
      • Renal transplantation

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