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Treatments for Juvenile nephronophthisis

Treatment List for Juvenile nephronophthisis

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

  • 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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