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Key Features

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  • Most commonly results from developmental abnormalities of the kidneys or urinary tract.

  • Obstructive uropathy or severe vesicoureteral reflux (VUR) without (or despite) surgical intervention is often associated with progressive kidney disease in children

  • In older children, the chronic glomerulonephritides, irreversible nephrotoxic injury, or hemolytic-uremic syndrome may also cause chronic kidney disease (CKD)

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Complications
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  • Polyuria and dehydration are more likely than fluid overload in children who have structural kidney lesions associated with impaired urinary concentration

  • Salt-wasting state can occur

  • However, sodium and water retention with associated hypertension and eventual loss of urinary output is characteristic in children with CKD due to glomerular disease or acute kidney injury

  • Metabolic acidosis and growth retardation occur early in CKD

  • Disturbances in calcium, phosphorus, and vitamin D metabolism leading to renal osteodystrophy and rickets require prompt attention

  • Anemia due to decreased erythropoietin production can occur relatively early

  • Anorexia, nausea, and malaise occur late in CKD (generally < 30% renal function)

  • CNS abnormalities such as confusion and lethargy are very late symptoms, followed even later by stupor and coma

    • Unusual in children because medical attention is sought before deteriorating to this point

    • Rise in blood urea nitrogen (BUN) is typically gradual

  • Other late complications of untreated CKD are platelet dysfunction and bleeding tendencies, pericarditis, and chronic fluid overload leading to heart failure, pulmonary edema, and worsening hypertension

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Treatment of Complications

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  • Acidosis may be treated with sodium citrate or bicarbonate solutions, as long as the added sodium does not aggravate hypertension

  • Sodium restriction is advisable when hypertension is present

  • Hyperphosphatemia is controlled by dietary restriction and dietary phosphate binders (eg, calcium carbonate, sevelamer)

  • Supplementation with vitamin D (cholecalciferol or ergocalciferol) and calcitriol

    • Typically required

    • Target the prevention of renal osteodystrophy or rickets

  • Dietary potassium restriction will be necessary as the glomerular filtration rate falls

  • Renal function must be monitored regularly (creatinine and BUN)

  • Serum electrolytes, calcium, phosphorus, intact parathyroid hormone, iron and ferritin, and hemoglobin and hematocrit levels must be monitored to guide changes in

    • Fluid and dietary management

    • Dosages of phosphate binder, citrate buffer, vitamin D supplements, blood pressure medications, iron supplements, and epoetin alfa

  • Linear growth failure may be treated with daily subcutaneous human recombinant growth hormone

  • Avoid medications that

    • Aggravate hypertension

    • Increase the body burden of sodium, potassium, or phosphate

    • Increase production of BUN

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Treatment: Dialysis

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  • Chronic peritoneal dialysis (home-based) and hemodialysis provide lifesaving treatment for children prior to kidney transplantation

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Outcome

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Prognosis
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  • Graft survival rate for living-related kidney transplants

    • 90% at 1 year

    • 85% at 2 years

    • 75% at 5 years

  • Graft survival rates for cadaveric transplantation

    • 76% at 1 year

    • 71% at 2 years

    • 62% at 5 years

  • Overall, the mortality rate is 4% for recipients of living-related donors and 6.8% for recipients of cadaver organs

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References

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Shellmer ...

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