Fanconi Syndrome


Risk

  • FS can be inherited, acquired, or caused by exogenous factors.

  • Incidence is sporadic. Exact incidence in USA is not clear.

  • Most diseases associated with FS are inherited in an autosomal recessive pattern.

  • Cystinosis is the most common cause in pediatric pts.

Perioperative Risks

  • Potential for hypotension secondary to hypovolemia

  • Renal failure, proximal renal tubular dysfunction

  • Lyte imbalance (especially hypokalemia causing tachyarrhythmias)

Worry About

  • Polyuria, polydipsia, and the resulting dehydration

  • Type 2 renal tubular acidosis (defect in the reabsorption of bicarbonate in the proximal tubule)

  • Hypokalemia-induced ventricular arrhythmogenicity

  • Hypophosphatemia and associated osteomalacia and loss of bone density

  • Hypokalemia may cause muscular weakness

  • May be part of a genetic syndrome, such as Lowe syndrome (oculocerebrorenal syndrome)

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