4.3 days

MEDICAL: Inborn Errors of Metabolism

Phosphorus is a major anion in the intracellular fluid that is measured in the serum; normal serum phosphorus levels range between 1.7 and 2.6 mEq/L (2.5 and 4.5 mg/dL). In children, the serum phosphorus level is higher, at 4.0 to 7.0 mg/dL. Hypophosphatemia occurs when the serum phosphorus levels fall below 1.7 mEq/L (2.5 mg/dL). Patients with moderate hypophosphatemia (1.0 to 2.5 mg/dL) are usually asymptomatic and require no treatment except to manage the underlying cause; patients with severe hypophosphatemia (<1 mg/dL) need more aggressive treatment to prevent complications. The possible complications of hypophosphatemia are grave and include dysrhythmias, heart failure, shock, destruction of striated muscles, seizures, and coma. Hypophos-phatemia occurs in approximately 1% to 5% of all hospitalized patients, 30% of patients admitted to critical care units, and 40% to 80% of patients with alcohol dependence, diabetic ketoacidosis, sepsis, or septic shock. Severe hypophosphatemia is rare and occurs in less than 1% of all hospitalized patients.

Approximately 85% of body phosphorus is in bone, and most of the remainder is intracel-lular. Only 1% is in the extracellular fluid. Phosphorus serves many functions in the body, such as maintenance of the normal nerve and muscle activity; formation and strength of bones and teeth; maintenance of cell membrane structure and function; metabolism of carbohydrates, proteins, and fats; maintenance of oxygen delivery to the tissue; maintenance of acid-base balance; and activation of the B complex vitamins. Phosphorus is excreted by the kidneys (90%) and gastrointestinal (GI) tract (10%). Regulation of phosphorus is controlled by parathyroid hormone (PTH). PTH stimulates a vitamin D derivative (calcitriol) to increase phosphorus absorption by the GI tract. PTH acts on the renal tubules to increase phosphate excretion.

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