PEDIATRICS Vol. 97 No. 1 January 1996, pp. 91-93
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Nephrocalcinosis Is Associated With Renal Tubular Acidosis in Children With X-Linked Hypophosphatemia

Mouin Seikaly MD1, Richard Browne PhD2, and Michel Baum MD3

1 University of Texas Southwestern Medical Center and Texas Scottish Rite Hospital, Dallas, TX
2 Texas Scottish Rite Hospital, Dallas, TX
3 University of Texas Southwestern Medical Center, Dallas, TX

Background. X-linked hypophosphatemia is characterized clinically by rickets and growth retardation. Therapy of this disorder with phosphate and vitamin D often produces nephrocalcinosis. The long-term effects of nephrocalcinosis on renal function in patients with X-linked hypophosphatemia are unknown. The purpose of this study was to evaluate the prevalence of glomerular and tubular disorders in patients with X-linked hypophosphatemia who developed nephrocalcinosis.

Methods. The creatinine clearance and the prevalence of renal tubular acidosis were compared in 19 patients with X-linked hypophosphatemia and nephrocalcinosis with 15 patients with X-linked hypophosphatemia Without nephrocalcinosis.

Results. Sixteen of the 19 patients (84%) with n nephrocalcinosis had a hyperchloremic metabolic acidosis compared with one of the 13 patients without nephrocalcinosis (P < .01). The serum bicarbonate of patients with nephrocalcinosis was 20.0 ± 0.7 as compared to 24.5 ± 0.6 mmol/L in patients without nephrocalcinosis (P < .01). The urinary anion gap was positive in all patients with acidosis (+62.1 ± 13.3 mmol/L). The creatinine clearance was 125 ± 6 mL/min/1.73 m2 in patients with nephrocal nephrocalcinosis and 124 ± 7 mLmin/1.73 m2 in those without nephrocalcinosis.

Conclusion. Therapy of X-linked hypophosphatemia is often associated with nephrocalcinosis. Nephrocaldnosis is associated with renal tubular acidosis in patients with X-linked hypophosphatemia.

Submitted on December 6, 1994
Accepted on January 31, 1995




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