Abstract
Nephrocalcinosis is a major complication in patients with XLHR. In a multicentre retrospective study on 155 children and adolescents we found in 62/133 patients signs of NC by ultrasound, 23 were /=4y. of age. With high oral phosphate load hyperphosphaturia and hyperoxa-luria occurs, but hypercalciuria due to secondary hyperparathyroidism may also develop. A subgroup(‘noD’)of 8 prepubertal(0.7-4.4y., median; 1.95y.)and 2 pubertal children were treated with oral phosphate (18-173mg/kg, median:71mg/kg)but very low vit.D2(7500U/day). They were compared with 14XLHR children (=‘D’, age:0.5-4.1y., median: 1.9y.)given 1, 25(OH)2D(max.dose:19-75ng/kg,median:38ng/kg)and oral phosphate(max. 64-150mg/kg, median :94rng/kg)daily.
Results; ‘noD’ :hypercalcemia(2.55mmol/l)were found in 8/10 more than once, PTH1-84(normal:5-55pg/ml)were elevated in 8/10(37-397pg/ml, median:90pg/ml). NC was confirmed in 6/9. Group‘D’ :hypercalcemia were found in 8/14 more than once, PTH1-84 were elevated in 1/14(13-80pg/ml, median:35pg/ml). NC occurs in 7/11.
Conclusions: 1)NC might occur in patients with XLHR even in cases with insufficient low vit.D therapy, but usual oral phosphate load(70mg/kg) 2) Hyperparathyroidism and hypercalcemia are frequent findings in patients with inadequate low doses of vit.D. 3) Combination of 1-OH-vit.D derivates (20-40ng/kg*d)and high oral phosphate prevents hyperparathyroidism.
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Mohnike, K., Knye, K., Cagnoli, M. et al. NEPHROCALCINOSIS (NC) DUE TO HYPERPARATHYROIDISM IN X-LINKED HYPOPHOSHATEMIC RICKETS (XLHR)-RESULTS OF A RETROSPECTIVE STUDY IN 155 CHILDREN AND ADOLESCENTS. Pediatr Res 33 (Suppl 5), S82 (1993). https://doi.org/10.1203/00006450-199305001-00472
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DOI: https://doi.org/10.1203/00006450-199305001-00472
