Tumoral calcinosis: Evidence for concurrent defects in renal tubular phosphorus transport and in 1α,25-dihydroxycholecalciferol synthesis
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A 50-year-old Latin American man with tumoral calcinosis presented with hyperphosphatemia (6.62±1.04 SD mg/dl), elevated renal threshold phosphorus concentration (TmP) (7.3 mg/GFR), and 1,25-dihydroxyvitamin D [1,25-(OH)2D] (69 pg/ml) hypercalciuria (239 mg/day), and a high fractional intestinal calcium (Ca) absorption (0.74). Sodium cellulose phosphate therapy (20 g/day) lowered urinary Ca, and partially reduced serum phosphorus (P) and TmP to 5.91±0.63 mg/dl and 6.2 mg/GFR, respectively. Serum 1,25-(OH)120D remained elevated at 58–64 pg/ml. Amphojel therapy (4 oz/day) decreased urinary P to 23±21 mg/day and lowered serum P to 5.75±0.36 mg/dl (P<0.05). TmP increased to a value of 8.0 mg/GFR while serum 1,25-(OH)2D continued to remain elevated at 53 pg/ml.
This case illustrates the probable operation of dual abnormalities in tumoral calcinosis represented by augmented renal conservation of P and an elevation in the circulating concentration of 1,25-(OH)2D.
Key wordsEctopic calcification 1,25-dihydroxyvitamin D hyperphosphatemia Bone Renal tubule
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