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DAVID L. HALSEY,
NANCY E. FRIEDMAN and
BRUCE LOBAUGH
Geriatrics Research, Education and Clinical Center, Veterans Administration Medical Center, and Departments of Medicine, Pediatrics, and Surgery, Duke University Medical Center Durham, North Carolina 27710
Address requests for reprints to: Kenneth W. Lyles, M.D., Geriatrics Research Education and Clinical Center, Veterans Administration Center, 508 Fulton Street, Durham, North Carolina 27705.
The inherited metabolic disorder tumoral calcinosis is characterized by elevated serum phosphorus and 1,25- dihydroxyvitamin D [1,25-(OH)2D] levels and paraarticular calcific tumors. The pathogenesis of this disease is obscure, but an elevated renal phosphate reabsorption threshold and increased production of 1,25-(OH)2D are postulated as defects. We studied nine affected patients and found that both serum phosphorus and renal phosphate reabsorption threshold (TmP/GFR) were positively correlated with serum 1,25-(OH)2D levels. Since tumoral calcinosis is a disorder with abnormal renal phosphate transport, we compared the TmP/GFR and serum 1,25-(OH)2D levels to values obtained in patients with two other diseases with renal phosphate transport defects: oncogenic osteomalacia and X-linked hypophosphatemic rickets. We found a significant correlation between TmP/GFR and 1,25-(OH)2D levels in all three diseases, suggesting that in these diseases 1,25-(OH)2D production is regulated in some manner by phosphate transport. Furthermore, previous work indicated that in tumoral calcinosis broad variation exists in serum phosphorus levels. In our patients a negative correlation was found between the serum PTH concentrations and both serum phosphorus levels and TmP/GFR values, respectively. We postulate that although the basic defect in tumoral calcinosis most likely resides in the proximal renal tubular cell, the variation in serum phosphorus levels and possibly disease expression is modulated in part by PTH.
* Portions of this work have been published in abstract form (J Bone Mineral Res 1:212, 1986; J Bone Mineral Res [Suppl 1] 1:200, 1986; and CPC Series: Cases in Metabolic Bone Disease, vol 2:1211, 1987. This work was supported by Grant RR-30 from the Division of Research Resources, General Clinical Research Centers Program, NIH, and the V.A.
Supported by Grant BRSG-807-RR-05405, awarded by the Biochemical Research Support Grant Program, Division of Research Resources, NIH. Recipient of NIA Academic Award AG-00367-01.
Supported by NIH Grant 1R01-AMAG-36903-01A1.
Received May 13, 1987.
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