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肿瘤性钙化:肾小管磷转运及1α,25-二羟胆钙化醇合成同时存在缺陷的证据。

Tumoral calcinosis: evidence for concurrent defects in renal tubular phosphorus transport and in 1 alpha,25-dihydroxycholecalciferol synthesis.

作者信息

Zerwekh J E, Sanders L A, Townsend J, Pak C Y

出版信息

Calcif Tissue Int. 1980;32(1):1-6. doi: 10.1007/BF02408515.

DOI:10.1007/BF02408515
PMID:6775776
Abstract

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)2D 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.

摘要

一名患有肿瘤性钙化的50岁拉丁裔男性,伴有高磷血症(6.62±1.04标准差mg/dl)、肾磷阈浓度(TmP)升高(7.3mg/GFR)、1,25-二羟维生素D[1,25-(OH)2D](69pg/ml)、高钙尿症(239mg/天)以及高小肠钙(Ca)吸收分数(0.74)。磷酸纤维素钠治疗(20g/天)降低了尿钙,并使血清磷(P)和TmP分别部分降至5.91±0.63mg/dl和6.2mg/GFR。血清1,25-(OH)2D仍维持在58 - 64pg/ml的高水平。氢氧化铝治疗(4盎司/天)使尿磷降至23±21mg/天,并使血清磷降至5.75±0.36mg/dl(P<0.05)。TmP升至8.0mg/GFR,而血清1,25-(OH)2D继续维持在53pg/ml的高水平。该病例说明了肿瘤性钙化中可能存在的双重异常,表现为肾脏对磷的保留增加以及循环中1,25-(OH)2D浓度升高。

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