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纯合子型地中海贫血患者的钙磷代谢与骨病

Calcium phosphate metabolism and bone disease in patients with homozygous thalassemia.

作者信息

de Vernejoul M C, Girot R, Gueris J, Cancela L, Bang S, Bielakoff J, Mautalen C, Goldberg D, Miravet L

出版信息

J Clin Endocrinol Metab. 1982 Feb;54(2):276-81. doi: 10.1210/jcem-54-2-276.

DOI:10.1210/jcem-54-2-276
PMID:7054221
Abstract

Calcium and phosphate metabolism were studied in 22 patients with homozygous thalassemia. The overall results showed no significant difference for serum calcium, phosphorus, alkaline phosphatase, immunoreactive parathyroid hormone, or 25-hydroxyvitamin D between thalassemic and control children. However, during the winter, serum 25-hydroxycholecalciferol levels were very significantly decreased in thalassemic children. A study of the hands showed thin metacarpal cortices related to increased resorption. Histomorphometric study of four iliac bone biopsies showed normal osteoclastic resorption and decreased bone formation. Prussian blue staining and x-ray electron microprobe analysis showed iron deposits inside the bone. Whether this finding is critical in the pathogenesis of the bone disease in unknown.

摘要

对22例纯合子型地中海贫血患者的钙磷代谢进行了研究。总体结果显示,地中海贫血患儿与对照儿童在血清钙、磷、碱性磷酸酶、免疫反应性甲状旁腺激素或25-羟维生素D方面无显著差异。然而,在冬季,地中海贫血患儿的血清25-羟胆钙化醇水平显著降低。手部研究显示掌骨皮质变薄与吸收增加有关。对4例髂骨活检进行组织形态计量学研究,结果显示破骨细胞吸收正常但骨形成减少。普鲁士蓝染色和X射线电子微探针分析显示骨内有铁沉积。这一发现对骨病发病机制是否至关重要尚不清楚。

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