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低磷性维生素D抵抗性佝偻病(磷酸盐糖尿病):通过125I计算机断层扫描和微放射摄影研究的骨矿物质问题

Hypophosphatemic vitamin D resistant rickets (phosphate diabetes): bone mineral problems studied by 125I-computed tomography and microradiography.

作者信息

Exner G U, Prader A, Elsasser U, Rüegsegger P, Anliker M, Steendijk R

出版信息

Helv Paediatr Acta. 1980 Mar;35(1):39-49.

PMID:6250997
Abstract

125I-computed tomography (CT) for the bone mineral analysis of the radius was performed on 9 children with vitamin D resistant hypophosphatemic rickets (VDRR) treated with vitamin D in pharmacological doses. Trabecular bone density was increased in 1 patient and normal in all others. The cortical width of the diaphysis of the radius was decreased, and the cross sectional area of the bone increased, but the total amount of compat bone mineral in the cross section was normal. Measurements of the second metacarpal bone on X-rays of the hand revealed similar findings. Microradiographic and histological examinations of the fibula on 9 different patients also treated with vitamin D showed grossly abnormal mineralization of bone tissue with a perilacunar mineral deficit. It is concluded, that the amount of compact bone and trabecular bone is not decreased in children with VDRR. The normal mineral content determined by CT and the impaired mineralization of the bone material examined by microradiography indicate an over-abundance of incompletely mineralized bone.

摘要

对9名接受药理剂量维生素D治疗的维生素D抵抗性低磷血症佝偻病(VDRR)患儿进行了125I计算机断层扫描(CT)以分析桡骨的骨矿物质。1例患者的小梁骨密度增加,其他所有患者的小梁骨密度正常。桡骨干骺端的皮质宽度减小,骨横截面积增加,但横截面上骨矿物质总量正常。对手部X线片上的第二掌骨进行测量也得到了类似结果。对另外9名同样接受维生素D治疗的不同患者的腓骨进行微射线照相和组织学检查,结果显示骨组织矿化严重异常,存在骨陷窝周围矿物质缺乏。得出的结论是,VDRR患儿的密质骨和小梁骨数量并未减少。CT测定的正常矿物质含量以及微射线照相检查显示的骨材料矿化受损表明存在大量矿化不完全的骨。

相似文献

1
Hypophosphatemic vitamin D resistant rickets (phosphate diabetes): bone mineral problems studied by 125I-computed tomography and microradiography.低磷性维生素D抵抗性佝偻病(磷酸盐糖尿病):通过125I计算机断层扫描和微放射摄影研究的骨矿物质问题
Helv Paediatr Acta. 1980 Mar;35(1):39-49.
2
Familial hypophosphatemic rickets: bone mass measurements in children following therapy with calcitriol and supplemental phosphate.家族性低磷血症性佝偻病:骨化三醇和补充磷酸盐治疗后儿童的骨量测量
Calcif Tissue Int. 1989 Feb;44(2):86-92. doi: 10.1007/BF02556466.
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Bone mineral density of the spine and radius shaft in children with X-linked hypophosphatemic rickets (XLH).X连锁低磷性佝偻病(XLH)患儿的脊柱和桡骨干骨密度
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Evaluation of a role for 1,25-dihydroxyvitamin D3 in the pathogenesis and treatment of X-linked hypophosphatemic rickets and osteomalacia.评估1,25-二羟维生素D3在X连锁低磷性佝偻病和骨软化症的发病机制及治疗中的作用。
J Clin Invest. 1980 Nov;66(5):1020-32. doi: 10.1172/JCI109930.
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Bone response to phosphate salts, ergocalciferol, and calcitriol in hypophosphatemic vitamin D-resistant rickets.低磷性维生素D抵抗性佝偻病中骨骼对磷酸盐、麦角钙化醇和骨化三醇的反应
N Engl J Med. 1980 Oct 30;303(18):1023-31. doi: 10.1056/NEJM198010303031802.
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Cortical and trabecular bone density in X-linked hypophosphatemic rickets.X 连锁低磷血症性佝偻病的皮质骨和小梁骨密度。
J Clin Endocrinol Metab. 2013 May;98(5):E954-61. doi: 10.1210/jc.2012-4133. Epub 2013 Mar 26.
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Metacarpal measurements in X-linked hypophosphataemic rickets.X连锁低磷性佝偻病的掌骨测量
Helv Paediatr Acta. 1986 Oct;41(4):331-8.
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Stimulation of cortical bone mineralization and remodeling by phosphate and 1,25-dihydroxyvitamin D in vitamin D-resistant rickets.
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[Pathomorphology and mineral saturation of the bone tissue in congenital vitamin D-resistant rickets].[先天性维生素D抵抗性佝偻病骨组织的病理形态学与矿物质饱和度]
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[Tubular and intestinal phosphate transport in vitamin D-resistant (hypophosphatemic) rickets (author's transl)].维生素D抵抗性(低磷血症性)佝偻病中的肾小管和肠道磷转运(作者译)
Padiatr Padol. 1977;12(2):152-7.

引用本文的文献

1
Familial hypophosphatemic rickets: bone mass measurements in children following therapy with calcitriol and supplemental phosphate.家族性低磷血症性佝偻病:骨化三醇和补充磷酸盐治疗后儿童的骨量测量
Calcif Tissue Int. 1989 Feb;44(2):86-92. doi: 10.1007/BF02556466.