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心房内钙输注、生长以及终末器官对维生素D的抵抗与发育

Intra-atrial calcium infusions, growth, and development in end organ resistance to vitamin D.

作者信息

Lin J P, Uttley W S

机构信息

Royal Hospital for Sick Children, Edinburgh.

出版信息

Arch Dis Child. 1993 Dec;69(6):689-92. doi: 10.1136/adc.69.6.689.

DOI:10.1136/adc.69.6.689
PMID:8285784
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1029657/
Abstract

A five and a half year follow up of a girl with end organ resistant rickets is described. After failing to respond to high dose oral alfacalcidol (4 micrograms/kg/day) and calcium supplements, treatment for one year with domiciliary intra-atrial calcium infusions at 2 years of age induced a complete remission, which was maintained on subsequent high dose oral calcium supplement. Overnight infusions were well tolerated without adverse cardiovascular or renal sequelae or ectopic calcification. If the first three years of life are survived, the prognosis for a normal life on oral treatment is excellent.

摘要

本文描述了一名患有终末器官抵抗性佝偻病女孩的五年半随访情况。在对高剂量口服阿法骨化醇(4微克/千克/天)和钙剂补充治疗无反应后,该女孩在2岁时接受了为期一年的家庭心房内钙剂输注治疗,随后完全缓解,并通过后续高剂量口服钙剂得以维持。夜间输注耐受性良好,未出现不良心血管或肾脏后遗症或异位钙化。如果能度过生命的头三年,口服治疗下正常生活的预后极佳。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ac9/1029657/cec5eeab4aa7/archdisch00560-0071-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ac9/1029657/dd64b4c87302/archdisch00560-0069-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ac9/1029657/cec5eeab4aa7/archdisch00560-0071-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ac9/1029657/dd64b4c87302/archdisch00560-0069-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ac9/1029657/cec5eeab4aa7/archdisch00560-0071-a.jpg

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本文引用的文献

1
Vitamin D is not directly necessary for bone growth and mineralization.
Am J Physiol. 1984 Jun;246(6 Pt 1):E493-8. doi: 10.1152/ajpendo.1984.246.6.E493.
2
Vitamin D-dependent rickets type II: extreme end organ resistance to 1,25-dihydroxy vitamin D3 in a patient without alopecia.II型维生素D依赖性佝偻病:一名无脱发患者对1,25 - 二羟维生素D3的极端终末器官抵抗
Eur J Pediatr. 1986 Oct;145(5):389-95. doi: 10.1007/BF00439245.
3
Long-term nocturnal calcium infusions can cure rickets and promote normal mineralization in hereditary resistance to 1,25-dihydroxyvitamin D.长期夜间输注钙可治愈佝偻病,并促进对1,25 - 二羟维生素D具有遗传性抵抗的患者实现正常矿化。
J Clin Invest. 1986 May;77(5):1661-7. doi: 10.1172/JCI112483.
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Molecular biology of the vitamin D hormone.维生素D激素的分子生物学
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Point mutations in the human vitamin D receptor gene associated with hypocalcemic rickets.与低钙血症性佝偻病相关的人类维生素D受体基因突变
Science. 1988 Dec 23;242(4886):1702-5. doi: 10.1126/science.2849209.
6
Cloning and expression of full-length cDNA encoding human vitamin D receptor.编码人维生素D受体的全长cDNA的克隆与表达。
Proc Natl Acad Sci U S A. 1988 May;85(10):3294-8. doi: 10.1073/pnas.85.10.3294.
7
Absent intestinal response to calciferols in hereditary resistance to 1,25-dihydroxyvitamin D: documentation and effective therapy with high dose intravenous calcium infusions.
J Clin Endocrinol Metab. 1988 Feb;66(2):294-300. doi: 10.1210/jcem-66-2-294.
8
Long-term intracaval calcium infusion therapy in end-organ resistance to 1,25-dihydroxyvitamin D.长期腔内钙输注疗法治疗终末器官对1,25 - 二羟维生素D的抵抗
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9
The role of the vitamin D endocrine system in health and disease.维生素D内分泌系统在健康与疾病中的作用。
N Engl J Med. 1989 Apr 13;320(15):980-91. doi: 10.1056/NEJM198904133201506.
10
Calcium therapy for calcitriol-resistant rickets.
J Pediatr. 1992 Nov;121(5 Pt 1):803-8. doi: 10.1016/s0022-3476(05)81919-5.