Dental abnormalities in patients with familial hypophosphatemic vitamin D-resistant rickets:: Prevention by early treatment with 1-hydroxyvitamin D

Dental abnormalities in patients with familial hypophosphatemic vitamin D-resistant rickets:: Prevention by early treatment with 1-hydroxyvitamin D
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DOI:
10.1067/mpd.2003.119
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发表时间:
2003-03-01
影响因子:
5.1
通讯作者:
Garabédian, M
Garabédian, M
中科院分区:
医学2区
文献类型:
--
作者:
Chaussain-Miller, C;Sinding, C;Garabédian, M

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目的评价1-羟基维生素D3治疗家族性低磷血症性维生素D抵抗性软骨病的牙科疗效。研究设计48例儿童和成人患者,其中16例青春期前未接受治疗或补充维生素D/25-(OH)D3磷酸盐。年龄较小的32名儿童从婴儿期起就服用了含有1α-(OH)D3的磷酸盐补充剂。所有患者均行临床检查,并拍摄全景片和根尖周片。对龋齿、缺牙或充填牙、龋齿或充填牙的指数和牙髓比率的评估可以与年龄匹配的健康对照组进行比较。结果16名老年患者的牙齿健康状况较差,并存在特征性的牙齿异常。相比之下,32名年轻患者在年龄匹配的健康人群中牙齿状况正常,但他们乳牙上仍有突出的牙髓角,牙髓面积/牙齿面积比增加。结论本次调查显示了1α-(OH)D3治疗对维生素D抵抗性软骨病患者牙齿状况的有利影响,强调了早期治疗的必要性。剩余的缺陷可能是在治疗开始之前,成牙本质细胞和周围的成骨细胞早期暴露于低磷血症,和/或与基因改变相关的内在细胞障碍造成的(S)。
Objective To evaluate the dental effects of 1-hydroxylated vitamin D3 treatment in patients with familial hypophosphatemic vitamin D-resistant rickets.Study design Forty-eight children and adult patients were included in the study; 16 had received no treatment or phosphate supplements with vitamin D/25-(OH) D3 before puberty. The 32 younger ones had received phosphate supplements with 1alpha-(OH)D3 from infancy. All patients were clinically examined, and panoramic and periapical radiographs were made. Evaluations of decayed, missing, or filled teeth and decayed or filled teeth indexes and of pulp ratios allowed comparison with healthy age-matched control patients.Results Poor dental health and characteristic dental anomalies were found in the 16 older patients. In contrast, the 32 younger patients had a normal dental status as regards reference ranges in healthy age-matched populations, although they still showed prominent pulp horns on deciduous teeth and increased pulp area/tooth area ratios.Conclusions This investigation shows the beneficial effects of 1alpha-(OH)D3 treatment on the dental status of vitamin D-resistant rickets patients and emphasizes the necessity of early treatment. Remaining defects may result from early exposure of odontoblasts and surrounding osteoblasts to hypophosphatemia, before the commencement of treatment, and/or from intrinsic cell disturbances linked to the genetic alteration(s).