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良性人类骨硬化症中骨代谢的生化标志物:两种类型在基线及三碘甲状腺原氨酸刺激期间的研究

Biochemical markers of bone metabolism in benign human osteopetrosis: a study of two types at baseline and during stimulation with triiodothyronine.

作者信息

Bollerslev J, Ueland T, Grodum E, Haug E, Brixen K, Djøseland O

机构信息

Department of Medical Endocrinology, National University Hospital, Oslo, Norway.

出版信息

Eur J Endocrinol. 1998 Jul;139(1):29-35. doi: 10.1530/eje.0.1390029.

DOI:10.1530/eje.0.1390029
PMID:9703375
Abstract

Biochemical markers of bone remodelling were used to evaluate bone turnover in two types of autosomal dominant osteopetrosis (ADO) at baseline and during stimulation with triiodothyronine (T3). Eight patients with Type I (aged 23-61 years, mean 40.4 years) and nine patients with Type II ADO (aged 20-49 years, mean 32.8 years) were compared with 10 normal controls (aged 22-54 years, mean 35.4 years). The participants were treated with 100 microg T3 daily for 7 days and followed for a total of 16 weeks. Serum concentrations of T3 increased and corresponding suppression of TSH was observed in all participants. Both formative and resorptive bone markers were normal at baseline. After stimulation with T3, a significant increase was seen in all groups for the formative markers used. Secondary increments were observed at the end of the observation period for all groups, indicating activation of bone remodelling. A variety of resorptive markers was assessed, but no differences between patients and controls were seen. After stimulation, highly significant responses were found in all parameters in all groups, in accordance with stimulation of existing resorptive cells. However, no secondary increments were seen at the end of the observation period. A more pronounced response was found in crosslinks-related assays. The study demonstrates that it is possible to stimulate bone resorptive and formative cells with thyroid hormones in both types of ADO. Moreover, it indicates that the remodelling process is activated by a short course of T3 treatment.

摘要

骨重塑的生化标志物被用于评估两种常染色体显性遗传性骨硬化症(ADO)在基线时以及用三碘甲状腺原氨酸(T3)刺激期间的骨转换情况。将8例I型患者(年龄23 - 61岁,平均40.4岁)和9例II型ADO患者(年龄20 - 49岁,平均32.8岁)与10名正常对照者(年龄22 - 54岁,平均35.4岁)进行比较。参与者每天接受100微克T3治疗,为期7天,总共随访16周。所有参与者血清T3浓度均升高,且观察到相应的促甲状腺激素抑制。在基线时,成骨和破骨骨标志物均正常。用T3刺激后,所有组中所使用的成骨标志物均显著增加。在观察期结束时,所有组均观察到二次升高,表明骨重塑被激活。评估了多种破骨标志物,但患者与对照之间未见差异。刺激后,所有组的所有参数均出现高度显著的反应,这与现有破骨细胞的刺激情况一致。然而,在观察期结束时未见二次升高。在与交联相关的检测中发现了更明显的反应。该研究表明,在两种类型的ADO中,用甲状腺激素刺激骨吸收和成骨细胞是可行的。此外,这表明短期T3治疗可激活重塑过程。

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引用本文的文献

1
Osteoclasts from patients with autosomal dominant osteopetrosis type I caused by a T253I mutation in low-density lipoprotein receptor-related protein 5 are normal in vitro, but have decreased resorption capacity in vivo.由低密度脂蛋白受体相关蛋白5中的T253I突变引起的常染色体显性I型骨硬化症患者的破骨细胞在体外是正常的,但在体内的吸收能力下降。
Am J Pathol. 2005 Nov;167(5):1341-8. doi: 10.1016/S0002-9440(10)61221-7.