RECOMBINANT HUMAN INTERFERON-GAMMA THERAPY FOR OSTEOPETROSIS

RECOMBINANT HUMAN INTERFERON-GAMMA THERAPY FOR OSTEOPETROSIS
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DOI:
10.1016/s0022-3476(05)82557-0
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发表时间:
1992-07-01
影响因子:
5.1
通讯作者:
HATCHER, HC
HATCHER, HC
中科院分区:
医学2区
文献类型:
--
作者:
KEY, LL;RIES, WL;HATCHER, HC

文献摘要

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在先天性骨质疏松症患者中已经证实了白细胞超氧化物形成的缺陷。这种白细胞缺陷似乎与骨吸收缺陷有关。由于重组人干扰素γ治疗可提高慢性肉芽肿病患者的超氧化物产生,我们试图确定类似的策略是否可以逆转骨质疏松症。对8例骨质疏松患者皮下注射γ干扰素,1.5 μ g/kg,每周3次,连续6个月。治疗期间尿羟脯氨酸和尿钙排泄量显著增加,同时小梁骨体积显著减少。骨髓扫描显示骨髓生成增加。血红蛋白浓度、血小板计数和白细胞生成超氧化物显著增加。治疗期间未发生严重感染。这些数据表明,干扰素γ能增强骨质疏松患者的骨吸收和白细胞功能。
A defect in leukocytic superoxide formation has been demonstrated in patients with congenital osteopetrosis. This leukocyte defect appears to be related to defective bone resorption. Because recombinant human interferon gamma therapy enhances superoxide production in patients with chronic granulomatous disease, we sought to determine whether a similar strategy could reverse the osteopetrotic condition. Interferon gamma, 1.5-mu-g/kg three times a week, was administered by subcutaneous injection for 6 months to eight patients with osteopetrosis. Urinary hydroxyproline and urinary calcium excretion increased markedly during therapy in parallel with a significant decrease in trabecular bone volume. Bone marrow scans demonstrated increased bone marrow production. The hemoglobin concentration, platelet count, and leukocyte production of superoxide increased significantly. No serious infections were encountered during the therapy. These data suggest that interferon gamma administration enhances bone resorption and leukocyte function in patients with osteopetrosis.