Osteoporosis in children with severe congenital neutropenia:: Bone mineral density and treatment with bisphosphonates
Osteoporosis in children with severe congenital neutropenia:: Bone mineral density and treatment with bisphosphonates
复制标题
DOI：
10.1097/01.mph.0000210409.48877.c3
复制
发表时间：
2006-04-01
影响因子：
1.2
通讯作者：
Cavieres, Mirta
中科院分区：
文献类型：
--
作者：
Borzutzky, Arturo;Reyes, Maria Loreto;Cavieres, Mirta
在严重的先天性中性粒细胞减少症(SCN)患者中，骨密度降低的发生率很高。本研究的目的是描述粒细胞集落刺激因子治疗SCN儿童的骨密度变化，并评价双磷酸盐对这两种骨质疏松症儿童的治疗效果。一项前瞻性开放研究评估了9名患有SCN的智利患者的骨密度和代谢，对骨质疏松患者使用双膦酸类药物。术后随访7个月至3.5年。9名患者中有6名在最初评估时骨密度降低：3名患有骨质疏松症(z评分-2)，3名患者有骨量减少(z评分-1)。4名儿童出现脊椎骨折。两名患者在无临床症状的随访中出现骨量减少。5名患者接受了双磷酸盐治疗，增加了他们的BMD z评分(平均增加1.2分。范围为0.27到2.62)。4例骨质疏松症患者羟脯氨酸/肌酐比值升高，治疗过程中Z评分下降(平均下降2.18，范围1.56~2.53)。4例患者在治疗过程中对骨折椎体进行了重塑和再扩张。随访期间未见双膦酸类药物的副作用。骨质疏松是SCN患者的重要共病，可能是由于骨吸收增加所致。双膦酸盐似乎是治疗这些患者骨质疏松症的有效方法。
A high incidence of decreased bone mineral density (BMD) has been described in patients with severe congenital neutropenia (SCN). The objectives of the study are to describe changes in BMD in children with SCN treated with granulocyte colony-stimulating factor and evaluate the response to treatment with bisphosphonates in those two had osteoporosis. A prospective open-label study was performed evaluating BMD and metabolism in 9 Chilean patients with SCN, administrating bisphosphonates in those with osteoporosis. Follow-up ranged between 7 months and 3.5 years. Six out of 9 patients had reduced BMD on initial assessment: 3 had osteoporosis (z score < - 2) and 3, had osteopenia (z score < - 1). Four children presented vertebral fractures. Two presented osteopenia on follow-up without Clinical Symptoms. Five patients were treated with biphosphonates, increasing their BMD z Score (mean increase 1.2. range 0.27 to 2.62). z Score of hydroxyproline/ creatinine ratios, which was elevated in 4 patients with osteoperosis, decreases during treatment (mean decrease 2.18, range 1.56 to 2.53). Four patients remodeled and reexpanded fractured vertebrae during treatment. No side effects of bisphosphonate were seen on follow-up. Osteoporosis is an important comorbidity in SCN patients probably due to an increased bone resorption. Bisphosphonates seem to be an effective treatment for osteoporosis in these patients.