Increased rate of fractures and severe kyphosis: Sequelae of living into adulthood with cystic fibrosis
Increased rate of fractures and severe kyphosis: Sequelae of living into adulthood with cystic fibrosis
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DOI：
10.7326/0003-4819-128-3-199802010-00004
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发表时间：
1998-02-01
影响因子：
39.2
通讯作者：
Ontjes, DA
中科院分区：
文献类型：
--
作者：
Aris, RM;Renner, JB;Ontjes, DA
背景资料：骨质疏松症发生在囊性纤维化患者，因为他们的年龄，但其临床意义还没有得到很好的界定。目的：确定骨密度降低的临床效果在成人囊性纤维化和评估可能的临床预测骨质疏松症。设计：回顾性队列研究。设置：学术囊性纤维化中心。患者：70名患有晚期囊性纤维化的成年人接受肺移植。采用双能x线吸收法测量骨密度，患者报告的骨折事件通过X线摄影证实，结果：囊性纤维化患者的脊柱、股骨和全身的平均骨密度均严重降低，平均低于年龄匹配的正常对照组2SD（P < 0.001）。患者访谈显示，在1410患者年内发生了54例骨折，胸片显示有14例肋骨和62例椎体压缩性骨折的证据。数据库（涵盖1410患者-年）显示，16 - 34岁女性囊性纤维化患者（P = 0.015）和25 - 45岁男性囊性纤维化患者（P = 0.04）的骨折率约为一般人群的两倍。椎体压缩和肋骨骨折的发生率分别是预期的100倍和10倍（两项比较P < 0.001）。该组的平均后凸角（+/-SD）明显异常（44 ± 14度; 62%大于或等于40度），可能导致身高降低（平均身高降低，囊性纤维化男性为5.8 cm，囊性纤维化女性为5.9 cm）。累积泼尼松剂量，体重指数，青春期年龄是最强的预测骨矿物质density.Conclusions：骨质疏松症是普遍的成人晚期囊性纤维化，其并发症包括骨折率增加和严重的脊柱后凸。
Background: Osteoporosis occurs in patients with cystic fibrosis as they age, but its clinical implications are not well defined.Objective: To determine the clinical effect of decreased bone mineral density in adults with cystic fibrosis and to assess possible clinical predictors of osteoporosis.Design: Retrospective cohort study.Setting: Academic cystic fibrosis center.Patients: 70 adults with late-stage cystic fibrosis who were referred for lung transplantation.Measurements: Bone mineral density was measured with dual-energy x-ray absorptiometry, patient-reported fracture events were confirmed by radiography, and kyphosis angles were measured by using the Cobb method.Results: Mean bone mineral densities for the spine, femur, and total body were severely depressed in patients with cystic fibrosis, averaging 2 SDs below those of age-matched normal controls (P < 0.001). Patient interviews showed that 54 fractures had occurred over 1410 patient-years, and chest radiographs showed evidence of 14 additional rib and 62 additional vertebral compression fractures. The database (which covered 1410 patient-years) showed that fracture rates were approximately twofold greater in women with cystic fibrosis aged 16 to 34 years (P = 0.015) and men with cystic fibrosis aged 25 to 45 years (P = 0.04) than in the general population. Vertebral compression and rib fractures were 100- and 10-fold more common than expected, respectively (P < 0.001 for both comparisons). The mean kyphosis angle (+/-SD) for this group was markedly abnormal (44 +/- 14 degrees; 62% greater than or equal to 40 degrees) and probably contributed to diminished stature (mean height loss, 5.8 cm in men with cystic fibrosis and 5.9 cm in women with cystic fibrosis). Cumulative prednisone dose, body mass index, and age at puberty were the strongest predictors of bone mineral density.Conclusions: Osteoporosis is universal in adults with late-stage cystic fibrosis, and its complications include increased fracture rates and severe kyphosis.