Brenckmann Christine, Papaioannou Alexandra, Freitag Andreas, Hennessey Rosamund, Hansen Suzanne, Ioannidis George, Webber Colin, Adachi Jonathan
Department of Community Health and Epidemiology, Dalhousie University, Halifax, (B3H 4H7), Canada.
BMC Musculoskelet Disord. 2003 Jun 24;4:13. doi: 10.1186/1471-2474-4-13.
Cystic fibrosis is the most common fatal autosomal recessive genetic disease in the Caucasian population. Osteoporosis is increasingly being recognised as an important complication in people with cystic fibrosis.
A descriptive study of adult cystic fibrosis patients receiving care at a Canadian tertiary care hospital was conducted to evaluate the prevalence of osteoporosis, the prevalence of non-vertebral fractures, and the change in bone mineral density during the course of a year. Data on bone mineral density were obtained for 40 adult cystic fibrosis patients by reviewing dual x-ray absorptiometry scans taken at baseline (when annual scans became standard clinical practice) and one year prior to baseline. Data on prevalent fractures were obtained by reviewing all available patient charts. Clinical and laboratory data were collected from an existing clinic database.
Over half of the 40 patients had reduced T- and Z-scores at baseline. For the 27 patients who had data available one year prior to baseline, total hip and lumbar spine bone mineral density had decreased by 3.04% and 0.86% after one year while total body bone mineral density had not changed significantly. Four prior non-vertebral fractures were reported in three patients (1,146 patient-years).
This study confirms that osteoporosis is a significant problem in adult cystic fibrosis patients, and constitutes the first published evidence of cystic fibrosis bone disease in Canadians.
囊性纤维化是白种人群中最常见的致命性常染色体隐性遗传病。骨质疏松症越来越被认为是囊性纤维化患者的一种重要并发症。
对在加拿大一家三级护理医院接受治疗的成年囊性纤维化患者进行了一项描述性研究,以评估骨质疏松症的患病率、非椎体骨折的患病率以及一年内骨矿物质密度的变化。通过回顾在基线时(当年度扫描成为标准临床实践时)和基线前一年进行的双能X线吸收测定扫描,获取了40名成年囊性纤维化患者的骨矿物质密度数据。通过查阅所有可用的患者病历,获取了既往骨折的数据。临床和实验室数据从现有的诊所数据库中收集。
在40名患者中,超过一半在基线时T值和Z值降低。对于在基线前一年有可用数据的27名患者,一年后全髋和腰椎的骨矿物质密度分别下降了3.04%和0.86%,而全身骨矿物质密度没有显著变化。三名患者报告了4例既往非椎体骨折(1146患者年)。
本研究证实骨质疏松症是成年囊性纤维化患者的一个重要问题,并且是加拿大首次发表的关于囊性纤维化骨病的证据。