Alex George, Catto-Smith Anthony G, Ditchfield Michael, Roseby Robert, Robinson Philip J, Cameron Fergus J, Oliver Mark R
Department of Gastroenterology and Nutrition, Royal Children's Hospital, Melbourne, Australia.
Pediatr Pulmonol. 2006 Apr;41(4):338-44. doi: 10.1002/ppul.20337.
In order to assess the effects of significant cystic fibrosis-related liver disease (CFLD) on bone health, we compared the bone mineral status of older children and adolescents with CFLD to those with cystic fibrosis (CF) alone. Thirteen children (age range, 10-19 years) from our clinical CF services were identified with significant CFLD (9 of these 13 patients had clinical and radiological evidence of portal hypertension). This cohort was then matched by age, gender, and anthropometric measurements with equal numbers of patients with CF alone. All patients had a dual-energy X-ray absorptiometry (DEXA) scan to determine bone mineral content (BMC), bone area (BA), bone mineral density (BMD), and bone mineral apparent density (BMAD) in the region of the lumbar spine. Blood was drawn to determine serum vitamin A, D, E, and K status and liver function tests. The best forced expired volume in 1 sec (FEV1) for each patient in the 12 months around the time of the scan was also documented. Patients with CFLD had slightly worse FEV1 (82 +/- 20% vs. 91 +/- 16%, P = 0.05) and significantly higher alanine aminotransferase (65.5 +/- 35 IU/l vs. 30 +/- 20 IU/l, P = 0.01) than those with CF alone. The mean lumbar spine BA, BMC, BMD, and BMAD were not different between children with CFLD and CF. In conclusion, the presence of significant liver disease in children with CF does not appear to be an additional risk factor for the development of abnormal bone mineralization.
为了评估严重的囊性纤维化相关肝病（CFLD）对骨骼健康的影响，我们将患有CFLD的大龄儿童和青少年的骨矿物质状况与仅患有囊性纤维化（CF）的儿童和青少年进行了比较。我们从临床CF服务中确定了13名患有严重CFLD的儿童（年龄范围为10 - 19岁）（这13名患者中有9名有门静脉高压的临床和放射学证据）。然后根据年龄、性别和人体测量数据，将该队列与相同数量的仅患有CF的患者进行匹配。所有患者均接受了双能X线吸收法（DEXA）扫描，以确定腰椎区域的骨矿物质含量（BMC）、骨面积（BA）、骨矿物质密度（BMD）和骨矿物质表观密度（BMAD）。采集血液以测定血清维生素A、D、E和K水平以及肝功能测试。还记录了扫描前后12个月内每位患者的最佳1秒用力呼气量（FEV1）。与仅患有CF的患者相比，患有CFLD的患者FEV1略差（82±20%对91±16%，P = 0.05），丙氨酸转氨酶显著更高（65.5±35 IU/l对30±20 IU/l，P = 0.01）。患有CFLD和CF的儿童之间，腰椎的平均BA、BMC、BMD和BMAD没有差异。总之，CF患儿中存在严重肝病似乎并不是骨矿化异常发展的额外危险因素。
