读书报告Sagliker综合征.pptVIP

SAGLIKER SYNDROME HISTORY In the XX century ugly facial changes with leontiasis ossea as an extremely rare form of renal osteodystrophy have been occasionally reported in the literature Sagliker and his co-workers have demon-trated that in the XXI century, despite great advances in medicine it is still possible to meet patients with ugly face appearances and other disturbances of the skeletal system, which emerged in the course of untreated or inadequately treated sHPT. Their efforts in looking for such patients resuited in 2004 in a creation of a new syndrome一Sagliker syndrome (SS), which is defined as uglifying face appearance in severe and late sHPT in the course of chronic renal failure. Incidence of SS in hemodialysis (HD) patients was evaluated to be approximately 0.5%. In 2008, Sagliker et al.have encountered 40 cases of SS by performing an international study in Turkey,India, Romania, Egypt, Maleysia, Tunis, and China. J Ren Nutr. 2012 Jan;22(1):157-61. DEVELOPMENT In 2006, abnormalities in cephalometric evaluation (malocclusion of the upper and lower jaws) and neurologic manifestations (the most frequently headache, polyneuropathy, cranial neuropathy, fatique)in patients with SS were presented. Recently (2008-2010), quality of life ，psychiatric manifestations (depression, hopeles-sness) and audiological findings (hearing loss) were described in these patients. J Ren Nutr. 2012 Jan;22(1):157-61. MANIFESTATION Sagliker综合征患者男/女比例为0.6∶1、平均年龄为44.38岁、平均透析龄为145月,单纯SHPT患者平均年龄为51.15岁、男/女比例为1∶1、平均透析龄为125月,2组患者在性别、年龄均值和透析龄均值方面有统计学差异(P＜0.05); 中国血液净化 2012, 11( 07):352-356. MANIFESTATION Sagliker综合征患者的ALP均值为1184U/L、全段甲状旁腺激素(iPTH)均值为2109pg/ml,单纯SHPT患者的ALP为491U/L、iPTH为2371pg/ml,2组患者在碱性磷酸酶(ALP)均值上有显著统计学差异(P＜0.01),在iPTH均值上没有统计学差异(P＞0.05 中国血液净化 2012, 11( 07):352-356 MANIFESTATION 头影测量结果显示Sagliker综合征患者,上颌骨整体体积变大,矢状方向和垂直方向明显增大;下颌骨前部垂直方向明显增加,其余部分没有明显变化;软组织变化和颌骨变化基本一致;牙齿未出现明显的代偿性移位. 中国血液净化 2012, 11( 07):352-356 MANIFESTATION 性别,年龄、透析龄等因素可能是Sagliker综合征的危险因素 Sagli