Congenital aniridia is a panocular disorder that is typically characterized by iris hypoplasia and aniridia-associated keratopathy (AAK). AAK results in the progressive loss of corneal transparency and thereby loss of vision. Currently, there is no approved therapy to delay or prevent its progression, and clinical management is challenging because of phenotypic variability and high risk of complications after interventions; however, new insights into the molecular pathogenesis of AAK may help improve its management. Here, we review the current understanding about the pathogenesis and management of AAK. We highlight the biological mechanisms involved in AAK development with the aim to develop future treatment options, including surgical, pharmacological, cell therapies, and gene therapies. 相似文献
目的 分析适量运动对于心房颤动(房颤)患者的运动能力以及远期临床预后的影响。方法 通过检索中国知网,万方,维普,Pubmed,OVID,Cochrane Central Register of Controlled Trials (CENTRAL),web of science数据库,纳入对房颤患者进行适量体育活动干预的临床试验。本研究的主要终点为静息心率,最大心率,6 min步行试验,最大运动功率,全因死亡率以及卒中发生率,用以评估适量运动对房颤患者活动耐力以及预后的影响。结果 本研究共纳入7项试验,2 452例患者,试验组为适量运动干预组,对照组为不活跃组。适量运动并不会显著增加患者的静息心率(MD=-1.68,P=0.70)以及最大心率(RD=9.72,P=0.11)。运动训练可显著提高房颤患者的运动能力,明显增加6 min步行距离(MD=59.07,95%CI=11.70-106.44,P<0.05),并且在一定程度上提高运动功率(MD=17.96,95%CI=-6.30-42.22,P=0.15)。适量运动对房颤患者的远期预后不会造成不良影响,适量运动组对比不活跃组,全因死亡率为15.7% vs 14.2%(RD=0.03,95%CI=-0.18-0.25,P=0.75);卒中发生率5.0% vs 2.9%(RD=0.02,95%CI=-0.06-0.09,P=0.69),两组差异无统计学意义。结论 适量运动可在一定程度上提高房颤患的活动耐力,且不增加卒中以及全因死亡率。 相似文献