原发性肥大性骨关节病临床与基础研究进展  

Clinical and basic research progress in primary hypertrophic osteoarthropathy

作　　者：张增[1,2] 章振林[1] 

机构地区：[1]上海交通大学附属第六人民医院骨质疏松和骨病科和骨代谢病遗传研究室,上海200233 [2]上海交通大学附属第六人民医院骨科

出　　处：《中华骨质疏松和骨矿盐疾病杂志》2014年第4期293-297,共5页Chinese Journal Of Osteoporosis And Bone Mineral Research

基　　金：科技部973重大研究项目(2014CB942903);国家自然科学基金(81370978);上海市科委重点项目(14JC1405000)

摘　　要：肥大性骨关节病(HO)是以杵状指和长骨骨膜增生为特征的骨关节病综合征,按病因不同分为原发性(PHO)和继发性(SHO)2个亚型。人类对该疾病的认识可以追溯到希波克拉底时代,但该病发病机制至今尚不十分明确。对PHO家系的遗传学研究为认识HO发病机制提供了机会。随着PHO致病基因的发现,PHO的发病机制业已明确,即前列腺素降解障碍导致循环和局部微环境前列腺素E2(PGE2)水平显著增高。SHO同样由前列腺素代谢障碍所致。本文就PHO临床表现及其发病机制的研究进展和治疗手段的革新作一综述。Hypertrophic osteoarthropathy（ HO） is an ancient syndrome characterized by digital clubbing,periostosis and pachydermia. According to the etiology,it can be divided into two subtypes： primary hypertrophic osteoarthropathy（ PHO） and secondary hypertrophic osteoarthropathy（ SHO）. However,the pathogenesis of HO has hitherto been poorly understood. The genetic study of PHO pedigrees provide an opportunity to understand the pathogenesis of HO. With the identification of the causative genes of PHO,it is believed that increased levels of prostaglandin E2 due to the failure of its degradation are involved in the pathogenesis of PHO. Meanwhile,there is evidence that SHO is also caused by impaired metabolism of prostaglandins. This review will mainly focus on the clinical manifestations of PHO and recent research advances on its pathogenesis. Additionally,treatment innovation promoted by the recent breakthrough on the pathogenesis is discussed. At last,several questions which deserve further investigation are raised.

关 键 词：杵状指 肥大性骨关节病 前列腺素E2 

分 类 号：R596[医药卫生—内科学]