儿童早老症研究进展

Chinese Journal of Applied Clinical Pediatrics(2021)

Cited 0|Views4
No score
Abstract
儿童早老症(HGPS)是一种极为罕见的疾病,以加速衰老为重要特征。该病可累及皮肤、脂肪、心血管、骨骼等多器官系统,发病率为1∶8 000 000~1∶4 000 000。多数患儿仅能活到6~20岁,平均寿命14.6岁。本病临床表现极具特征性,表现为严重生长迟缓、特殊皮肤表现、典型颅面表现等。HGPS预后不良,目前尚无特效疗法。确诊后应对患儿进行长期细致地随访,观察并监测病情的进展,尽可能延长患儿的寿命。现对HGPS的疾病类型、临床表现、发病机制和临床检查进行综述。
More
Translated text
Key words
Hutchinson-Gilford progeria syndrome,LMNA gene ,Presenilin,Farnesyltransferase inhibitors
AI Read Science
Must-Reading Tree
Example
Generate MRT to find the research sequence of this paper
Chat Paper
Summary is being generated by the instructions you defined