关键词: Achondroplasia Acute pain Chronic pain European reference network Skeletal dysplasia

Mesh : Adolescent Humans Child Adult Quality of Life Chronic Pain Achondroplasia / complications epidemiology

来  源:   DOI:10.1016/j.ejmg.2023.104850

Abstract:
The clinical features of achondroplasia can cause acute self-limited pain that can evolve into chronic pain. Pain causes a low quality of life, in terms of physical, emotional, social, and school functioning in both adult and children with achondroplasia. We conducted a systematic review according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) statement to describe prevalence, assessment tools, causes and management strategies of pain in this rare disease. We found that shoulder and knee pain is typically referred during infancy, while knee pain is generally referred around 5-6 years of age. The prevalence of general pain in adolescence can be as high as 90%. Chronic pain in the achondroplasia population increases with age, with up to 70% of adults reporting general pain and back pain. Recognizing the multiple determinants of acute and chronic pain in patients with achondroplasia may enable physicians to better understand and manage this burden, particularly with the advent of new drugs that may modify some of the striking features of achondroplasia.
摘要:
软骨发育不全的临床特征可引起急性自限性疼痛,可演变成慢性疼痛。疼痛导致生活质量低下,在物理方面,情感,社会,以及成人和儿童软骨发育不全的学校功能。我们根据系统评价和荟萃分析(PRISMA)声明的首选报告项目进行了系统评价,以描述患病率。评估工具,这种罕见疾病疼痛的原因和管理策略。我们发现肩部和膝盖疼痛通常在婴儿期出现,而膝关节疼痛通常在5-6岁左右。青春期一般疼痛的患病率可高达90%。软骨发育不全人群的慢性疼痛随着年龄的增长而增加,多达70%的成年人报告一般疼痛和背痛。认识到软骨发育不全患者急性和慢性疼痛的多种决定因素可能使医生更好地理解和管理这种负担,特别是随着新药物的出现,这些药物可能会改变软骨发育不全的一些显著特征。
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