Feldman Estée C H, Hivick Daniel P, Slepian P Maxwell, Tran Susan T, Chopra Pradeep, Greenley Rachel Neff
Department of Psychology, Rosalind Franklin University of Medicine and Science, North Chicago, IL 60064, USA.
Chicago Medical School, Rosalind Franklin University of Medicine and Science, North Chicago, IL 60064, USA.
Children (Basel). 2020 Sep 21;7(9):146. doi: 10.3390/children7090146.
Ehlers-Danlos syndromes (EDS) are a group of connective tissue disorders that manifest with hyperextensibility of joints and skin, and general tissue fragility. While not a major criterion for clinical diagnosis, pain is a frequently endorsed symptom across subtypes of EDS. As such, the present review aims to summarize research to date on pain characteristics and management, and the relationship between such pain symptomatology and quality of life in pediatric EDS. Characteristics of pain, including theorized etiology, relative intensity and extent of pain are described, as well as descriptions of frequently endorsed pain sites (musculoskeletal, and non-musculoskeletal). Interventions related to the management of musculoskeletal (e.g., pharmaceutical intervention, physical therapy) and non-musculoskeletal pain (e.g., pharmaceutical and psychological interventions) are discussed, highlighting the need for additional research related to pediatric pain management in the context of hypermobility syndromes. In addition, the relationship between pain in pediatric EDS and quality of life is described. Finally, limitations of literature to date are described and recommendations for future lines of research are outlined.
埃勒斯-当洛综合征（EDS）是一组结缔组织疾病，表现为关节和皮肤过度伸展以及组织普遍脆弱。虽然疼痛并非临床诊断的主要标准，但它是EDS各亚型中常见的症状。因此，本综述旨在总结迄今为止关于疼痛特征与管理以及小儿EDS疼痛症状与生活质量之间关系的研究。文中描述了疼痛的特征，包括理论病因、相对强度和疼痛程度，以及常见疼痛部位（肌肉骨骼和非肌肉骨骼）的描述。讨论了与肌肉骨骼疼痛（如药物干预、物理治疗）和非肌肉骨骼疼痛管理（如药物和心理干预）相关的干预措施，强调了在关节过度活动综合征背景下开展更多小儿疼痛管理相关研究的必要性。此外，还描述了小儿EDS疼痛与生活质量之间的关系。最后，阐述了现有文献的局限性，并概述了未来研究方向的建议。
