Physical Medicine and Rehabilitation, Department of Public Health, University Federico II of Naples, Via S. Pansini, 5, 80131, Naples, Italy.
Orphanet J Rare Dis. 2022 Jul 8;17(1):255. doi: 10.1186/s13023-022-02402-w.
Pain of musculoskeletal origin is very common in young patients affected by Mucopolysaccharidoses. This scoping review evaluates the evidence for assessment, pharmacological treatment and rehabilitation management for musculoskeletal pain of the latter.
A Medline search through PubMed has been performed for studies published in English at least for the past twenty years. Two investigators independently reviewed all search results and extracted those that met the inclusion criteria.
29 studies have been selected and analysed in depth, of which 10 related to pain assessment, 11 concerned pharmacological approach, and 8 reported rehabilitation approaches.
Few data are available in literature concerning the classification and management of pain in children with Mucopolysaccharidoses. Notwithstanding, pain evaluation methods are effectively used to classify pain intensity, according to the age group and communication abilities of young Mucopolysaccharidoses patients. The review emphasizes that drug therapies have a palliative purpose, while rehabilitation reduces musculoskeletal pain and can provide a therapeutic effect on disabilities.
患有黏多糖贮积症的年轻患者常出现肌肉骨骼源性疼痛。本范围性综述评估了黏多糖贮积症患者肌肉骨骼疼痛的评估、药物治疗和康复管理的证据。
通过 PubMed 对至少过去 20 年发表的英文研究进行了 Medline 检索。两位研究者独立审查了所有检索结果,并提取了符合纳入标准的研究。
选择并深入分析了 29 项研究,其中 10 项与疼痛评估有关,11 项涉及药物治疗方法,8 项报告了康复方法。
文献中关于黏多糖贮积症儿童疼痛的分类和管理的数据很少。尽管如此,疼痛评估方法可有效地用于根据黏多糖贮积症患儿的年龄组和沟通能力来对疼痛强度进行分类。该综述强调,药物治疗具有缓解疼痛的目的,而康复可以减轻肌肉骨骼疼痛,并对残疾产生治疗效果。