Dipartimento di Salute della Donna e del Bambino, Università degli studi di Padova, 35128 Padua, Italy.
Scuola di Formazione Maruzza in Cure Palliative Pediatriche "Maruzza Lyceum", Fondazione Maruzza Lefebvre D'Ovidio Onlus, 00135 Rome, Italy.
Int J Mol Sci. 2018 Oct 8;19(10):3063. doi: 10.3390/ijms19103063.
Mucopolysaccharidosis (MPS) are a group of lysosomal storage disorders that are caused by the deficiency of enzymes involving in the catabolism of glycosaminoglycan (GAGs). GAGs incompletely degraded accumulate in many sites, damaging tissues and cells, leading to a variety of clinical manifestations. Many of these manifestations are painful, but few data are available in the literature concerning the prevalence, etiology, and pathogenesis of pain in children with MPS. This review, through the analysis of the data available the in literature, underscores the relevant prevalence of pain in MPSs' children, provides the instruments to discern the etiopathogenesis of the disease and of pain, illustrates the available molecules for the management of pain and the possible advantages of non-pharmacological pain therapy in MPSs' patients.
黏多糖贮积症（MPS）是一组溶酶体贮积症，由参与糖胺聚糖（GAG）分解代谢的酶缺乏引起。不完全降解的 GAG 在许多部位积累，损害组织和细胞，导致多种临床表现。这些表现中有许多是疼痛，但文献中关于 MPS 儿童疼痛的患病率、病因和发病机制的数据很少。本综述通过分析文献中的现有数据，强调了 MPS 儿童疼痛的相关患病率，提供了识别疾病和疼痛病因发病机制的工具，说明了可用于管理疼痛的分子以及在 MPS 患者中应用非药物性疼痛治疗的可能优势。
