Titman P, Allwood Z, Gilmour C, Malcolmson C, Duran-Persson C, Cale C, Davies G, Gaspar H, Jones A
Immunology Department, Great Ormond Street Hospital, London, WC1N 3JH, United Kingdom.
J Clin Immunol. 2014 Oct;34(7):844-52. doi: 10.1007/s10875-014-0072-x. Epub 2014 Jul 9.
Primary antibody deficiency disorders (PADs) can have an excellent outlook if diagnosed early and treated appropriately, but require lifelong treatment with immunoglobulin replacement. Some carry risks of inflammatory complications even with optimal treatment. Quality of life (QoL) and the psychological impact of PADs has been relatively little studied, particularly in children. The purpose of this study was to evaluate QoL and psychological impact in a large group of children affected by a range of PADs, as well as a group with transient hypogammaglobulinemia of infancy (THI). Both parental and, where appropriate, child ratings, were collected using standardised questionnaires (PedsQL and SDQ). Higher rates of psychological difficulties, particularly emotional and peer-relationship difficulties were found in children with PAD when compared with healthy controls. Quality of life was poorer than in healthy controls, and also worse than in children affected by diabetes mellitus. Variations in QoL and the degree of psychological difficulties were found between specific diagnostic groups, with children affected by THI being amongst those with the lowest scores for QoL. Further studies are needed to corroborate and extend these findings, but this study confirms previous findings that primary antibody deficiency has a significant impact on quality of life and psychological well-being, and additionally suggests that the impact varies according to severity of the underlying condition. For those with significant difficulties psychological intervention at an early stage may be beneficial.
原发性抗体缺陷疾病(PADs)如果能早期诊断并得到适当治疗,预后可能良好,但需要终生进行免疫球蛋白替代治疗。即使接受最佳治疗,一些患者仍有发生炎症并发症的风险。PADs对生活质量(QoL)和心理的影响相对研究较少,尤其是在儿童中。本研究的目的是评估一大群受多种PADs影响的儿童以及一组患有婴儿期短暂性低丙种球蛋白血症(THI)的儿童的生活质量和心理影响。使用标准化问卷(儿童生活质量量表和优势与困难问卷)收集了父母以及在适当情况下儿童的评分。与健康对照组相比,PADs患儿出现心理问题的发生率更高,尤其是情绪和同伴关系方面的问题。其生活质量比健康对照组差,也比患糖尿病的儿童差。在特定诊断组之间发现了生活质量和心理问题程度的差异,THI患儿的生活质量得分是最低的。需要进一步研究来证实和扩展这些发现,但本研究证实了先前的发现,即原发性抗体缺陷对生活质量和心理健康有重大影响,此外还表明这种影响因潜在疾病的严重程度而异。对于那些有严重困难的患者,早期进行心理干预可能有益。