Department of Pediatrics, Jósa András County Hospital, Nyíregyháza, Hungary.
Council for Nutritional and Environmental Medicine, Mo i Rana, Norway.
Int J Rheum Dis. 2019 Sep;22(9):1762-1767. doi: 10.1111/1756-185X.13639. Epub 2019 Aug 16.
Human α-fucosidase (EC 3.2.1.51) is a hydrolase the importance of which has been increasing in the latest years. However, data about its plasma level in children with autoimmune disorders, particularly Sjögren's syndrome (SS), are lacking. In this study, the plasma activity of L-α-fucosidase-1 (α-L-FUCA-1) was assayed in hospitalized children and adults and its association with SS and other rheumatic disorders further evaluated.
In total 73 Hungarian hospitalized patients, 32 children (2.5-10 years) and 41 adults (32-68 years), were enrolled in the study and underwent plasma assay of α-L-FUCA1 activity. Linear regression, Durbin-Watson (DW), and Pearson tests were evaluated to investigate the relationship between α-L-FUCA-1 plasma levels and autoimmune manifestations.
α-L-FUCA-1 correlated with SS both in children (2-sided t test, P = 0.0023) and in adults (2-sided t test, P = 0.00035). Linear regressions showed that in other rheumatic disorders, α-L-FUCA1 did not show any differential distribution related to the particular pathology (r = 0.2042, P = 0.1531, DW test = 2.2139 positive), while this trend was radically opposite for patients with SS (r = 0.1462, P = 0.0032, DW test = 1.3664, negative).
Alterations in plasma level of α-L-FUCA-1 were significantly associated with SS. This preliminary result should encourage further research on α-L-FUCA-1 as a possible differential serological marker of SS.
人类 α-岩藻糖苷酶(EC 3.2.1.51)是一种水解酶,其重要性近年来一直在增加。然而,关于其在自身免疫性疾病(尤其是干燥综合征[SS])患儿血浆水平的数据尚缺乏。在这项研究中,测定了住院患儿和成人的 L-α-岩藻糖苷酶-1(α-L-FUCA-1)血浆活性,并进一步评估了其与 SS 和其他风湿性疾病的关系。
共纳入 73 名匈牙利住院患者,其中 32 名儿童(2.5-10 岁)和 41 名成人(32-68 岁),进行了α-L-FUCA1 活性的血浆测定。采用线性回归、杜宾-沃森(Durbin-Watson,DW)和皮尔逊检验评估α-L-FUCA-1 血浆水平与自身免疫表现之间的关系。
α-L-FUCA-1 在儿童(双侧 t 检验,P=0.0023)和成人(双侧 t 检验,P=0.00035)中均与 SS 相关。线性回归显示,在其他风湿性疾病中,α-L-FUCA1 与特定病理无差异分布相关(r=0.2042,P=0.1531,DW 检验=2.2139 阳性),而 SS 患者则截然相反(r=0.1462,P=0.0032,DW 检验=1.3664,阴性)。
α-L-FUCA-1 血浆水平的改变与 SS 显著相关。这一初步结果应鼓励进一步研究 α-L-FUCA-1 作为 SS 可能的差异血清学标志物。