Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease.
Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease.
复制标题
DOI：
10.1038/s41598-021-87589-x
复制
发表时间：
2021-04-14
影响因子：
4.6
通讯作者：
Malik RA
中科院分区：
文献类型：
--
作者：
Bitirgen G;Turkmen K;Zengin N;Malik RA
自主神经功能障碍的症状在法布里病中很常见。在这项研究中，我们旨在评估Fabry病患者对白光刺激的瞳孔反应的变化及其与自主神经症状的严重程度的关系。连续14名Fabry病患者和14名健康对照参与者参加了这项横断面研究。采用美因茨严重程度评分指数(MSSI)评定Fabry病的严重程度，采用综合自主神经症状量表(COMPASS 31)评定自主神经症状的严重程度。用红外线动态瞳孔测量仪评估瞳孔光反应。与对照组相比，法布里病患者的瞳孔收缩幅度(P = 0.048)、持续时间(P = 0.048)和散瞳潜伏期(P = 0.048)均显著缩短。加权COMPASS总分与MSSI(r = 0.592；P = 0.026)和散瞳时间(ρ = 0.561；P = 0.037)相关。COMPASE 31的瞳孔运动加权亚分与瞳孔收缩持续时间(r = − 0.600；P = 0.023)和散瞳潜伏期(ρ = − 0.541；P = 0.046)呈负相关，与散瞳持续时间(ρ = 0.877；P <； 0.001)和MSSI(r = 0.533；P = 0.049)呈正相关。综上所述，Fabry病患者存在瞳孔功能异常，这与自主神经症状的严重程度有关。
Symptoms of autonomic dysfunction are common in Fabry disease. In this study we aimed to evaluate alterations in the pupillary response to white light stimulation in patients with Fabry disease and their association with the severity of autonomic symptoms. Fourteen consecutive patients with Fabry disease and 14 healthy control participants were enrolled in this cross-sectional study. The Mainz Severity Score Index (MSSI) was used to measure the severity of Fabry disease and the Composite Autonomic Symptom Scale 31 (COMPASS 31) questionnaire was used to evaluate the severity of autonomic symptoms. The pupil light responses were assessed with an infrared dynamic pupillometry unit. There were significant reductions in the amplitude (P = 0.048) and duration (P = 0.048) of pupil contraction, and the latency of pupil dilation (P = 0.048) in patients with Fabry disease compared to control subjects. The total weighted COMPASS 31 score correlated with MSSI (r = 0.592; P = 0.026) and the duration of pupil dilation (ρ = 0.561; P = 0.037). The pupillomotor weighted sub-score of the COMPASS 31 correlated inversely with the duration of pupil contraction (r = − 0.600; P = 0.023) and latency of pupil dilation (ρ = − 0.541; P = 0.046), and directly with the duration of pupil dilation (ρ = 0.877; P < 0.001) and MSSI (r = 0.533; P = 0.049). In conclusion, abnormal pupillary function is demonstrated in patients with Fabry disease, which is associated with the severity of autonomic symptoms.
登录
查看更多内容
影响因子：
2.2
作者：
Giza, Evangelia;Fotiou, Dimitrios;Karlovasitou, Anna
通讯作者：
Karlovasitou, Anna
影响因子：
8
作者：
Ries, Markus;Clarke, Joe T. R.;Schiffmann, Raphael
通讯作者：
Schiffmann, Raphael
影响因子：
3.7
作者：
Kim Y;Seok JM;Park J;Kim KH;Min JH;Cho JW;Park S;Kim HJ;Kim BJ;Youn J
通讯作者：
Youn J
影响因子：
3.5
作者：
Greco, C.;Di Gennaro, F.;Spallone, V.
通讯作者：
Spallone, V.
影响因子：
2.6
作者：
Biegstraaten, Marieke;van Schaik, Ivo N.;Hollak, Carla E. M.
通讯作者：
Hollak, Carla E. M.