Ophthalmology Department, 9342Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Eur J Ophthalmol. 2022 Nov;32(6):3358-3362. doi: 10.1177/11206721221078649. Epub 2022 Feb 7.
The aim of this study was to evaluate the tear film in infantile nephropathic cystinosis patients with corneal crystals treated with topical viscous cysteamine hydrochloride (Cystadrops).
Ten eyes of five patients with nephropathic cystinosis aged from 10 to 35 years were included in this study. The patients were under treatment with viscous cysteamine hydrochloride formulation containing 3.8 mg/mL cysteamine (vCH 0.55%, equivalent to 0.55% CH; Cystadrops; Recordati rare Diseases, Puteaux, France) to reduce corneal crystal density. Five age and sex matched individuals were randomly selected as control group. Tear osmolarity testing (TearLab) was performed to assess the osmolarity of patients under treatment and compared to control group values. Tear film break-up time (TBUT) and basic tear secretion (Schirmer test) were also assessed.
Mean tear osmolarity was 294.8 mOsms/L (±10.4), with a mean absolute difference of 1.85 mOsms/L (±2.13) between the eyes. There was no statistically significant difference between the osmolarity readings of cystinosis and the control group (294.8 ± 10.4 vs 299.4 ± 6.2mOsm/L, respectively; p = 0.39). The mean TBUT was 10.2 ± 0.83 s in the study group versus 10 ± 0.7 s in controls (p = 0.62). The mean Schirmer test score was 9.2 ± 0.83 mm in the patients versus 10.2 ± 0.83 mm in the controls (p = 0.14).
The TearLab osmolarity system test showed good reliability and precision in repeated measurements. This is the first report using the TearLab osmolarity system to assess tear film in patients with cystinosis treated with vCH 0.55%. TearLab examination showed that the use of vCH 0.55% drops does not determine alterations of the tear film quality.
本研究旨在评估接受局部粘性盐酸半胱氨酸（Cystadrops）治疗的婴儿胱氨酸贮积症患者的角膜晶体的泪膜。
本研究纳入了 5 名年龄在 10 至 35 岁的胱氨酸贮积症患者的 10 只眼。这些患者正在接受 3.8mg/ml 半胱氨酸（vCH 0.55%，相当于 0.55% CH；Cystadrops；Recordati 罕见病公司，法国普瓦捷）粘性制剂治疗，以降低角膜晶体密度。随机选择 5 名年龄和性别匹配的个体作为对照组。使用 TearLab 进行泪液渗透压测试（TearLab），以评估治疗患者的渗透压，并与对照组的值进行比较。还评估了泪膜破裂时间（TBUT）和基础泪液分泌（Schirmer 测试）。
平均泪液渗透压为 294.8 mOsms/L（±10.4），双眼之间的平均绝对差异为 1.85 mOsms/L（±2.13）。胱氨酸贮积症和对照组的渗透压读数之间无统计学显著差异（分别为 294.8±10.4 和 299.4±6.2mOsm/L；p=0.39）。研究组的平均 TBUT 为 10.2±0.83 s，对照组为 10±0.7 s（p=0.62）。患者的平均 Schirmer 测试评分 9.2±0.83mm，对照组为 10.2±0.83mm（p=0.14）。
TearLab 渗透压系统测试在重复测量中显示出良好的可靠性和精度。这是首次使用 TearLab 渗透压系统评估接受 0.55%vCH 治疗的胱氨酸贮积症患者的泪膜。TearLab 检查表明，使用 0.55%vCH 滴眼液不会导致泪膜质量发生变化。
