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AimsWolfram syndrome (WFS) is a recessively inherited monogenic form of diabetes coexisting with optic atrophy and neurodegenerative disorders with no currently recognized markers of disease progression. The aim of the study was to evaluate retinal parameters by using optical coherence tomography (OCT) in WFS patients after 2 years of follow-up and analysis of the parameters in relation to visual acuity.MethodsOCT parameters and visual acuity were measured in 12 WFS patients and 31 individuals with type 1 diabetes.ResultsTotal thickness of the retinal nerve fiber layer (RNFL), average retinal thickness and total retinal volume decreased in comparison with previous OCT examination. Significant decreases were noted for RNFL (average difference −17.92 µm 95% CI −30.74 to −0.10; p = 0.0157), macular average thickness (average difference −5.38 µm 95% CI −10.63 to −2.36; p = 0.0067) and total retinal volume (average difference −0.15 mm3 95% CI −0.30 to −0.07; p = 0.0070). Central thickness remained unchanged (average difference 1.5 µm 95% CI −7.61 to 10.61; p = 0.71). Visual acuity of WFS patients showed a strong negative correlation with diabetes duration (R = −0.82; p = 0.0010). After division of WFS patients into two groups (with low-vision and blind patients), all OCT parameters except for the RNFL value were lower in blind WFS patients.ConclusionsOCT measures structural parameters and can precede visual acuity loss. The OCT study in WFS patients should be performed longitudinally, and serial retinal examinations may be helpful as a potential end point for future clinical trials.
AimsSome patients with diabetic ketoacidosis develop cerebral edema (CE) in the course of type 1 diabetes mellitus (T1D), which may result in central nervous system disorders and high mortality. The imperfection of existing neuroimaging techniques for early recognition of CE forces us to search for the new and non-invasive methods. The aim of the study was to assess the usefulness of new methods (pachymetry, transorbital ultrasonography—USG, optical coherence tomography—OCT study) in the assessment of the risk of CE occurrence in children with newly diagnosed T1D.MethodsThe study group included 50 children with newly diagnosed T1D, 54 patients with long-term T1D as a reference group and 40 children without glucose tolerance disorders as controls. In all subjects, a corneal thickness (CCT) index with pachymeter, optic nerve sheath diameter (ONSD) using transorbital USG and retinal nerve fiber layer (RNFL) during OCT study were measured and compared with selected clinical parameters of T1D.ResultsIn patients from a study group at onset of T1D, the higher CCT (p < 0.001) and ONSD (p < 0.001) values were observed as compared to the results obtained after 48 h of metabolic compensation. The ONSD correlated negatively with pH value (r = − 0.64; p < 0.001), BE (r = − 0.54, p < 0.001) and HCO3− (r = − 0.50; p < 0.001). A positive correlation between RNFL and Na+ levels (r = 0.47; p < 0.005) was also observed.ConclusionsTransorbital USG and pachymetry may serve as the potential promising methods for the non-invasive assessment of the increased risk of development of CE in patients with T1D.Electronic supplementary materialThe online version of this article (10.1007/s00592-018-1242-8) contains supplementary material, which is available to authorized users.
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