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Radiomics features (RFs) serve as quantitative metrics to characterize shape, density/intensity, and texture patterns in radiological images. Despite their promise, RFs exhibit reproducibility challenges across acquisition settings, thus limiting implementation into clinical practice. In this investigation, we evaluate the effects of different CT scanners and CT acquisition protocols (KV, mA, field-of-view, and reconstruction kernel settings) on RFs extracted from lumbar vertebrae of a cadaveric trunk. Employing univariate and multivariate Generalized Linear Models (GLM), we evaluated the impact of each acquisition parameter on RFs. Our findings indicate that variations in mA had negligible effects on RFs, while alterations in kV resulted in exponential changes in several RFs, notably First Order (94.4%), GLCM (87.5%), and NGTDM (100%). Moreover, we demonstrated that a tailored GLM model was superior to the ComBat algorithm in harmonizing CT images. GLM achieved R2 > 0.90 in 21 RFs (19.6%), contrasting ComBat's mean R2 above 0.90 in only 1 RF (0.9%). This pioneering study unveils the effects of CT acquisition parameters on bone RFs in cadaveric specimens, highlighting significant variations across parameters and scanner datasets. The proposed GLM model presents a robust solution for mitigating these differences, potentially advancing harmonization efforts in Radiomics-based studies across diverse CT protocols and vendors., (© 2024. The Author(s).)

Quantitative muscle MRI (water-T2 and fat mapping) is being increasingly used to assess disease involvement in muscle disorders, while imaging techniques for assessment of the dynamic and elastic muscle properties have not yet been translated into clinics. In this exploratory study, we quantitatively characterized muscle deformation (strain) in patients affected by facioscapulohumeral muscular dystrophy (FSHD), a prevalent muscular dystrophy, by applying dynamic MRI synchronized with neuromuscular electrical stimulation (NMES). We evaluated the quadriceps muscles in 34 ambulatory patients and 13 healthy controls, at 6-to 12-month time intervals. While a subgroup of patients behaved similarly to controls, for another subgroup the median strain decreased over time (approximately 57% over 1.5 years). Dynamic MRI parameters did not correlate with quantitative MRI. Our results suggest that the evaluation of muscle contraction by NMES-MRI is feasible and could potentially be used to explore the elastic properties and monitor muscle involvement in FSHD and other neuromuscular disorders., (© 2022. The Author(s).)

We assess repeatability of automatic measurements of a new anterior segment optical coherence tomographer and biometer (ANTERION) and their agreement with those provided by an anterior segment-optical coherence tomography device combined with Placido-disk corneal topography (MS-39) and a validated optical biometer (IOLMaster 500). A consecutive series of patients underwent three measurements with ANTERION and one with MS-39. A subgroup of patients underwent biometry also with IOLMaster 500. Repeatability was assessed by means of within-subject standard deviation, coefficient of variation (COV), and intraclass correlation coefficient (ICC). Agreement was investigated with the 95% limits of agreement. Paired t-test and Wilcoxon matched-pairs test were performed to compare the measurements of the different devices. Repeatability of ANTERION measurements was high, with ICC > 0.98 for all parameters except astigmatism (0.963); all parameters apart from those related to astigmatism revealed a COV < 1%. Repeatability of astigmatism improved when only eyes whose keratometric astigmatism was higher than 1.0 D were investigated. Most measurements by ANTERION and MS-39 showed good agreement. No significant differences were found between measurements by ANTERION and IOLMaster, but for corneal diameter. ANTERION revealed high repeatability of automatic measurements and good agreement with both MS-39 and IOLMaster for most parameters.

Wolfram syndrome (WS) is a recessive multisystem disorder defined by the association of diabetes mellitus and optic atrophy, reminiscent of mitochondrial diseases. The role played by mitochondria remains elusive, with contradictory results on the occurrence of mitochondrial dysfunction. We evaluated 13 recessive WS patients by deep clinical phenotyping, including optical coherence tomography (OCT), serum lactic acid at rest and after standardized exercise, brain Magnetic Resonance Imaging, and brain and muscle Magnetic Resonance Spectroscopy (MRS). Finally, we investigated mitochondrial bioenergetics, network morphology, and calcium handling in patient-derived fibroblasts. Our results do not support a primary mitochondrial dysfunction in WS patients, as suggested by MRS studies, OCT pattern of retinal nerve fiber layer loss, and, in fibroblasts, by mitochondrial bioenergetics and network morphology results. However, we clearly found calcium mishandling between endoplasmic reticulum (ER) and mitochondria, which, under specific metabolic conditions of increased energy requirements and in selected tissue or cell types, may turn into a secondary mitochondrial dysfunction. Critically, we showed that Wolframin (WFS1) protein is enriched at mitochondrial-associated ER membranes and that in patient-derived fibroblasts WFS1 protein is completely absent. These findings support a loss-of-function pathogenic mechanism for missense mutations in WFS1, ultimately leading to defective calcium influx within mitochondria.

Corneal tomography is an important tool to identify and follow up eyes with keratoconus. Our study evaluate the repeatability of the automatic measurements provided in keratoconic eyes by a new anterior-segment optical coherence tomographer (AS-OCT) combined with Placido-disk topography (MS-39, CSO) and assess their agreement with the corresponding measurements taken with a rotating Scheimpflug camera combined with Placido-disk topography (Sirius, CSO). Mean simulated keratometry, posterior and total corneal power, total corneal astigmatism, corneal asphericity, thinnest corneal thickness, epithelial thickness, corneal diameter, and aqueous depth were evaluated. Repeatability was assessed using test-retest variability, the coefficient of variation, and the intraclass correlation coefficient; agreement was assessed by the 95% limits of agreement. Good repeatability was achieved for most parameters. Moderate repeatability was found for total corneal astigmatism measurements. The repeatability of mean simulated keratometry and total corneal power measurements worsened with more severe stages of keratoconus with a statistically significant relationship between the individual coefficient of variation and corneal power values. Agreement with the Scheimpflug camera was moderate for aqueous depth and thinnest corneal thickness and poor for most other measured parameters. The good repeatability of automatic measurements suggests the new AS-OCT device to be a viable option in clinical practice of eyes with keratoconus.

The purpose was to define a normative database for a grating test for contrast sensitivity, based on a chart monitor with high-definition liquid crystal display, and validate its measurements by assessing their repeatability and determining responsiveness to cataract surgery. Three samples were analyzed: (1) healthy volunteers to assess the repeatability of measurements, (2) healthy subjects to develop the normative database, (3) patients undergoing cataract surgery. All subjects were tested with the grating contrast sensitivity test (Vision Chart, CSO) at 1.5, 3, 6, 12 and 18 cycles per degree. The instrument software progressively reduces the contrast of the gratings according to the Quick Estimate by Sequential Testing (QUEST) procedure. In the subjects of the first sample, three consecutive measurements were taken and repeatability was assessed on the basis of the intra-session test-retest variability and the coefficient of variation. The test offered high repeatability, with test-retest variability ranging between 0.05 and 0.23 Log CS and the coefficient of variation between 0.61 and 4.21%. Normative data did not show a normal distribution. The highest median values were observed at 1.5, 3 and 6 cycles per degree frequencies. At these frequencies a ceiling effect was evident. In cataract patients, postoperative values showed an improvement at all spatial frequencies. In conclusion, the new contrast sensitivity test provides repeatable measurements that can be used for clinical purposes. In patients with healthy eyes and good vision, attention has to be paid to the ceiling effect.

We describe the design, build and characterization of a novel two-output port configuration for a THz-Time Domain Spectroscopy (TDS) system. By introducing a tilted THz ultra-broadband polarizer, we split the THz beam in two orthogonal polarization detector branches. The probe laser is similarly split (with an optical polarizer) replicating the detection chain to obtain two independent orthogonal polarization detection units. We describe the system's performance highlighting some of the advantages of this system in one of its two modes of operation: optimized polarimetry for Jones matrix measurements. A bi-refringent sapphire standard was measured to confirm its capabilities and assess the performance of the system showing good agreement with existing literature data.

To assess the repeatability and reproducibility of AL-Scan in agreement with those by the IOLMaster in healthy children, two skilled operators measured ocular parameters in 58 children. The parameters included keratometry (K) values, anterior chamber depth (ACD), axial length (AL), central corneal thickness (CCT), pupil diameter (PD), and corneal diameter (CD). The cohort comprised of 32 boys and 26 girls. The AL-Scan measurements showed high repeatability, as the test-retest repeatability (TRT) values of AL, CCT, ACD, Kf, Ks, Km, CD, and PD were 0.09 mm, 5.1 μm, 0.04 mm, 0.28 D, 0.24 D, 0.21 D, 0.39 mm, and 0.22 mm, respectively. The within-subject coefficient of variation (CoV) was low (<0.35%) and the intraclass correlation coefficients (ICC) of all parameters were >0.85. The interobserver reproducibility was excellent with low values of TRT and ICC > 0.95. The CoV of AL, CCT, ACD, and K was <0.22%. The 95% limits of agreement between the AL-Scan and the IOLMaster were narrow for all parameters except for CD. The repeatability and reproducibility of the new biometer, Al-Scan, was excellent for all parameters and can be routinely used in children to measure the biometric values.

The present study was to assess the precision (repeatability and reproducibility) of a new optical biometer (OA-2000, Tomey, Japan) based on swept-source optical coherence tomography (SS-OCT) and Placido disk topography in eyes with cataracts. Seventy-eight eyes from seventy-eight patients with cataracts were evaluated. Axial length (AL), anterior chamber depth (ACD), keratometry (K) over a 2.5 mm and 3.0 mm diameter, lens thickness (LT), central corneal thickness (CCT) and white-to-white (WTW) distance were measured by 2 skilled operators. OA-2000 measurements were highly repeatable and reproducible for all parameters (intraclass correlation, 0.925 to 1.000). OA-2000 derived K-values with a diameter of 3.0mm showed narrower 95% limits of agreement (LoA) (SRK/T: -0.18 to 0.16D; Holladay 1: -0.20 to 0.19D; Hoffer Q: -0.22 to 0.20D) than those with a diameter of 2.5 mm for IOL power calculations (SRK/T: -0.20 to 0.20D; Holladay 1: -0.23 to 0.23D; Hoffer Q: -0.25 to 0.25D). The precision (repeatability and reproducibility) of the OA-2000 was excellent for all parameters. The 3.0mm diameter K-readings appear to be the most reliable choice for calculation of IOL power with the OA-2000. In addition, the average values determined from each operator's 3 consecutive readings were more reproducible.

The purpose of the current study was to compare the measurements between a new optical biometer based on swept-source optical coherence tomography (SS-OCT), the OA-2000 (Tomey, Japan), and an optical biometer based on optical low coherence reflectometry (OLCR), the Lenstar (Haag-Streit, Switzerland). Ninety-nine eyes of 99 healthy subjects were included. The axial length (AL), central corneal thickness (CCT), anterior chamber depth (ACD), aqueous depth (AD), lens thickness (LT), keratometry (K) readings, including flat K (Kf), steep K (Ks), mean K (Km), astigmatism vectors J 0 , J 45 at diameters of 2.5 and 3.0 mm, and white-to-white diameter (WTW) were measured three times each using both biometer in normal eyes by random sequence. Bland-Altman analysis showed good agreement between the SS-OCT and OLCR devices for AL, AD, ACD, LT, with narrow 95% LoA (-0.05 to 0.07 mm, -0.09 to 0.10 mm, -0.10 to 0.09 mm, and -0.06 to 0.22 mm, respectively), and the P values of ACD were both >0.05. The CCT, Kf, Ks, Km, J 0 , J 45 and WTW values provided by the OA-2000 were in good agreement with the Lenstar, and statistically significant differences were detected for some of them but not clinical differences. The agreement was excellent especially for AL.

A meta-analysis to compare ocular biometry measured by optical low-coherence reflectometry (Lenstar LS900; Haag Streit) and partial coherence interferometry (the IOLMaster optical biometer; Carl Zeiss Meditec). A systematic literature search was conducted for articles published up to August 6th 2015 in the Cochrane Library, PubMed, Medline, Embase, China Knowledge Resource Integrated Database and Wanfang Data. A total of 18 studies involving 1921 eyes were included. There were no statistically significant differences in axial length (mean difference [MD] 0 mm; 95% confidence interval (CI) -0.08 to 0.08 mm; p = 0.92), anterior chamber depth (MD 0.02 mm; 95% CI -0.07 to 0.10 mm; p = 0.67), flat keratometry (MD -0.05 D; 95% CI -0.16 to 0.06 D; p = 0.39), steep keratometry (MD -0.09 D; 95% CI -0.20 to 0.03 D; p = 0.13), and mean keratometry (MD -0.15 D; 95% CI -0.30 to 0.00 D; p = 0.05). The white to white distance showed a statistically significant difference (MD -0.14 mm; 95% CI -0.25 to -0.02 mm; p = 0.02). In conclusion, there was no difference in the comparison of AL, ACD and keratometry readings between the Lenstar and IOLMaster. However the WTW distance indicated a statistically significant difference between the two devices. Apart from the WTW distance, measurements for AL, ACD and keratometry readings may be used interchangeability with both devices.

Leber's hereditary optic neuropathy (LHON) is typically characterized by vascular alterations in the acute phase. The aim of this study was to evaluate choroidal changes occurring in asymptomatic, acute and chronic stages of LHON. We enrolled 49 patients with LHON, 19 with Dominant Optic Atrophy (DOA) and 22 healthy controls. Spectral Domain-Optical Coherence Tomography (SD-OCT) scans of macular and peripapillary regions were performed in all subjects, to evaluate macular and peripapillary choroidal thickness, and retinal nerve fiber layer (RNFL) thicknes. Macular and peripapillary choroidal thicknesses were significantly increased in the acute LHON stage. On the contrary, macular choroidal thickness was significantly reduced in the chronic stage. Furthermore, peripapillary choroidal thickness was decreased in chronic LHON and in DOA. Both RNFL and choroid had the same trend (increased thickness, followed by thinning), but RNFL changes preceded those affecting the choroid. In conclusion, our study quantitatively demonstrated the involvement of the choroid in LHON pathology. The increase in choroidal thickness is a feature of the LHON acute stage, which follows the thickening of RNFL. Conversely, thinning of the choroid is the common outcome in chronic LHON and in DOA.