Search

Study Design: Retrospective cohort study., Objective: To investigate long-term outcomes after short or long fusion for adult spinal deformity using lateral interbody fusion., Summary of Background Data: Lateral interbody fusion is commonly used in adult spinal deformity surgery. Favorable short-term outcomes have been reported, but not long-term outcomes. Lateral interbody fusion with strong ability to correct deformity may allow the selection of short fusion techniques., Materials and Methods: We retrospectively reviewed adults who underwent this surgery with a minimum of 5 years of follow-up. Short fusion with the uppermost instrumented vertebra in the lumbar spine was performed in patients without degenerative changes at the thoracolumbar junction (S-group); others underwent long fusion with the uppermost instrumented vertebra in the thoracic spine (L-group). We assessed radiographic and clinical outcomes., Results: Short fusion was performed in 29 of 54 patients. One patient per group required revision surgery. Of the remainder, with similar preoperative characteristics and deformity correction between groups, correction loss (pelvic incidence-lumbar lordosis, P =0.003; pelvic tilt, P =0.005; sagittal vertical axis, P ˂0.001) occurred within 2 years postoperatively in the S-group, and sagittal vertical axis continued to increase until the 5-year follow-up ( P =0.021). Although there was a significant change in Oswestry disability index in the S-group ( P =0.031) and self-image of Scoliosis Research Society 22r score in both groups ( P =0.045 and 0.02) from 2- to 5-year follow-up, minimum clinically important differences were not reached. At 5-year follow-up, there was a significant difference in Oswestry Disability Index ( P =0.013) and Scoliosis Research Society 22r scores (function: P =0.028; pain: P =0.003; subtotal: P =0.006) between the groups, but satisfaction scores were comparable and Oswestry Disability Index score (29.8%) in the S-group indicated moderate disability., Conclusions: Health-related quality of life was maintained between 2- and 5-year follow-up in both groups. Short fusion may be an option for patients without degenerative changes at the thoracolumbar junction., Level of Evidence: III., Competing Interests: The authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Pulmonary alveolar proteinosis (PAP) is a rare disease, which is characterized by the alveolar accumulation of surfactant. A crazy-paving appearance on chest thin-section computed tomography (TSCT) is a characteristic feature of this disease. We report an unusual case of PAP, which presented as multiple localized ground glass opacites (GGOs) on TSCT in an 80-year-old female. As one of these lesions at the apex of the right lung increased in size, it was suspected to be a pulmonary adenocarcinoma. However, the others became smaller during the follow-up period. Right upper lobectomy was performed, and PAP was histologically diagnosed. In cases exhibiting multiple localized GGOs, PAP should be considered, even if GGOs with a crazy-paving-like appearance are distributed in a lobular rather than diffuse manner., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Understanding the complex formation of metal ions in room-temperature ionic liquids (ILs) is essential for the application of ILs in solvent extraction. Nevertheless, the research on metal complex formation in ILs lags behind other applications. The complex formation equilibria may be influenced by specific interactions among the metal ion, ligand molecule, and IL cation and anion. In the present investigation, the complex formation of Ni 2+ with ethanol (EtOH) and methanol (MeOH) molecules in ILs, 1-alkyl-3-methylimidazolium bis(trifluoromethylsulfonyl)amide ([C N mim][TFSA], where N represents the alkyl chain lengths of 2 and 8) was discussed in terms of the microscopic interactions among alcohol molecules, [C N mim] + and [TFSA] - , and the mesoscopic mixing states of alcohols in [C N mim][TFSA], with N = 2-12. The microscopic interaction of alcohol molecules with the imidazolium ring H atoms in the ILs was evaluated by using ATR-IR and 1 H and 13 C NMR spectroscopies. The self-hydrogen bonding of alcohol molecules was clarified from the O-H stretching vibration of alcohol molecules. MeOH molecules can be more strongly hydrogen-bonded with themselves than EtOH molecules due to the less steric hindrance and the weaker dispersion force of MeOH with the IL cation's alkyl chain. In fact, small-angle neutron scattering (SANS) experiments revealed the more heterogeneous mixing of MeOH with the ILs by the self-hydrogen bonding among MeOH molecules than EtOH. The longer the IL cation's alkyl chain, the more the MeOH clusters significantly form. In contrast, the formation of EtOH clusters becomes weaker with elongating the alkyl chain. Ultraviolet (UV)-visible spectroscopic measurements on Ni 2+ -[C N mim][TFSA]-alcohol solutions with N = 2 and 8 revealed that di-, tetra-, and hexa-alcohol-Ni 2+ complexes are formed in both the ILs. With N = 2, the stabilities of Ni 2+ -EtOH and Ni 2+ -MeOH complexes are comparable in the IL. However, with N = 8, the complexes are more stable in the EtOH solutions than in the MeOH solutions. This is because the less heterogeneous mixing of EtOH molecules with the IL results in the larger enthalpic contribution in the complex formation, as shown by the thermodynamic parameters estimated by the van't Hoff plots on the stability constants at several temperatures.

Agglutination of pathogenic microorganisms on the body surface is a significant phenomenon for the prevention of infection. In the present study, we show that an extract of the skin mucus from Japanese flounder (Paralichthys olivaceus) has agglutination activity against the yeast Saccharomyces cerevisiae. We purified this yeast-binding protein, which consists of an approximately 35-kDa homodimer, using affinity chromatography with yeast as a ligand. Multiple internal amino acid sequences of the protein, as determined using liquid chromatography with quadrupole time-of-flight tandem mass spectrometry, mapped to flounder glyceraldehyde 3-phosphate dehydrogenase (GAPDH). An anti-GAPDH antibody inhibited the yeast agglutination activity in the skin mucus extract and stained agglutinated yeast, indicating that flounder GAPDH could agglutinate yeast. The current study suggests that GAPDH, a well-known protein as the sixth enzyme in the glycolytic pathway, is a significant player in mucosal immunity in teleosts., (© 2024 The Societies and John Wiley & Sons Australia, Ltd.)

Despite less invasive surgical procedures in adult spinal deformity (ASD) surgery, some older patients have complications and long recovery time. We investigated patients' willingness to undergo the same surgery again and sought to elucidate the factors related to their perception of surgical outcomes. Enrolled were 60 of our patients (≥65 years old) that underwent long corrective fusion using lateral interbody fusion and who had a minimum of 2 years of follow-up. Patients were asked whether they would theoretically undergo the same surgery again: 28 answered yes (46.7 %; Group-Y), and 32 answered no (53.3 %; Group-N). There was no difference between the groups in age, sex, body mass index, frailty, preoperative patient-reported outcomes (PROs; Oswestry disability index [ODI] and Scoliosis Research Society 22r [SRS-22r]), surgical time, estimated blood loss, or pre-operative and 2-year post-operative radiographic parameters. Major complications had occurred more frequently in Group-N (P = 0.048). Although at 2-year follow-up there was significant improvement of spinal deformity and PROs (P < 0.001) in both groups, PROs in Group-N were inferior (Visual analogue scale [VAS] for low back pain, P = 0.043; VAS for satisfaction, P = 0.001; ODI: P = 0.005; SRS-22r: pain, P = 0.032; self-image, P = 0.014; subtotal, P = 0.005; satisfaction, P < 0.001). After multivariate logistic regression analysis with the willingness to undergo the same surgery again as an objective factor, incidence of major complication was found to be an independently-associated factor in unwillingness to undergo the same surgery again for older patients with ASD if they had the same condition in the future. Avoiding major perioperative complications is important in obtaining satisfactory perception of outcomes in ASD surgery., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Multiple sclerosis (MS) is a frequently disabling neurological disorder characterized by symptoms, clinical signs and imaging abnormalities that typically fluctuate over time, affecting any level of the CNS. Prominent lymphocytic inflammation, many genetic susceptibility variants involving immune pathways, as well as potent responses of the neuroinflammatory component to immunomodulating drugs, have led to the natural conclusion that this disease is driven by a primary autoimmune process. In this Hypothesis and Theory article, we discuss emerging data that cast doubt on this assumption. After three decades of therapeutic experience, what has become clear is that potent immune modulators are highly effective at suppressing inflammatory relapses, yet exhibit very limited effects on the later progressive phase of MS. Moreover, neuropathological examination of MS tissue indicates that degeneration, CNS atrophy, and myelin loss are most prominent in the progressive stage, when lymphocytic inflammation paradoxically wanes. Finally, emerging clinical observations such as "progression independent of relapse activity" and "silent progression," now thought to take hold very early in the course, together argue that an underlying "cytodegenerative" process, likely targeting the myelinating unit, may in fact represent the most proximal step in a complex pathophysiological cascade exacerbated by an autoimmune inflammatory overlay. Parallels are drawn with more traditional neurodegenerative disorders, where a progressive proteopathy with prion-like propagation of toxic misfolded species is now known to play a key role. A potentially pivotal contribution of the Epstein-Barr virus and B cells in this process is also discussed., Competing Interests: PS and ST have patents pertaining to MS. AG was an employee and shareholder of Biogen but the opinions expressed in this article are solely those of the author and not necessarily those of Biogen. SB was previously an employee and shareholder of Biogen. SB was employed by TheraPanacea and Indivi (DBA of Healios AG). The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Stys, Tsutsui, Gafson, ‘t Hart, Belachew and Geurts.)

Inelastic x-ray scattering (IXS) experiments on liquid sulphur were carried out below (140  ∘ C) and above (180  ∘ C) the polymerisation temperature T λ of about 159  ∘ C to investigate changes in the collective dynamics of this unique liquid, that exhibits a liquid-liquid transition. As reported earlier, broad longitudinal acoustic excitation signals were observed at both temperatures, and only the width of the quasielastic peaks slightly decreased when the temperature crossed the transition temperature. A model analysis was performed using a generalised Langevin formalism with a memory function having one thermal and two viscoelastic decay channels with the help of simple sparse modelling, and large positive deviations from the hydrodynamic sound velocity by 51%-54% were observed. The fast viscoelastic relaxation time τ µ is close to the correlation times of intermolecular stretching and bending motions of local sulphur connections in both ring and chain structures, and is similar to those of other molecular liquids. The small contrasts in the IXS spectra across the λ transition result in large changes in only the slow viscoelastic decay time τ C matches the mixed internal/external torsional modes of S α of the memory function. The τ α value at 140  ∘ C matches the mixed internal/external torsional modes of S 8 molecules well, whereas that at 180  ∘ C has no corresponding molecular motion mode. The kinematic viscosity values at theQ→0limit are much smaller than the minimum values of macroscopic shear viscosity, indicating that large changes in relaxation dynamics are expected with Q in the GHz and MHz excitation regimes., (© 2024 IOP Publishing Ltd.)

Objective: In this study, the authors aimed to determine the mid- to long-term outcomes of microendoscopic laminotomy (MEL) for lumbar spinal stenosis (LSS) with degenerative spondylolisthesis (DS) and identify preoperative predictors of poor mid- to long-term outcomes., Methods: The authors retrospectively reviewed the medical records of 274 patients who underwent spinal MEL for symptomatic LSS. The minimum postoperative follow-up duration was 5 years. Patients were classified into two groups according to DS: those with DS (the DS+ group) and those without DS (the DS- group). The patients were subjected to propensity score matching based on sex, age, BMI, surgical segments, and preoperative leg pain visual analog scale scores. Clinical outcomes were evaluated 1 year and > 5 years after surgery., Results: Surgical outcomes of MEL for LSS were not significantly different between the DS+ and DS- groups at the final follow-up (mean 7.8 years) in terms of Oswestry Disability Index (p = 0.498), satisfaction (p = 0.913), and reoperation rate (p = 0.154). In the multivariate analysis, female sex (standard β -0.260), patients with slip angle > 5° in the forward bending position (standard β -0.313), and those with dynamic progression of Meyerding grade (standard β -0.325) were at a high risk of poor long-term outcomes., Conclusions: MEL may have good long-term results in patients with DS without dynamic instability. Women with dynamic instability may require additional fusion surgery in approximately 25% of cases for a period of ≥ 5 years.

Introduction: Pedicle screws are commonly used in fixation to treat various spinal disorders. However, screw loosening is a prevalent complication, particularly in patients with osteoporosis. Various biomechanical studies have sought to address this issue, but the optimal depth and trajectory to increase the fixation strength of pedicle screws remain controversial. Therefore, a biomechanical study was conducted using finite element models., Methods: Three-dimensional finite element models of the L3 vertebrae were developed from the preoperative computed tomography images of nine patients with osteoporosis and nine patients without who underwent spine surgery. Unicortical and bicortical pedicle screws were inserted into the center and into the anterior wall of the vertebrae, respectively, in different trajectories in the sagittal plane: straightforward, cephalad, and caudal. Subsequently, three different external loads were applied to each pedicle screw at the entry point: axial pullout, craniocaudal, and lateromedial loads. Nonlinear analysis was conducted to examine the fixation strength of the pedicle screws., Results: Irrespective of osteoporosis, the bicortical pedicle screws had greater fixation strength than the unicortical pedicle screws in all trajectories and external loads. The fixation strength of the bicortical pedicle screws was not substantially different among the trajectories against any external loads in the nonosteoporotic vertebrae. However, the fixation strength of the bicortical pedicle screws against craniocaudal load in the cephalad trajectory was considerably greater than those in the caudal ( P =0.016) and straightforward ( P =0.023) trajectories in the osteoporotic vertebrae. However, this trend was not observed in pullout and lateromedial loads., Conclusions: Our results indicate that bicortical pedicle screws should be used, regardless of whether the patient has osteoporosis or not. Furthermore, pedicle screws should be inserted in the cephalad trajectory in patients with osteoporosis., Competing Interests: Conflicts of Interest: The authors declare that there are no relevant conflicts of interest., (Copyright © 2024 The Japanese Society for Spine Surgery and Related Research.)

Introduction: This study aimed to compare the outcomes of corrective fusion for adult spinal deformity (ASD) in older people using two different sagittal correction goals: the conventional formula of "pelvic incidence (PI)-lumbar lordosis (LL) mismatch <10°" and an undercorrection strategy based on the range of 10°≤PI-LL≤20°., Methods: A total of 102 consecutive patients (11 male and 91 female patients; mean age, 72.0 years) aged above 65 years with scoliosis >20° or LL<20° who had undergone long-segment fusion from the lower thoracic spine to the pelvis for ASD and had been followed-up for a minimum of two years at our institution since March 2013 were included in this retrospective study. After excluding patients with PI-LL≤-10° on postoperative standing radiographs, the remaining patients were divided into two groups: 31 patients with 10°≤PI-LL≤20° (U group) and 63 patients with -10°

Interspecific hybrids of farm-raised fish are becoming popular in aquaculture owing to their advantages over pure species, including improved growth and higher resistance to infectious diseases. Kue-Tama is a recently established hybrid grouper derived from the longtooth grouper Epinephelus bruneus (♀) × giant grouper E. lanceolatus (♂). In our previous study, this hybrid showed significantly higher resistance against the skin fluke Benedenia epinepheli, a problematic parasite in grouper farming, than the longtooth grouper. In the present study, we explored lectins in the skin mucus of hybrids and their parent species. While C-type lectins of approximately 15 kDa were obtained from longtooth groupers, additional C-type lectins with molecular masses of approximately 20 and 30 kDa, as well as 45-kDa F-type lectin, were also detected in Kue-Tama and giant groupers. Semi-quantitative reverse transcript-polymerase chain reaction (RT-PCR) demonstrated that the gene expression levels of both C-type and F-type lectins were significantly higher in the skin of the hybrid and giant groupers than that of the longtooth grouper. In addition, some skin mucus lectins of the hybrid and giant groupers were bound to the fluke, suggesting that these lectins conferred resistance to parasitic infections., (Copyright © 2023. Published by Elsevier Ltd.)

One-shot fine-grained visual recognition often suffers from the problem of having few training examples for new fine-grained classes. To alleviate this problem, off-the-shelf image generation techniques based on Generative Adversarial Networks (GANs) can potentially create additional training images. However, these GAN-generated images are often not helpful for actually improving the accuracy of one-shot fine-grained recognition. In this paper, we propose a meta-learning framework to combine generated images with original images, so that the resulting "hybrid" training images improve one-shot learning. Specifically, the generic image generator is updated by a few training instances of novel classes, and a Meta Image Reinforcing Network (MetaIRNet) is proposed to conduct one-shot fine-grained recognition as well as image reinforcement. Our experiments demonstrate consistent improvement over baselines on one-shot fine-grained image classification benchmarks. Furthermore, our analysis shows that the reinforced images have more diversity compared to the original and GAN-generated images.

Objectives: The high rate of delayed bleeding after colorectal endoscopic submucosal dissection (ESD) in patients undergoing anticoagulant therapy remains a problem. Whether prophylactic clip closure reduces the rate of delayed bleeding in these patients is unclear. This study aimed to evaluate the efficacy of prophylactic clip closure in patients receiving anticoagulants., Methods: This multicenter prospective interventional trial was conducted at nine referral centers in Japan. Patients regularly taking anticoagulants, including warfarin potassium or direct oral anticoagulants, and undergoing ESD for colorectal neoplasms were enrolled. The discontinuation of anticoagulants was minimized according to recent guidelines. After the ESD, post-ESD ulcers were prophylactically closed using endoclips. The primary end-point was the incidence of delayed bleeding. The sample size was 45 lesions, and prophylactic clip closure was considered effective when the upper limit of the 90% confidence interval (CI) for delayed bleeding did not exceed 20%., Results: Forty-five lesions were used, and three were excluded. Complete closure was achieved in 41/42 lesions (97.6%). The overall delayed bleeding rate was low, at 4.9% (2/41; 90% [CI] 0.8-14.5), which was significantly lower than that at the prespecified threshold of 20% (P = 0.007). The median closure procedure time was 17 min, and the median number of clips was nine. No massive delayed bleeding requiring transfusion, interventional radiology, or surgery was observed, and no thromboembolic events were observed., Conclusion: Prophylactic clip closure may reduce the risk of delayed bleeding following colorectal ESD in patients receiving anticoagulants., Trial Registration: UMIN Clinical Trial Registry (UMIN000036734)., (© 2024 The Authors. Digestive Endoscopy published by John Wiley & Sons Australia, Ltd on behalf of Japan Gastroenterological Endoscopy Society.)

Purpose: The present study aimed to investigate whether diffusion-weighted imaging (DWI) can qualify and quantify cerebrospinal fluid (CSF) dynamics in the brains of healthy subjects. For this purpose, we developed new DWI-based fluidography and compared the CSF dynamics seen on the fluidography with two apparent diffusion coefficients obtained with different DWI signal models at anatomical spaces filled by CSF., Methods: DWI with multiple b values was performed for 10 subjects using a 7T MRI scanner. DWI-fluidography based on the DWI signal variations in different motion probing gradient directions was developed for visualizing the CSF dynamics voxel-by-voxel. DWI signals were measured using an ROI in the representative CSF-filled anatomical spaces in the brain. For the multiple DWI signals, the mono-exponential and kurtosis models were fitted and two kinds of apparent diffusion coefficients (ADC C and ADC K ) were estimated in each space using the Gaussian and non-Gaussian diffusion models, respectively., Results: DWI-fluidography could qualitatively represent the features of CSF dynamics in each anatomical space. ADCs indicated that the motions at the foramen of Monro, the cistern of the velum interpositum, the quadrigeminal cistern, the Sylvian cisterns, and the fourth ventricle were more drastic than those at the subarachnoid space and anterior horns of the lateral ventricle. Those results seen in ADCs were identical to the findings on DWI-fluidography., Conclusion: DWI-fluidography based on the features of DWI signals could show differences of CSF dynamics among anatomical spaces.

Introduction: Postoperative spinal epidural hematoma (PSEH) is a severe complication of spinal surgery that necessitates accurate and timely diagnosis. This study aimed to assess the accuracy of ultrasonography as an alternative diagnostic tool for PSEH after microendoscopic laminotomy (MEL) for lumbar spinal stenosis, comparing it with magnetic resonance imaging (MRI)., Methods: A total of 65 patients who underwent MEL were evaluated using both ultrasound- and MRI-based classifications for PSEH. Intra- and interrater reliabilities were analyzed. Furthermore, ethical standards were strictly followed, with spine surgeons certified by the Japanese Orthopaedic Association performing evaluations., Results: Among the 65 patients, 91 vertebral segments were assessed. The intra- and interrater agreements for PSEH classification were almost perfect for both ultrasound (κ=0.824 [95% confidence interval (CI) 0.729-0.918] and κ=0.810 [95% CI 0.712-0.909], respectively) and MRI (κ=0.839 [95% CI 0.748-0.931] and κ=0.853 [95% CI 0.764-0.942], respectively). The results showed high concordance between ultrasound- and MRI-based classifications, validating the reliability of ultrasound in postoperative PSEH evaluation., Conclusions: This study presents a significant advancement by introducing ultrasound as a precise and practical alternative to MRI for PSEH evaluation. The comparable accuracy of ultrasound to MRI, rapid bedside assessments, and radiation-free nature make it valuable for routine postoperative evaluations. Despite the limitations related to specific surgical contexts and clinical outcome assessment, the clinical potential of ultrasound is evident. It offers clinicians a faster, cost-effective, and repeatable diagnostic option, potentially enhancing patient care. This study establishes the utility of ultrasound in evaluating postoperative spinal epidural hematomas after MEL. With high concordance to MRI, ultrasound emerges as a reliable, practical, and innovative tool, promising improved diagnostic efficiency and patient outcomes. Further studies should explore its clinical impact across diverse surgical scenarios., Competing Interests: Conflicts of Interest: The authors declare that there are no relevant conflicts of interest., (Copyright © 2024 The Japanese Society for Spine Surgery and Related Research.)

Objective: The objective of this study was to determine the long-term outcomes of microendoscopic foraminotomy in treating lumbar foraminal stenosis and identify the optimal extent of decompression that yields improved results and fewer complications., Methods: A retrospective cohort study reviewed the medical records of 95 consecutive patients who underwent microendoscopic foraminotomy for lumbar foraminal stenosis. Clinical outcomes were assessed using the Japanese Orthopaedic Association scoring system and visual analog scale for low back and leg pain. Surgical success was determined by meeting significant improvement thresholds for back and leg pain at 2 years postoperatively. Multiple regression analysis identified factors associated with improved pain scores. Receiver operating characteristic curve analysis determined the cut-off values for successful surgeries., Results: Significant improvements were observed in Japanese Orthopaedic Association and visual analog scale scores for back and leg pain 2 years postoperatively compared with preoperative scores (P < 0.0001) and sustained over a ≥5-year follow-up period. Reoperation rates were low and did not significantly increase over time. Multiple regression analysis identified occupancy of the vertebral osteophytes and bulging intervertebral discs (O/D complex) as surgical success predictors. A 45.0% O/D complex occupancy cutoff value was determined, displaying high sensitivity and specificity for predicting surgical success., Conclusions: This study provides evidence supporting the long-term efficacy of microendoscopic foraminotomy for lumbar foraminal stenosis and predicting surgical success. The 45.0% O/D complex occupancy cut-off value can guide patient selection and outcome prediction. These insights contribute to informed surgical decision-making and underscore the importance of evaluating the O/D complex in preoperative planning and predicting outcomes., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Background and Aim: Various techniques for direct biopsy from gastrointestinal subepithelial tumors (SETs) have been reported, although no standard method has been established. A common feature of these techniques is the removal of overlaying mucosa to enable direct biopsies from the SETs. These methods have been synthesized under the collective term "unroofing technique". We conducted a multicenter retrospective study to assess its efficacy and identify potential complications., Methods: This study was conducted in 10 hospitals and involved all eligible patients who underwent unroofing techniques to obtain biopsies for gastrointestinal SETs between April 2015 and March 2021. The primary endpoint was the diagnostic accuracy of the unroofing technique, and the secondary endpoints were the incidence of adverse events and the factors contributing to the accurate diagnosis., Results: The study included 61 patients with 61 gastrointestinal SETs. The median tumor size was 20 mm, and the median procedure time was 38 min, with 82% successful tumor exposure. The rate of pathological diagnosis was 72.1%. In 44 patients with a pathological diagnosis, two showed discrepancies with the postresection pathological diagnosis. No factors, including facility experience, organ, tumor size, or tumor exposure, significantly affected the diagnostic accuracy. There was one case of delayed bleeding and two cases of perforation., Conclusion: The diagnostic yield of the unroofing technique was acceptable. The unroofing technique was beneficial regardless of institutional experience, organ, tumor size, or actual tumor exposure., Competing Interests: None., (© 2024 The Authors. DEN Open published by John Wiley & Sons Australia, Ltd on behalf of Japan Gastroenterological Endoscopy Society.)

Purpose: To report the genotypes and clinical features of RHO-associated retinitis pigmentosa (RHO-RP) in the Kyushu region of Japan., Study Design: Retrospective, single-center study., Methods: Sixteen RP patients with pathogenic RHO variants seen at Kyushu University Hospital were investigated. Clinical data including age, best-corrected visual acuity (BCVA) in logarithm of the minimum angle of resolution (logMAR) units, visual field, fundus photography, and optical coherence tomography were retrospectively obtained. Visual outcomes were compared between classical and sector phenotypes and among genetic variants., Results: The mean age at the first visit was 54.0 ± 15.7 years, with a mean follow-up of 7.6 ± 4.0 years. Fourteen patients (87.5%) showed the classical RP phenotype, of whom four were associated with p.[Pro23Leu] and two had p.[Pro347Leu] variants. In addition, two patients with the sector phenotype harbored p.[Ala164Val] variants. Among the classical RHO-RP patients, the mean BCVA decreased from 0.60 to 1.08 logMAR over the follow-up period (7.4 ± 4.1 years) whereas BCVA was preserved at 0.04 logMAR in sector RHO-RP patients (9.0 ± 3.0 years). Genotype-to-phenotype analysis demonstrated that p.[Pro347Leu] was associated with severe vision loss at an earlier age. Macular complications such as epiretinal membrane and cystoid macular edema were observed in 5 classical RHO-RP patients., Conclusion: p.[Pro23Leu], but not p.[Pro23His], was a frequent variant causing RHO-RP in the Kyushu region of Japan. As reported in previous studies, patients with the p.[Pro347Leu] variant showed a more severe phenotype, and variants causing sector RHO-RP were associated with a good prognosis., (© 2023. Japanese Ophthalmological Society.)