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Scientific Reports Jun 2024This study aimed to investigate the effect of orbital wall decompression surgery and reduction of proptosis on the choroidal vascularity index (CVI) and subfoveal...
This study aimed to investigate the effect of orbital wall decompression surgery and reduction of proptosis on the choroidal vascularity index (CVI) and subfoveal choroidal thickness (SFCT) in patients with thyroid eye disease (TED). Fifty-one eyes from 38 patients with controlled TED and proptosis were enrolled in this study. The majority of the patients (50.9%) had a clinical activity score (CAS) of zero, and none had a CAS greater than 2. The patients underwent a complete baseline ophthalmologic examination, and their choroidal profile alterations were monitored using enhanced depth imaging optical coherence tomography (EDI-OCT) before and during the three months after surgery. Changes in SFCT, luminance area (LA), total choroidal area (TCA), and the choroidal vascularity index (CVI) were measured as the ratio of LA to TCA in EDI-OCT images. The participants had an average age of 46.47 years, and 22 were female (57.9%). The SFCT of the patients exhibited a significant reduction over the follow-up period, decreasing from 388 ± 103 to 355 ± 95 µm in the first month (p < 0.001) and further decreasing to 342 ± 109 µm by the third month compared to baseline (p < 0.001). The CVI exhibited a drop from 0.685 ± 0.037 at baseline to 0.682 ± 0.035 and 0.675 ± 0.030 at 1 and 3 months post-surgery, respectively. However, these changes were not statistically significant, indicating comparable decreases in both LA and TCA. There was a significant correlation between improved proptosis and reduction in SFCT (p < 0.001) but not with CVI (p = 0.171). In conclusion, during the three months of follow-up following orbital wall decompression, CVI did not change, while SFCT reduced significantly. Additionally, SFCT was significantly correlated with proptosis reduction, whereas CVI was not.
Topics: Humans; Female; Male; Middle Aged; Decompression, Surgical; Graves Ophthalmopathy; Choroid; Tomography, Optical Coherence; Adult; Orbit; Exophthalmos; Aged; Treatment Outcome
PubMed: 38942805
DOI: 10.1038/s41598-024-65884-7 -
Nature Communications Jun 2024While human vision spans 220°, traditional functional MRI setups display images only up to central 10-15°. Thus, it remains unknown how the brain represents a scene...
While human vision spans 220°, traditional functional MRI setups display images only up to central 10-15°. Thus, it remains unknown how the brain represents a scene perceived across the full visual field. Here, we introduce a method for ultra-wide angle display and probe signatures of immersive scene representation. An unobstructed view of 175° is achieved by bouncing the projected image off angled-mirrors onto a custom-built curved screen. To avoid perceptual distortion, scenes are created with wide field-of-view from custom virtual environments. We find that immersive scene representation drives medial cortex with far-peripheral preferences, but shows minimal modulation in classic scene regions. Further, scene and face-selective regions maintain their content preferences even with extreme far-periphery stimulation, highlighting that not all far-peripheral information is automatically integrated into scene regions computations. This work provides clarifying evidence on content vs. peripheral preferences in scene representation and opens new avenues to research immersive vision.
Topics: Humans; Visual Cortex; Magnetic Resonance Imaging; Male; Adult; Female; Young Adult; Visual Perception; Photic Stimulation; Brain Mapping; Neuroimaging; Visual Fields; Pattern Recognition, Visual
PubMed: 38942766
DOI: 10.1038/s41467-024-49669-0 -
Hydroxychloroquine in recurrent pregnancy loss: data from a French prospective multicenter registry.Human Reproduction (Oxford, England) Jun 2024What are the outcomes of pregnancies exposed to hydroxychloroquine (HCQ) in women with a history of recurrent pregnancy loss (RPL), and what factors predict the course...
STUDY QUESTION
What are the outcomes of pregnancies exposed to hydroxychloroquine (HCQ) in women with a history of recurrent pregnancy loss (RPL), and what factors predict the course of these pregnancies beyond the first trimester?
SUMMARY ANSWER
In our cohort of pregnancies in women with a history of RPL exposed to HCQ early in pregnancy, we found that the only factor determining the success of these pregnancies was the number of previous miscarriages.
WHAT IS KNOWN ALREADY
Dysregulation of the maternal immune system plays a role in RPL. HCQ, with its dual immunomodulating and vascular protective effects, is a potential treatment for unexplained RPL.
STUDY DESIGN, SIZE, DURATION
The FALCO (Facteurs de récidive précoce des fausses couches) registry is an ongoing French multicenter infertility registry established in 2017 that includes women (aged from 18 to 49 years) with a history of spontaneous RPL (at least three early miscarriages (≤12 weeks of gestation (WG)) recruited from several university hospitals.
PARTICIPANTS/MATERIALS, SETTING, METHODS
Spontaneous pregnancies enrolled in the FALCO registry with an exposure to HCQ (before conception or at the start of pregnancy) were included. Pregnancies concomitantly exposed to tumor necrosis factor inhibitors, interleukin-1 and -2 inhibitors, intravenous immunoglobulin, and/or intravenous intralipid infusion, were excluded. Concomitant treatment with low-dose aspirin (LDA), low-molecular weight heparin (LMWH), progesterone, and/or prednisone was allowed. All patients underwent the recommended evaluations for investigating RPL. Those who became pregnant received obstetric care in accordance with French recommendations and were followed prospectively. The main endpoint was the occurrence of a pregnancy continuing beyond 12 WG, and the secondary endpoint was the occurrence of a live birth.
MAIN RESULTS AND THE ROLE OF CHANCE
One hundred pregnancies with HCQ exposure in 74 women were assessed. The mean age of the women was 34.2 years, and the median number of previous miscarriages was 5. Concomitant exposure was reported in 78 (78%) pregnancies for prednisone, 56 (56%) pregnancies for LDA, and 41 (41%) pregnancies for LMWH. Sixty-two (62%) pregnancies ended within 12 WG, the other 38 (38%) continuing beyond 12 WG. The risk of experiencing an additional early spontaneous miscarriage increased with the number of previous miscarriages, but not with age. The distributions of anomalies identified in RPL investigations and of exposure to other drugs were similar between pregnancies lasting ≤12 WG and those continuing beyond 12WG. The incidence of pregnancies progressing beyond 12 WG was not higher among pregnancies with at least one positive autoantibody (Ab) (i.e. antinuclear Ab titer ≥1:160, ≥1 positive conventional and/or non-conventional antiphospholipid Ab, and/or positive results for ≥1 antithyroid Ab) without diminished ovarian reserve (18/51, 35.3%) than among those without such autoantibody (18/45, 40.0%) (P = 0.63). Multivariate analysis showed that having ≤4 prior miscarriages was the only factor significantly predictive for achieving a pregnancy > 12 WG, after adjustment for age and duration of HCQ use prior to conception (adjusted odds ratio (OR) = 3.13 [1.31-7.83], P = 0.01).
LIMITATIONS, REASONS FOR CAUTION
Our study has limitations, including the absence of a control group, incomplete data for the diagnostic procedure for RPL in some patients, and the unavailability of results from endometrial biopsies, as well as information about paternal age and behavioral factors. Consequently, not all potential confounding factors could be considered.
WIDER IMPLICATIONS OF THE FINDINGS
Exposure to HCQ in early pregnancy for women with a history of RPL does not seem to prevent further miscarriages, suggesting limited impact on mechanisms related to the maternal immune system.
STUDY FUNDING/COMPETING INTEREST(S)
The research received no specific funding, and the authors declare no competing interests.
TRIAL REGISTRATION NUMBER
clinicaltrial.gov NCT05557201.
PubMed: 38942601
DOI: 10.1093/humrep/deae146 -
BMJ Paediatrics Open Jun 2024Raised intracranial pressure (ICP) contributes to approximately 20% of the admissions in the paediatric intensive care unit (PICU) in our setting. Timely identification... (Observational Study)
Observational Study
BACKGROUND
Raised intracranial pressure (ICP) contributes to approximately 20% of the admissions in the paediatric intensive care unit (PICU) in our setting. Timely identification and treatment of raised ICP is important to prevent brain herniation and death in such cases. The objective of this study was to examine the role of optic nerve sheath diameter (ONSD) in detecting clinically relevant raised ICP in children.
METHODS
A hospital-based observational analytical study in a PICU of a tertiary care institute in India on children aged 2-14 years. ONSD was measured in all children on three time points that is, day 1, day 2 and between day 4 and 7 of admission. ONSD values were compared between children with and without clinical signs of raised ICP.
RESULTS
Out of 137 paediatric patients recruited, 34 had signs of raised ICP. Mean ONSD on day 1 was higher in children with signs of raised ICP (4.99±0.57 vs 4.06±0.40; p<0.01). Mean ONSD on day 2 also was higher in raised ICP patients (4.94±0.55 vs 4.04±0.40; p<0.01). The third reading between days 4 and 7 of admission was less than the first 2 values but still higher in raised ICP patients (4.48±1.26 vs 3.99±0.57; p<0.001). The cut-off ONSD value for detecting raised ICP was 4.46 mm on the ROC curve with an area under curve 0.906 (95% CI 0.844 to 0.968), 85.3% sensitivity and 86.4% specificity. There was no difference in ONSD between the right and the left eyes at any time point irrespective of signs of raised ICP.
CONCLUSION
We found that measurement of ONSD by transorbital ultrasound was able to detect clinically relevant raised ICP with an excellent discriminatory performance at the cut-off value of 4.46 mm.
Topics: Humans; Child; Optic Nerve; Intracranial Hypertension; Child, Preschool; Female; Male; Adolescent; Intensive Care Units, Pediatric; India; Ultrasonography; Intracranial Pressure; ROC Curve; Sensitivity and Specificity
PubMed: 38942587
DOI: 10.1136/bmjpo-2023-002353 -
Neuroimaging Clinics of North America Aug 2024The diagnostic workup of multiple sclerosis (MS) has evolved considerably. The 2017 revision of the McDonald criteria shows high sensitivity and accuracy in predicting... (Review)
Review
The diagnostic workup of multiple sclerosis (MS) has evolved considerably. The 2017 revision of the McDonald criteria shows high sensitivity and accuracy in predicting clinically definite MS in patients with a typical clinically isolated syndrome and allows an earlier MS diagnosis. Neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein-immunoglobulin G-associated disease (MOGAD) are recognized as separate conditions from MS, with specific diagnostic criteria. New MR imaging markers may improve diagnostic specificity for these conditions, thus reducing the risk of misdiagnosis. This study summarizes the most recent updates regarding the application of MR imaging for the diagnosis of MS, NMOSD, and MOGAD.
Topics: Humans; Neuromyelitis Optica; Multiple Sclerosis; Myelin-Oligodendrocyte Glycoprotein; Magnetic Resonance Imaging; Immunoglobulin G; Diagnosis, Differential
PubMed: 38942518
DOI: 10.1016/j.nic.2024.03.001 -
Journal of the Academy of... Jun 2024Adverse Childhood Experiences (ACE) are associated with the development of negative health behaviors and medical illness. ACE's association with poor health outcomes has...
INTRODUCTION
Adverse Childhood Experiences (ACE) are associated with the development of negative health behaviors and medical illness. ACE's association with poor health outcomes has been well documented in the general population; however, this relationship remains less clear in liver transplant (LT) recipients. The aims of this study therefore were to determine the prevalence of ACE and the influence of ACE on LT outcomes.
METHODS
A retrospective electronic medical record review of all LT recipients over 11 years at an academic liver transplant center. Demographic, diagnostic, and disease characteristics were extracted and compared for a history of ACE. Associations between a history of ACE and extracted variables were statistically tested using Student's t-test and Chi-square tests or Fisher's Exact Test where appropriate. Graft and patient survival were tested using log-rank tests.
RESULTS
Of 1,172 LT recipients, 24.1% endorsed a history of ACE. Females (p = 0.017) and recipients with lower level of education (p < 0.001) had a higher frequency of ACE. Those with a history of ACE had a higher prevalence of HCV (p < 0.001) and higher pre-transplant BMI (P<0.001). Recipients with a history of ACE had higher prevalence of mood (p < 0.001), anxiety (p < 0.001), PTSD (p < 0.001), alcohol use (p < 0.001), and cannabis use (p < 0.001) disorders as well has higher PHQ-9 (p < 0.001) and GAD-7 (p < 0.001) scores pre and post-transplant. Those with ACE had higher incidence of recorded relapse to alcohol by 3 years post-transplant (p = 0.027). Mean lab values, graft survival, and patient survival were not significantly different between those with and without a history of ACE except for total bilirubin at 6 months (p = 0.021).
CONCLUSION
One quarter of LT recipients have experienced ACE. ACE was associated with a history of a psychiatric diagnoses, substance use disorders, elevated PHQ-9 and GAD-7 scores, and a higher prevalence of relapse to alcohol use after transplant. This population may benefit from increased/improved access to appropriate mental health and substance use services and support in the peri and post transplant period.
PubMed: 38942236
DOI: 10.1016/j.jaclp.2024.06.006 -
Molecular Genetics and Metabolism Jun 2024Glutaric aciduria type II (GAII) is a heterogeneous genetic disorder affecting mitochondrial fatty acid, amino acid and choline oxidation. Clinical manifestations vary...
Glutaric aciduria type II (GAII) is a heterogeneous genetic disorder affecting mitochondrial fatty acid, amino acid and choline oxidation. Clinical manifestations vary across the lifespan and onset may occur at any time from the early neonatal period to advanced adulthood. Historically, some patients, in particular those with late onset disease, have experienced significant benefit from riboflavin supplementation. GAII has been considered an autosomal recessive condition caused by pathogenic variants in the gene encoding electron-transfer flavoprotein ubiquinone-oxidoreductase (ETFDH) or in the genes encoding electron-transfer flavoprotein subunits A and B (ETFA and ETFB respectively). Variants in genes involved in riboflavin metabolism have also been reported. However, in some patients, molecular analysis has failed to reveal diagnostic molecular results. In this study, we report the outcome of molecular analysis in 28 Australian patients across the lifespan, 10 paediatric and 18 adult, who had a diagnosis of glutaric aciduria type II based on both clinical and biochemical parameters. Whole genome sequencing was performed on 26 of the patients and two neonatal onset patients had targeted sequencing of candidate genes. The two patients who had targeted sequencing had biallelic pathogenic variants (in ETFA and ETFDH). None of the 26 patients whose whole genome was sequenced had biallelic variants in any of the primary candidate genes. Interestingly, nine of these patients (34.6%) had a monoallelic pathogenic or likely pathogenic variant in a single primary candidate gene and one patient (3.9%) had a monoallelic pathogenic or likely pathogenic variant in two separate genes within the same pathway. The frequencies of the damaging variants within ETFDH and FAD transporter gene SLC25A32 were significantly higher than expected when compared to the corresponding allele frequencies in the general population. The remaining 16 patients (61.5%) had no pathogenic or likely pathogenic variants in the candidate genes. Ten (56%) of the 18 adult patients were taking the selective serotonin reuptake inhibitor antidepressant sertraline, which has been shown to produce a GAII phenotype, and another two adults (11%) were taking a serotonin-norepinephrine reuptake inhibitor antidepressant, venlafaxine or duloxetine, which have a mechanism of action overlapping that of sertraline. Riboflavin deficiency can also mimic both the clinical and biochemical phenotype of GAII. Several patients on these antidepressants showed an initial response to riboflavin but then that response waned. These results suggest that the GAII phenotype can result from a complex interaction between monoallelic variants and the cellular environment. Whole genome or targeted gene panel analysis may not provide a clear molecular diagnosis.
PubMed: 38941880
DOI: 10.1016/j.ymgme.2024.108516 -
The Neurodiagnostic Journal Jun 2024Intraoperative neurophysiological monitoring (IONM) is shown to be useful in surgeries when the nervous system is at risk. Its success in part relies upon proper setup...
Intraoperative neurophysiological monitoring (IONM) is shown to be useful in surgeries when the nervous system is at risk. Its success in part relies upon proper setup of often dozens of electrodes correctly placed and secured upon patients and inserted in specific stimulating and recording receptacles. Given the complicated setups and the demanding operating room environment, errors in setup are bound to occur. These have led to false negatives associated with new patient morbidities including, at times, paralysis. No studies quantify the prevalence of these types of setup errors. Approximately 800,000 operations annually utilize intraoperative neuromonitoring in the US alone, so even a small percentage of errors suggests clinical significance. In addition, these types of errors hinder the overall effectiveness of IONM and may result in lower reported sensitivities and lower cost-effectiveness of this important service. We sought to discover through a prospective study and verification through chart review the prevalence of "electrode-swap" errors (when recording and/or stimulating electrodes are incorrectly placed on the patient or in the IONM equipment during setup) across all procedures monitored. We found recording and/or stimulating electrode set up errors in 24 of 454 cases (5.3%). These data and examples of how errors were discovered intraoperatively are reported. We also offer techniques to help reduce this error rate. This study demonstrates a significant potential avoidable error in IONM diagnostic utility, patient outcome, and sensitivity/specificity of alert criteria. The value of identifying and correcting these errors is consequential, multifaceted, and far-reaching.
PubMed: 38941588
DOI: 10.1080/21646821.2024.2366563 -
Neurology(R) Neuroimmunology &... Sep 2024To systematically describe the clinical picture of double-antibody seronegative neuromyelitis optica spectrum disorders (DN-NMOSD) with specific emphasis on retinal...
BACKGROUND AND OBJECTIVES
To systematically describe the clinical picture of double-antibody seronegative neuromyelitis optica spectrum disorders (DN-NMOSD) with specific emphasis on retinal involvement.
METHODS
Cross-sectional data of 25 people with DN-NMOSD (48 eyes) with and without a history of optic neuritis (ON) were included in this study along with data from 25 people with aquaporin-4 antibody seropositive neuromyelitis optica spectrum disorder (AQP4-NMOSD, 46 eyes) and from 25 healthy controls (HCs, 49 eyes) for comparison. All groups were matched for age and sex and included from the collaborative retrospective study of retinal optical coherence tomography (OCT) in neuromyelitis optica (CROCTINO). Participants underwent OCT with central postprocessing and local neurologic examination and antibody testing. Retinal neurodegeneration was quantified as peripapillary retinal nerve fiber layer thickness (pRNFL) and combined ganglion cell and inner plexiform layer thickness (GCIPL).
RESULTS
This DN-NMOSD cohort had a history of [median (inter-quartile range)] 6 (5; 9) attacks within their 5 ± 4 years since onset. Myelitis and ON were the most common attack types. In DN-NMOSD eyes after ON, pRNFL ( < 0.001) and GCIPL ( = 0.023) were thinner compared with eyes of HCs. Even after only one ON episode, DN-NMOSD eyes already had considerable neuroaxonal loss compared with HCs. In DN-NMOSD eyes without a history of ON, pRNFL ( = 0.027) and GCIPL ( = 0.022) were also reduced compared with eyes of HCs. However, there was no difference in pRNFL and GCIPL between DN-NMOSD and AQP4-NMOSD for the whole group and for subsets with a history of ON and without a history of ON-as well as between variances of retinal layer thicknesses.
DISCUSSION
DN-NMOSD is characterized by severe retinal damage after ON and attack-independent retinal neurodegeneration. Most of the damage occurs during the first ON episode, which highlights the need for better diagnostic markers in DN-NMOSD to facilitate an earlier diagnosis as well as for effective and early treatments. In this study, people with DN-NMOSD presented with homogeneous clinical and imaging findings potentially suggesting a common retinal pathology in these patients.
Topics: Humans; Neuromyelitis Optica; Female; Male; Adult; Cross-Sectional Studies; Middle Aged; Tomography, Optical Coherence; Aquaporin 4; Retrospective Studies; Autoantibodies; Retina
PubMed: 38941573
DOI: 10.1212/NXI.0000000000200273 -
PloS One 2024Obesity is a high-morbidity chronic condition and risk factor for multiple diseases that necessitate imaging. This study assesses the relationship between BMI and...
OBJECTIVE
Obesity is a high-morbidity chronic condition and risk factor for multiple diseases that necessitate imaging. This study assesses the relationship between BMI and same-year utilization of CT and MR imaging in a large healthcare population.
METHODS
In this retrospective population-based study, all patients aged ≥18 years with a documented BMI in the multi-institutional Cosmos database were included. Cohorts were identified based on ≥1 documented BMI in 2021 within pre-defined ranges. For each cohort, we assessed the percentage of patients undergoing head, neck, chest, spine, or abdomen/pelvis CT and MR during the same year. Disease severity was quantified based on emergency department (ED) visits and mortality.
RESULTS
In our population of 49.6 million patients, same-year CT and MR utilization was 14.5 ±0.01% and 6.0±0.01%, respectively. The underweight cohort had the highest CT (25.8±0.1%) and MR (8.01 ± 0.05) imaging utilization. At high extremes of BMI (>50 kg/m2), CT utilization mildly increased (18.4±0.1%), but MR utilization decreased (5.3±0.04%). While morbidity differences may explain some BMI-utilization relationships, lower MR utilization in the BMI>50 cohort contrasts with higher age-adjusted mortality (1.8±0.03%) and ED utilization (32.4±0.1%) in this cohort relative to normal weight (1.5±0.01% and 25.7±0.02%, respectively).
CONCLUSION
Underweight patients had disproportionately high CT/MR utilization, and high extremes of BMI are associated with mildly higher CT and lower MR utilization than the normal weight cohort. The elevated mortality and ED utilization in severely obese patients contrasts with their lower MR imaging utilization. Our findings may assist public health efforts to accommodate obesity trends.
Topics: Humans; Body Mass Index; Magnetic Resonance Imaging; Male; Female; Tomography, X-Ray Computed; Middle Aged; Retrospective Studies; Adult; Obesity; Aged; Emergency Service, Hospital; Morbidity
PubMed: 38941332
DOI: 10.1371/journal.pone.0306087