PIEZO1 activation may serve as an early tissue biomarker for the prediction of irradiation-induced salivary gland dysfunction.

Irradiation (IR)-induced xerostomia is the most common side effect of radiation therapy in patients with head and neck cancer (HNC). Xerostomia diagnosis is mainly based on the patient's medical history and symptoms. Currently, no direct biomarkers are available for the early prediction of IR-induced xerostomia. Here, we identified PIEZO1 as a novel predictive tissue biomarker for xerostomia. Our data demonstrate that PIEZO1 is significantly upregulated at the gene and protein levels during IR-induced salivary gland (SG) hypofunction. Notably, PIEZO1 upregulation coincided with that of inflammatory (F4/80) and fibrotic markers (fibronectin and collagen fibers accumulation). These findings suggest that PIEZO1 upregulation in SG tissue may serve as a novel predictive marker for IR-induced xerostomia.

Do toenail manganese and iron levels reflect brain metal levels or brain metabolism in welders?

Inhalation of welding fumes can cause metal accumulation in the brain, leading to Parkinsonian-like symptoms. Metal accumulation and altered neurochemical profiles have been observed using magnetic resonance imaging (MRI) in highly exposed welders, being associated with decreased motor function and cognition. While MRI is impractical to use as a health risk assessment tool in occupational settings, toenail metal levels are easier to assess and have been demonstrated to reflect an exposure window of 7-12 months in the past. Yet, it is unclear whether toenail metal levels are associated with brain metal levels or changes in metabolism, which are the root of potential health concerns. This study investigates whether toenail manganese (Mn) and iron (Fe) levels, assessed at several time points, correlate with brain Mn and Fe levels, measured by MRI, as well as brain GABA, glutamate (Glu), and glutathione (GSH) levels, measured by Magnetic Resonance Spectroscopy (MRS), in seventeen Mn-exposed welders. Quantitative T1 and R2* MRI maps of the whole brain, along with GABA, Glu, and GSH MRS measurements from the thalamus and cerebellum were acquired at baseline (T0). Toenail clippings were collected at T0 and every three months after the MRI for a year to account for different exposure periods being reflected by toenail clippings and MRI. Spearman correlations of toenail metal levels were run against brain metal and metabolite levels, but no significant associations were found for Mn at any timepoint. Cerebellar GSH positively correlated with toenail Fe clipped twelve months after the MRI (p = 0.05), suggesting an association with Fe exposure at the time of the MRI. Neither thalamic GABA nor Glu correlated with toenail Fe levels. In conclusion, this study cannot support toenail Mn as a proxy for brain Mn levels or metabolic changes, while toenail Fe appears linked to brain metabolic alterations, underscoring the importance of considering other metals, including Fe, in studying Mn neurotoxicity.

Blood Fluke Infection (Spirorchidiasis) and Systemic Granulomatous Inflammation: A Case Study of Green Sea Turtles (Chelonia mydas) on Jeju Island, South Korea.

Despite the precarious state of marine turtles as a highly endangered species, our understanding of their diseases remains limited. This case report presents a detailed pathological investigation of spirorchiidiasis, a blood fluke infection that poses a substantial threat to marine turtles. This retrospective study examined three cases of spirorchiid-infected sea turtles, specifically, green sea turtles stranded on Jeju Island, South Korea. Premortem examination of the three spirorchiid-infected green sea turtles demonstrated nonspecific clinical symptoms; blood analysis revealed dehydration, malnutrition, and anemia. Computed tomography scans provided insights into severe pulmonary and extrapulmonary manifestations, including the mass present in the joint region. Post-mortem examinations consistently indicated severe lung lesions and systemic manifestations, with histopathological examination confirming the presence of spirorchiid ova across various organs. Despite the global prevalence of spirorchiidiasis in sea turtles, disease severity varies regionally. This report provides a detailed demonstration of the pathology of spirorchiidiasis in sea turtles from Northeast Asia.

The incidences of nausea and vomiting after general anesthesia with remimazolam versus sevoflurane: a prospective randomized controlled trial.

Background: Postoperative nausea and vomiting (PONV) refers to nausea and vomiting that occurs within 24-h after surgery or in the post-anesthesia care unit (PACU). Previous studies have reported that the use of remimazolam, a newer benzodiazepine (BDZ) hypnotic, for anesthesia results in less PONV. In this study, we compared the rate of PONV between sevoflurane and remimazolam after general anesthesia. Methods: In this prospective randomized controlled trial (RCT), participants aged 20-80 years who underwent elective laparoscopic cholecystectomy or hemicolectomy were randomized to either the remimazolam or sevoflurane group. The primary outcome was PONV incidence for 24-h after surgery. Secondary outcomes comprised of PONV at 30-min post-surgery, postoperative additional antiemetic use, and Quality of Recovery-15 (QOR-15) score at 24-h postoperatively. Results: Forty patients were enrolled in the study. The remimazolam group exhibited significantly lower rates of PONV for 24-h after surgery than did the sevoflurane group (remimazolam group vs. sevoflurane group; 5% vs. 45%, P = 0.003, respectively). The use of dexamethasone, a rescue antiemetic administered within 24 h of surgery, was substantially lower in the remimazolam group than in the sevoflurane group (0% in remimazolam vs. 30% in sevoflurane, P = 0.020). The QOR-15 score at 24-h after surgery showed no significant difference between the two groups. Conclusions: Compared to sevoflurane, opting for remimazolam as an intraoperative hypnotic may decrease the incidence of PONV and reduce antiemetic use for 24 h after laparoscopic surgery.

Effect of dietary heat-killed Lactiplantibacillus plantarum VSG3 on growth, immunity, antioxidant status, and immune-related gene expression in pathogen-aggravated Cyprinus carpio.

Heat-killed probiotics offer an alternative approach to enhance growth and disease resistance in farmed fish. In this study, we isolated Lactiplantibacillus plantarum VSG3 from the gut of Labeo rohita to investigate the effects of heat-killed L. plantarum (HK-LP) on the health and growth performance of Cyprinus carpio fingerlings. Different concentrations of HK-LP (0, 50, 100, 200, 300, and 400 mg/kg) were administered to the fish, followed by a challenge with Aeromonas hydrophila after 8 weeks of feeding. Notably, the LP200 group exhibited significantly improved percentage weight gain and specific growth rate, accompanied by the lowest feed conversion ratio. Post-challenge survival rates were considerably enhanced in the LP200 group, reaching 60.65%. Moreover, serum analysis indicated significantly higher levels of total protein and albumin in the LP200 group than in the control group. Although HK-LP had no substantial impact on certain serum parameters (glucose, total cholesterol, cortisol, and alanine aminotransferase), aspartate aminotransferase levels were considerably low in the LP200 group. Intestinal protease and trypsin activities significantly increased in the LP200 group, while no significant changes were observed in lipase and amylase activities post-pathogen challenge. Serum immunological indices, including lysozyme, alternative complement pathway, and phagocytic activity, improved considerably in the LP200 group. Additionally, serum antioxidant enzyme activities (superoxide dismutase [SOD], glutathione peroxidase [GPx], catalase [CAT], and myeloperoxidase) were significantly elevated in the LP200 group, while malondialdehyde level was reduced. Gene expression analysis in liver tissue indicated strong upregulation of antioxidant-related genes (SOD, CAT, nuclear factor erythroid 2 [NFE2]-related factor 2 [Nrf2], Kelch-like ECH-associated protein 1[Keap1]) in the LP100 and LP200 groups. Pro-inflammatory cytokines (IL-1ß and TNF-α) were considerably downregulated in the kidneys of the LP200 post-challenged fish, although the anti-inflammatory cytokine IL-10 showed an increased expression. Quadratic regression analysis identified the optimal dietary HK-LP level for maximizing growth and immune performance (200.381-270.003 mg/kg). In summary, our findings underscore the potential of HK-LP as a valuable dietary supplement for enhancing carp aquaculture, particularly at the appropriate concentration.

Factors Associated with Reaching Mid-Parental Height in Patients Diagnosed with Inflammatory Bowel Disease in Childhood and Adolescent Period.

Background/Aims: The recent update on the Selecting Therapeutic Targets in Inflammatory Bowel Disease initiative has added normal growth in children as an intermediate target in Crohn's disease and ulcerative colitis. We aimed to investigate factors associated with reaching mid-parental height (MPH) in patients diagnosed with inflammatory bowel disease in childhood and the adolescent period. Methods: This multicenter retrospective observational study included pediatric patients with inflammatory bowel disease that had reached adult height. Factors associated with reaching MPH were investigated by logistic regression analyses. Results: A total of 166 patients were included in this study (128 Crohn's disease and 38 ulcerative colitis). Among them, 54.2% (90/166) had reached their MPH. Multivariable logistic regression analysis revealed that height Z-score at diagnosis and MPH Z-score were independently associated with reaching MPH (odds ratio [OR], 8.45; 95% confidence interval [CI], 4.44 to 17.90; p<0.001 and OR, 0.11; 95% CI, 0.04 to 0.24; p<0.001, respectively). According to the receiver operating characteristic curve analysis, the optimal cutoff level of "height Z-score at diagnosis minus MPH Z-score" that was associated with reaching MPH was -0.01 with an area under the curve of 0.889 (95% CI [0.835 to 0.944], sensitivity 88.9%, specificity 84.2%, positive predictive value 87.0%, negative predictive value 86.5%, p<0.001). Conclusions: Height Z-score at diagnosis and MPH Z-score were the only factors associated with reaching MPH. Efforts should be made to restore growth in pediatric patients who present with a negative "height Z-score at diagnosis minus MPH Z-score."

Effect of statin use on head and neck cancer prognosis in a multicenter study using a Common Data Model.

Few studies have found an association between statin use and head and neck cancer (HNC) outcomes. We examined the effect of statin use on HNC recurrence using the converted Observational Medical Outcome Partnership (OMOP) Common Data Model (CDM) in seven hospitals between 1986 and 2022. Among the 9,473,551 eligible patients, we identified 4669 patients with HNC, of whom 398 were included in the target cohort, and 4271 were included in the control cohort after propensity score matching. A Cox proportional regression model was used. Of the 4669 patients included, 398 (8.52%) previously received statin prescriptions. Statin use was associated with a reduced rate of 3- and 5-year HNC recurrence compared to propensity score-matched controls (risk ratio [RR], 0.79; 95% confidence interval [CI], 0.61-1.03; and RR 0.89; 95% CI 0.70-1.12, respectively). Nevertheless, the association between statin use and HNC recurrence was not statistically significant. A meta-analysis of recurrence based on subgroups, including age subgroups, showed similar trends. The results of this propensity-matched cohort study may not provide a statistically significant association between statin use and a lower risk of HNC recurrence. Further retrospective studies using nationwide claims data and prospective studies are warranted.

Self-organized insulin-producing ß-cells differentiated from human omentum-derived stem cells and their in vivo therapeutic potential.

BACKGROUND: Human omentum-derived mesenchymal stem cells (hO-MSCs) possess great potential to differentiate into multiple lineages and have self-renewal capacity, allowing them to be utilized as patient-specific cell-based therapeutics. Although the use of various stem cell-derived ß-cells has been proposed as a novel approach for treating diabetes mellitus, developing an efficient method to establish highly functional ß-cells remains challenging. METHODS: We aimed to develop a novel cell culture platform that utilizes a fibroblast growth factor 2 (FGF2)-immobilized matrix to regulate the adhesion and differentiation of hO-MSCs into insulin-producing ß-cells via cell-matrix/cell-cell interactions. In our study, we evaluated the in vitro differentiation potential of hO-MSCs cultured on an FGF2-immobilized matrix and a round-bottom plate (RBP). Further, the in vivo therapeutic efficacy of the ß-cells transplanted into kidney capsules was evaluated using animal models with streptozotocin (STZ)-induced diabetes. RESULTS: Our findings demonstrated that cells cultured on an FGF2-immobilized matrix could self-organize into insulin-producing ß-cell progenitors, as evident from the upregulation of pancreatic ß-cell-specific markers (PDX-1, Insulin, and Glut-2). Moreover, we observed significant upregulation of heparan sulfate proteoglycan, gap junction proteins (Cx36 and Cx43), and cell adhesion molecules (E-cadherin and Ncam1) in cells cultured on the FGF2-immobilized matrix. In addition, in vivo transplantation of differentiated ß-cells into animal models of STZ-induced diabetes revealed their survival and engraftment as well as glucose-sensitive production of insulin within the host microenvironment, at over 4 weeks after transplantation. CONCLUSIONS: Our findings suggest that the FGF2-immobilized matrix can support initial cell adhesion, maturation, and glucose-stimulated insulin secretion within the host microenvironment. Such a cell culture platform can offer novel strategies to obtain functional pancreatic ß-cells from patient-specific cell sources, ultimately enabling better treatment for diabetes mellitus.

Compositional variations in metal nanoparticle components of welding fumes impact lung epithelial cell toxicity.

Welding fumes contain harmful metals and gas by-products associated with development of lung dysfunction, asthma, bronchitis, and lung cancer. Two prominent welding fume particulate metal components are nanosized iron (Fe) and manganese (Mn) which might induce oxidative stress and inflammation resulting in pulmonary injury. Welding fume toxicity may be dependent upon metal nanoparticle (NP) components. To examine toxicity of welding fume NP components, a system was constructed for controlled and continuous NP generation from commercial welding and customized electrodes with varying proportions of Fe and Mn. Aerosols generated consisted of nanosized particles and were compositionally consistent with each electrode. Human alveolar lung A459 epithelial cells were exposed to freshly generated metal NP mixtures at a target concentration of 100 µg/m3 for 6 hr and then harvested for assessment of cytotoxicity, generation of reactive oxygen species (ROS), and alterations in the expression of genes and proteins involved in metal regulation, inflammatory responses, and oxidative stress. Aerosol exposures decreased cell viability and induced increased ROS production. Assessment of gene expression demonstrated variable up-regulation in cellular mechanisms related to metal transport and storage, inflammation, and oxidative stress based upon aerosol composition. Specifically, interleukin-8 (IL-8) demonstrated the most robust changes in both transcriptional and protein levels after exposure. Interleukin-8 has been determined to serve as a primary cytokine mediating inflammatory responses induced by welding fume exposures in alveolar epithelial cells. Overall, this study demonstrated variations in cellular responses to metal NP mixtures suggesting compositional variations in NP content within welding fumes may influence inhalation toxicity.

Efficacy and safety of oblique posterior endplate resection for wider decompression (trumpet-shaped decompression) during anterior cervical discectomy and fusion.

OBJECTIVE: Anterior cervical discectomy and fusion (ACDF) provides a limited workspace, and surgeons often need to access the posterior aspect of the vertebral body to achieve sufficient decompression. Oblique resection of the posterior endplate (trumpet-shaped decompression [TSD]) widens the workspace, enabling removal of lesions behind the vertebral body. This study was conducted to evaluate the efficacy and safety of oblique posterior endplate resection for wider decompression. METHODS: In this retrospective study, 227 patients who underwent ACDF for the treatment of cervical myelopathy or radiculopathy caused by spondylosis or ossification of the posterior longitudinal ligament and were followed up for ≥ 1 year were included. Patient characteristics, fusion rates, subsidence, and patient-reported outcome measures, including the neck pain visual analog scale (VAS) score, arm pain VAS score, and Neck Disability Index (NDI), were assessed. Patients who underwent TSD during ACDF (TSD group) and those who underwent surgery without TSD (non-TSD group) were compared. RESULTS: Fifty-seven patients (25.1%) were included in the TSD group and 170 patients (74.9%) in the non-TSD group. In the TSD group, 28.2% ± 5.5% of the endplate was resected, and 26.0% ± 6.1% of the region behind the vertebral body could be visualized via the TSD technique. The resection angle was 26.9° ± 5.9°. The fusion rate assessed on the basis of interspinous motion, intragraft bone bridging, and extragraft bone bridging did not significantly differ between the two groups. Furthermore, there were no significant intergroup differences in subsidence. The patient-reported outcome measures at the 1-year follow-up were also not significantly different between the groups. CONCLUSIONS: TSD widened the workspace during ACDF, and 26% of the region posterior to the vertebral body could be accessed using this technique. The construct stability was not adversely affected by TSD as demonstrated by the similar fusion and subsidence rates among patients who underwent TSD and those who did not. Therefore, TSD can be safely applied during ACDF when compressive lesions extend behind the vertebral body and are not limited to the disc space, enabling adequate decompression without disrupting the construct stability.

Fecal Calprotectin Assay at an Early Stage of Treatment Can Be Used as a Surrogate Marker to Predict Clinical Remission and Mucosal Healing in Pediatric Crohn's Disease.

Purpose: This study evaluated the predictive role of fecal calprotectin (FC) measured at an early stage of treatment for monitoring clinical remission (CR) after six months and endoscopic remission (ER) after one year of treatment in pediatric Crohn's disease (CD). Methods: This retrospective study included 45 patients who simultaneously underwent ileocolonoscopy and FC testing during follow-up. FC levels were measured before and after six weeks of treatment. CR was assessed after six months of treatment using Pediatric Crohn' s Disease Activity Index and acute-phase reactants. ER was assessed after one year using the Simple Endoscopic Score for Crohn's Disease. Results: Twenty-nine (64.4%) patients used oral prednisolone for remission induction and 16 (35.6%) patients used anti-tumor necrosis factor-alpha. Thirty (66.7%) patients achieved CR, while 24 (53.3%) achieved ER. The FC level measured after six weeks of treatment could predict CR (χ2=9.15, p=0.0025) and ER (χ2=12.31, p=0.0004). The δFC could predict CR (χ2=7.91, p=0.0049), but not ER (χ2=1.85, p=0.1738). With a threshold of ≤950.4 µg/g, FC at week six could predict CR with 76.7% sensitivity and 73.3% specificity. The area under the curve (AUC) was 0.769 (standard error 0.0773, p=0.0005). The same threshold predicted ER with 87.5% sensitivity and 71.4% specificity. The AUC was 0.774 (standard error 0.074, p=0.0002). Conclusion: FC assay at an early stage of treatment can be used as a surrogate marker to predict CR and mucosal healing in pediatric CD.

Epidemiological Trends of Pediatric Inflammatory Bowel Disease in Korea: A Multicenter Study of the Last 3 Years Including the COVID-19 Era.

BACKGROUND: Studies on how the coronavirus pandemic has affected pediatric inflammatory bowel disease (PIBD) are lacking. We aimed to investigate the trends in epidemiology, characteristics, initial management, and short-term outcomes of PIBD in South Korea over the recent three years including the era of coronavirus disease 2019 (COVID-19). METHODS: This multicenter study retrospectively investigated temporal trends in the epidemiology of PIBD in Korea. Annual occurrences, disease phenotypes, and initial management at diagnosis were analyzed from January 2018 to June 2021. RESULTS: A total of 486 patients from 17 institutions were included in this epidemiological evaluation. Analysis of the occurrence trend confirmed a significant increase in PIBD, regardless of the COVID-19 pandemic. In Crohn's disease, patients with post-coronavirus outbreaks had significantly higher fecal calprotectin levels than those with previous onset (1,339.4 ± 717.04 vs. 1,595.5 ± 703.94, P = 0.001). Patients with post-coronavirus-onset ulcerative colitis had significantly higher Pediatric Ulcerative Colitis Activity Index scores than those with previous outbreaks (48 ± 17 vs. 36 ± 15, P = 0.004). In the initial treatment of Crohn's disease, the use of 5-aminosalicylic acid (5-ASA) and steroids significantly decreased (P = 0.006 and 0.001, respectively), and enteral nutrition and the use of infliximab increased significantly (P = 0.045 and 0.009, respectively). There was a significant increase in azathioprine use during the initial treatment of ulcerative colitis (P = 0.020). CONCLUSION: Regardless of the COVID-19 pandemic, the number of patients with PIBD is increasing significantly annually in Korea. The initial management trends for PIBD have also changed. More research is needed to establish appropriate treatment guidelines considering the epidemiological and clinical characteristics of Korean PIBD.

Uncompetitive, adduct-forming SARM1 inhibitors are neuroprotective in preclinical models of nerve injury and disease.

Axon degeneration is an early pathological event in many neurological diseases. The identification of the nicotinamide adenine dinucleotide (NAD) hydrolase SARM1 as a central metabolic sensor and axon executioner presents an exciting opportunity to develop novel neuroprotective therapies that can prevent or halt the degenerative process, yet limited progress has been made on advancing efficacious inhibitors. We describe a class of NAD-dependent active-site SARM1 inhibitors that function by intercepting NAD hydrolysis and undergoing covalent conjugation with the reaction product adenosine diphosphate ribose (ADPR). The resulting small-molecule ADPR adducts are highly potent and confer compelling neuroprotection in preclinical models of neurological injury and disease, validating this mode of inhibition as a viable therapeutic strategy. Additionally, we show that the most potent inhibitor of CD38, a related NAD hydrolase, also functions by the same mechanism, further underscoring the broader applicability of this mechanism in developing therapies against this class of enzymes.

Effect of performing preoperative echocardiography in patients with cardiovascular risk on intraoperative anesthetic management and postoperative outcomes: A retrospective study.

Although echocardiography is widely used for preoperative cardiac risk evaluation, few studies have analyzed the effect of performing preoperative echocardiography on intraoperative anesthetic management and postoperative outcomes. We investigated the effect of performing echocardiography on intraoperative anesthetic management and postoperative outcomes in patients with cardiovascular risk. We retrospectively evaluated patients who had undergone major abdominal surgery and satisfied 2 or more of the following criteria: hypertension, diabetes mellitus, age ≥70 years, and previous cardiac disease. Patients were categorized into a group in which preoperative echocardiography was performed (echo) and a group in which it was not (non-echo). The primary outcomes were postoperative 30-day mortality and incidence of cardiovascular complications. Secondary outcomes were length of hospital stay, intraoperative incidence of hypotension, use of vasopressors, and findings on intraoperative invasive hemodynamic monitoring. There were no differences in 30-day mortality, incidence of postoperative cardiovascular complications, length of hospital stay, and intraoperative events between the groups. Only the incidence of cardiac output monitoring was lower in the echo group than in the non-echo group (59.6% vs 73.9%). Preoperative echocardiography does not affect postoperative outcomes, but it has the potential to affect intraoperative anesthetic management such as invasive hemodynamic monitoring during surgery.

Silymarin seed extract supplementation enhances the growth performance, meat quality, and nutrients digestibility, and reduces gas emission in broilers.

OBJECTIVE: A feeding trial was carried out to determine the effect of dietary inclusion of silymarin seed extract on growth performance, nutrient digestibility, excreta microbiota, excreta gas emission, blood profiles, and meat quality in broilers. METHODS: A total of 1,088 one-day-old Ross 308 broiler chicks (mixed-sex) with an initial body weight of 42.34±0.82 g, were randomly allocated into 1 of 4 dietary treatments with 17 replicates of 16 chicks per cage and fed a basal diet supplemented with 0%, 0.02%, 0.04%, and 0.06% of silymarin. RESULTS: The inclusion of silymarin supplementation linearly increased the body weight of broilers during days 7 to 21 and 1 to 35 days. On day 35, broilers fed a diet containing graded levels of silymarin supplementation linearly increased the nutrient digestibility of dry matter, gross energy, and nitrogen and cecal Lactobacillus counts (p = 0.038). While silymarin supplement linearly reduced the methyl mercaptans (p = 0.039) and acetic acid (p = 0.007) emission in broilers. No significant effects were observed on the blood profile. Relative weights of organs such as breast muscle, bursa of fabricius were increased (linear effect, p<0.05), water holding capacity was enhanced by increasing the silymarin level from 0% to 0.06%. A linear reduction (p>0.05) in drip loss from meat samples during days 1, 3, 5, and 7 by the addition of graded level of silymarin to the diet. CONCLUSION: An increasing level of silymarin supplementation to the diet of broiler would be beneficial to enhance growth performance, nutrient digestibility, excreta microflora, blood profile, and meat quality traits.

Image quality and diagnostic accuracy of reduced-dose computed tomography enterography with model-based iterative reconstruction in pediatric Crohn's disease patients.

This study assessed the image quality and diagnostic accuracy in determining disease activity of the terminal ileum of the reduced-dose computed tomography enterography using model-based iterative reconstruction in pediatric patients with Crohn's disease (CD). Eighteen patients were prospectively enrolled and allocated to the standard-dose (SD) and reduced-dose (RD) computed tomography enterography (CTE) groups (n = 9 per group). Image quality, reader confidence in interpreting bowel findings, accuracy in determining active CD in the terminal ileum, and radiation dose were evaluated. Objective image quality did not show intergroup differences, except for image sharpness. Although reader confidence in detecting mural stratification, ulcer, and perienteric fat stranding of the RD-CTE were inferior to SD-CTE, RD-CTE correctly diagnosed active disease in all patients. The mean values of radiation dose metrics (SD-CTE vs. RD-CTE) were 4.3 versus 0.74 mGy, 6.1 versus 1.1 mGy, 211.9 versus 34.5 mGy∙cm, and 4.4 versus 0.7 mSv mGy∙cm for CTDIvol, size-specific dose estimation, dose-length product, and effective dose, respectively. RD-CTE showed comparable diagnostic accuracy to SD-CTE in determining active disease of the terminal ileum in pediatric CD patients. However, image quality and reader confidence in detecting ulcer and perienteric fat stranding was compromised.

Phenotypic heterogeneity in patients with NEFL-related Charcot-Marie-Tooth disease.

Charcot-Marie-Tooth disease (CMT) is the most common hereditary peripheral neuropathy. Mutations in the neurofilament light polypeptide (NEFL) gene produce diverse clinical phenotypes, including demyelinating (CMT1F), axonal (CMT2E), and intermediate (CMTDIG) neuropathies. From 2005 to 2020, 1,143 Korean CMT families underwent gene sequencing, and we investigated the clinical, genetic, and neuroimaging spectra of NEFL-related CMT patients. Ten NEFL mutations in 17 families (1.49%) were identified, of which three (p.L312P, p.Y443N, and p.K467N) were novel. Eight de novo cases were identified at a rate of 0.47 based on a cosegregation analysis. The age of onset was ≤3 years in five cases (13.5%). The patients revealed additional features including delayed walking, ataxia, dysphagia, dysarthria, dementia, ptosis, waddling gait, tremor, hearing loss, and abnormal visual evoked potential. Signs of ataxia were found in 26 patients (70.3%). In leg MRI analyses, various degrees of intramuscular fat infiltration were found. All compartments were evenly affected in CMT1F patients. The anterior and anterolateral compartments were affected in CMT2E, and the posterior compartment was affected in CMTDIG. Thus, NEFL-related CMT patients showed phenotypic heterogeneities. This study's clinical, genetic, and neuroimaging results could be helpful in the evaluation of novel NEFL variants and differential diagnosis against other CMT subtypes.

Early-onset persistent type IIIb endoleak following incraft stent graft implantation in an abdominal aortic aneurysm patient.

The type IIIb endoleak a rare complication of endovascular aneurysmal repair caused by endoprosthesis problem leading to deteriorating rupture, which is one of the major reasons for lifelong imaging surveillance. We report a case of an early-onset type IIIb endoleak from a tear in main body bifurcation (flow divider) area like crotch point tearing of pants following additional intervention to reduce immediate type Ia endoleak. Successful treatment of the endoleak was achieved by surgical conversion.

Aerosol and Droplet Generation from Open Rhinoplasty: Surgical Risk in the Pandemic Era.

Introduction: The coronavirus disease 2019 pandemic has led to concerns over transmission risk from head and neck operations including facial cosmetic surgeries. Objectives: To quantify droplet and aerosol generation from rhinoplasty techniques in a human anatomic specimen model using fluorescein staining and an optical particle sizer. Methods: Noses of human anatomic specimens were infiltrated using 0.1% fluorescein. Droplets and aerosols were measured during rhinoplasty techniques including opening the skin-soft tissue envelope, monopolar electrocautery, endonasal rasping, endonasal osteotomy, and percutaneous osteotomy. Results: No visible droplet contamination was observed for any rhinoplasty techniques investigated. Compared with the negative control of anterior rhinoscopy, total 0.300-10.000 µm aerosols were increased after monopolar electrocautery (p < 0.001) and endonasal rasp (p = 0.003). Opening the skin-soft tissue envelope, endonasal osteotomies, and percutaneous osteotomies did not generate a detectable increase in aerosols (p > 0.15). Discussion and Conclusions: In this investigation, droplets were not observed under ultraviolet light, and aerosol generation was noted only with cautery and endonasal rasping.

Clinical and Neuroimaging Features in Charcot-Marie-Tooth Patients with GNB4 Mutations.

Charcot-Marie-Tooth disease (CMT) is the most common inherited peripheral neuropathy. Mutations in the GNB4 gene cause dominant intermediate CMT type F (CMTDIF). The aim of this study is to investigate phenotypic heterogeneities and characteristics of CMT patients with GNB4 mutations. We enrolled 1143 Korean CMT families and excluded 344 families with a PMP22 duplication. We further analyzed the 799 remaining families to find their GNB4 mutations using whole-exome sequencing (WES). We identified two mutations (p.Gly77Arg and p.Lys89Glu) in three families, among which a heterozygous p.Gly77Arg mutation was novel. In addition, a significant uncertain variant (p.Thr177Asn) was observed in one family. The frequency of the GNB4 mutation in the Korean population is 0.38% in PMP22 duplication-negative families. All three families showed de novo mutation. Electrophysiological findings regarding the p.Lys89Glu mutation showed that the motor nerve conduction velocity (MNCV) of the median nerve was markedly reduced, indicating demyelinating neuropathy, and sural nerve biopsy revealed severe loss of myelinated axons with onion bulb formation. Lower extremity Magnetic Resonance Imaging (MRI) demonstrated relatively more severe intramuscular fat infiltrations in demyelinating type (p.Lys89Glu mutation) patients compared to intermediate type (p.Gly77Arg mutation) patients. The anterolateral and superficial posterior compartment muscles of the distal calf were preferentially affected in demyelinating type patients. Therefore, it seems that the investigated GNB4 mutations do cause not only the known intermediate type but also demyelinating-type neuropathy. We first presented three Korean families with GNB4 mutations and found phenotypic heterogeneities of both intermediate and demyelinating neuropathy. We suggest that those findings are useful for the differential diagnosis of CMT patients with unknown GNB4 variants.