Search

Abstract Background Dravet syndrome is a severe epilepsy disorder characterized by drug-resistant seizures and cognitive dysfunction, often caused by SCN1A gene mutations. It leads to neurodevelopmental delays and motor, behavioral, and cognitive impairments, with a high mortality rate. Treatment options include sodium valproate, clobazam, and newer agents such as cannabidiol and fenfluramine. Zonisamide, which is used in some cases, can cause hyperthermia and oligohydrosis. Herein, we present a case of a patient with Dravet syndrome whose seizures were controlled by treating infections and switching from zonisamide to perampanel. Case presentation A 24-year-old Japanese man with Dravet syndrome presented to our department with aspiration pneumonia. The patient had been treated with valproate, sodium bromide, and zonisamide for a long time. His seizures were triggered by hyperthermia. The patient was experiencing a sustained pattern of hyperthermia caused by infection, zonisamide, and persistent convulsions, which caused a vicious cycle of further seizures. In this case, the control of infection and switching from zonisamide to perampanel improved seizure frequency. Conclusion Dravet syndrome usually begins with generalized clonic seizures in its infancy because of fever and progresses to various seizure types, often triggered by fever or seizure-induced heat due to mutations in the SCN1A gene that increases neuronal excitability. Seizures usually diminish with age, but the heat sensitivity remains. In this case, seizures were increased by repeated infections, and hyperthermia was induced by zonisamide, resulting in status epilepticus. Perampanel, an aminomethylphosphonic acid receptor antagonist, decreased seizures but caused psychiatric symptoms. It was effective in suppressing seizures of Dravet syndrome in this patient.

A 72-year-old female diagnosed with rectal cancer treated with a surgical procedure was reported. As 3 liver metastases (LMs) appeared in multidetector CT, adjuvant chemotherapy using Bevacizumab combined with modified FOLFOX-6 was completed. LMs were changed to cystic lesions during the follow-up period, consistent with liquefactive necrosis. These cystic lesions that appeared in the course of disappearing LMs (DLMs) were identified by CT as homogeneous low signal intensity in hepatocyte specific Gd-enhanced MRI. This might be pathognomonic radiological footprint equivalent to liquefactive necrosis observed in the process of DLM and must be carefully followed in the course of radiological complete response. The radiological changing findings of LMs to cystic changes, high sensitivity of detecting DLM, and limitations of Gd-MRI might be meaningful to clinicians.

ABSTRACT: Background: Recent data from clinical trials suggest that antibody cocktail therapy, which combined casirivimab and imdevimab, is linked to the reduction of the risk of hospitalization or death among high-risk patients with COVID-19. However, it remains unclear how effective the therapy is in a real-life clinical practice. Methods: We retrospectively analyzed patients with COVID-19 with high-risk factors who underwent the antibody cocktail therapy, compared with those who were not given the cocktail therapy while being isolated in nonmedical facilities during the same period. Results: Data from 55 patients who received the antibody cocktail therapy and 53 patients with initial isolation in nonmedical facilities were analyzed. A total of 22 (41.5 %) of 53 patients staying in isolation facilities were eventually hospitalized and received medical interventions. By contrast, 13 (23.6 %) of 55 patients who received the antibody cocktail therapy subsequently underwent further medical interventions. In multivariate analysis, the antibody cocktail therapy significantly reduced the need for further medical interventions by 70 % compared with isolation (odds ratio=0.30, 95%CI [0.10-0.87], p=0.027). Patients with percutaneous oxygen saturation 96% or higher were significantly favoured for the therapy and had an advantage. Conclusion: The results of this study indicate that the antibody cocktail therapy is associated with reducing burden on hospitals during the COVID-19 pandemic.

Modern aquaculture methods help fish get better potential in their growth and development. In the last decades, people have gained an interest in fish welfare aspects such as stress, which strongly affect fish performance. However, to grasp such a critical but complicated stress response is problematic easily affected by external factors. To this end, we developed a wireless biosensor system for fish stress monitoring. This system can monitor the glucose level (stress indicator) in fish eyeball scleral interstitial fluid using the proposed amperometry glucose biosensor based on glucose oxidase immobilized on the Pt–Ir electrode surface. In the presence of glucose, a typical catalytic oxidation current could be observed at +650 mV. Besides, the system shows the different stress statuses of fish by using a multi-color LED like a traffic light. The LED color-switching points, however, had to be manually calibrated. Besides, the process itself may induce stress to the fish. In this study, a novel interactive biosensor system that introduces bi-directional communication technology has been introduced. Datalink functions such as remote calibration and individual identification were advocated and checked. Also, some system characteristic evaluation tests were conducted in vitro and in vivo using Nile tilapia Oreochromis niloticus as test fish and got some satisfactory results. According to the results, we believe our proposed system could handle various stress monitoring intelligently with only some clicks on the software without unnecessarily handling the test fish.

The acetabular labrum enhances hip joint stability and plays a key role in osteoarthritis (OA) progression. Labral nerve endings contribute to hip OA pain. Moreover, vascular endothelial growth factor (VEGF) and nerve growth factor (NGF) are associated with pain. Consequently, we analysed VEGF and NGF expression levels in the labrum and their roles in OA. Labra obtained from OA patients were stained immunohistochemically, and labral cells were cultured and subjected to a reverse transcription (RT)–polymerase chain reaction (PCR) to analyse VEGF and NGF mRNA expression. VEGF and NGF expression were compared in each region of the labrum. Correlations between VEGF and NGF expression and age, body mass index, Kellgren–Lawrence grade, Harris Hip Score, the visual analogue scale (VAS), and Krenn score were analysed, and the RT-PCR confirmed the findings. VEGF and NGF expression were high on the labral articular side, negatively correlated with the Krenn score, and positively correlated with the VAS in early OA. VEGF and NGF mRNA expression increased significantly in patients with severe pain and decreased significantly in severely degenerated labra. In early OA, VEGF and NGF expression in the acetabular labrum was associated with the occurrence of hip pain; therefore, these factors could be effective targets for pain management.

Osteoarthritis (OA) occurs not only in the knee but also in peripheral joints throughout the whole body. Previously, we have shown that the expression of cellular communication network factor 3 (CCN3), a matricellular protein, increases with age in knee articular cartilage, and the misexpression of CCN3 in cartilage induces senescence-associated secretory phenotype (SASP) factors, indicating that CCN3 promotes cartilage senescence. Here, we investigated the correlation between CCN3 expression and OA degenerative changes, principally in human femoral head cartilage. Human femoral heads obtained from patients who received total hip arthroplasty were categorized into OA and femoral neck fracture (normal) groups without significant age differences. Gene expression analysis of RNA obtained from femoral head cartilage revealed that CCN3 and MMP-13 expression in the non-weight-bearing part was significantly higher in the OA group than in the normal group, whereas the weight-bearing OA parts and normal cartilage showed no significant differences in the expression of these genes. The expression of COL10A1, however, was significantly higher in weight-bearing OA parts compared with normal weight-bearing parts, and was also higher in weight-bearing parts compared with non-weight-bearing parts in the OA group. In contrast, OA primary chondrocytes from weight-bearing parts showed higher expression of CCN3, p16, ADAMTS4, and IL-1β than chondrocytes from the corresponding normal group, and higher ADAMTS4 and IL-1β in the non-weight-bearing part compared with the corresponding normal group. Acan expression was significantly lower in the non-weight-bearing group in OA primary chondrocytes than in the corresponding normal chondrocytes. The expression level of CCN3 did not show significant differences between the weight-bearing part and non-weight-bearing part in both OA and normal primary chondrocytes. Immunohistochemical analysis showed accumulated CCN3 and aggrecan neoepitope staining in both the weight-bearing part and non-weight-bearing part in the OA group compared with the normal group. The CCN3 expression level in cartilage had a positive correlation with the Mankin score. X-ray analysis of cartilage-specific CCN3 overexpression mice (Tg) revealed deformation of the femoral and humeral head in the early stage, and immunohistochemical analysis showed accumulated aggrecan neoepitope staining as well as CCN3 staining and the roughening of the joint surface in Tg femoral and humeral heads. Primary chondrocytes from the Tg femoral head showed enhanced expression of Ccn3, Adamts5, p16, Il-6, and Tnfα, and decreased expression of Col2a1 and -an. These findings indicate a correlation between OA degenerative changes and the expression of CCN3, irrespective of age and mechanical loading. Furthermore, the Mankin score indicates that the expression level of Ccn3 correlates with the progression of OA.

Abstract Background Inadequate acetabular component orientation is associated with postoperative impingement, dislocation, and accelerated polyethylene wear. Computed tomography (CT)-based navigation systems provide accuracy for total hip arthroplasty (THA) but are not available in all facilities. Accelerometer-based navigation systems are inexpensive, but their accuracy remains undetermined. This study compares the accuracy of cup orientation in THA using CT-based and accelerometer-based navigation systems. Methods This retrospective study included 35 consecutive patients (11 males, 24 females; mean age, 65 years) who underwent primary cementless THA via an anterolateral approach in the supine position. Both CT-based and accelerometer-based navigation systems were used simultaneously. The accuracy of cup orientation was compared between the two systems using postoperative CT. Results The accuracy of cup inclination was 2.7° ± 2.0° in the CT-based group and 3.3° ± 2.4° in the accelerometer-based group. The accuracy of cup anteversion was 2.8° ± 2.6° in the CT-based group and 3.4° ± 2.2° in the accelerometer-based group. No significant difference was observed in cup inclination (p = 0.29) or cup anteversion (p = 0.34) between CT-based and accelerometer-based navigation. Conclusions The accuracy of cup positioning did not differ significantly between CT-based and accelerometer-based navigation systems.

A short-range terahertz (THz) wireless transmission in the 300 GHz band is demonstrated using low-profile wavelength-scaled dielectric transmitting and receiving cuboid antennas (DCAs). These dielectric cuboid antennas are made of polytetrafluoroethylene with dimensions of approximately 1.2 mm × 1.2 mm × 1.3 mm. The near-field pattern of a DCA at 300 GHz was measured using an electro-optic sensing technique, and its far-field pattern characterization was based on the near-field to far-field transformation. The measured antenna gain was 15.06 ± 0.06 dBi. By employing DCAs as transmitting and receiving antennas, a 17.5 Gbps data transmission rate at distances of approximately 200 and 50 mm with bit error rates of 3.31 × 10–3 and 7.51 × 10–7 respectively, is demonstrated. The proposed mesoscopic scale DCA is a promising antenna type in intra-device communications and Kiosk download applications for future mobile devices operating in the 300 GHz band.

Objective In this study, we aimed to elucidate the relationship between the duration from diagnosis to femoral head collapse and the collapse rate among patients with pre-collapse osteonecrosis of the femoral head (ONFH). Methods In this retrospective, observational, multicenter study, we analyzed 268 patients diagnosed with ONFH and classified them using the Japanese Investigation Committee classification. The primary endpoint was duration from the time of diagnosis to femoral head collapse for each type of ONFH. Results The 12-, 24-, and 36-month collapse rates among participants were 0%, 0%, and 0% for type A, respectively; 0%, 2.0%, and 10.8% for type B, respectively; 25.5%, 40.8%, and 48.5% for type C-1, respectively; and 57.4%, 70.3%, and 76.7% for type C-2 ONFH, respectively. A comparison of unilateral and bilateral ONFH, using Kaplan–Meier survival curves demonstrated similar collapse rates. Conclusions The lowest collapse rate was observed for ONFH type A, followed by types B, C-1, and C-2. Additionally, a direct association was observed between the collapse rate and location of the osteonecrotic lesion on the weight-bearing surface.

Abstract Background In cases of avulsion fracture of the ischial tuberosity in which the bone fragments are substantially displaced, nonunion may cause pain in the ischial area. Various surgical procedures have been reported, but achieving sufficient fixation strength is difficult. Case presentation We treated a 12-year-old male track-and-field athlete with avulsion fracture of the ischial tuberosity by suture anchor fixation using the suture bridge technique. The boy felt pain in the left gluteal area while running. Radiography showed a left avulsion fracture of the ischial tuberosity with approximately 20-mm displacement. Union was not achieved by conservative non-weight-bearing therapy, and muscle weakness persisted; therefore, surgery was performed. A subgluteal approach was taken via a longitudinal incision in the buttocks, and the avulsed fragment was fixed with five biodegradable suture anchors using the suture bridge technique. Conclusions Although the majority of avulsion fractures of the ischial tuberosity can be treated conservatively, patients with excessive displacement require surgical treatment. The suture bridge technique provided secure fixation and enabled an early return to sports activities.

Acetabular bone cyst is usually associated with dysplastic secondary osteoarthritis of the hip joint. Acetabular reorientation osteotomy is one of the most common therapeutic options for treating patients with hip dysplasia. However, it may be too invasive for athletes and can also lead to some complications including intraoperative fracture and postoperative bone necrosis especially in patients with acetabular large bone cysts. Endoscopic shelf acetabuloplasty offers a less invasive option for athletic patients with dysplasia of severity greater than those indicated for isolated hip arthroscopy. This Technical Note demonstrates endoscopic shelf acetabuloplasty with bone grafting, simultaneously addressing a large bone cyst and acetabular undercoverage, as part of a comprehensive approach addressing chondrolabral pathology while restoring capsular integrity in an athlete with moderate dysplasia.

Germ cells are the only cells that can transfer genetic materials to the next generation via the sperm or egg. However, recent analyses in teleosts revealed another essential role of germ cells: feminizing the gonads. In our study, medaka mutants in which gametogenesis was blocked at specific stages provides the novel view that the feminizing effect of germ cells occurs in parallel with other reproductive elements, such as meiosis, the sexual fate decision of germ cells, and gametogenesis. Germ cells in medaka may have a potential to feminize gonads at the moment they have developed.

Paraneoplastic syndromes of hepatocellular carcinoma (HCC) are not uncommon. However, the prognosis is poor and follow-up and improvement of paraneoplastic syndromes with treatment have been reported rarely. We report a successful case in an aged man of a massive HCC with paraneoplastic syndrome, treated by combined intraarterial chemotherapy and hepatic resection. Paraneoplastic syndrome (erythrocytosis and hyperlipidemia) was monitored throughout the treatment and erythropoietin (EPO) mRNA also was analyzed in the resected liver. The hemoglobin level and serum levels of EPO and total cholesterol (T-cho) decreased dramatically with treatment, along with a decrease in serum levels of α-fetoprotein and protein induced by vitamin vitamin K absence II (PIVKA-II). Semiquantitative reverse transcription polymerase chain reaction (RT-PCR) revealed that the residual cancer expressed EPO RNA but the nontumor tissue did not. This was a rare case of paraneoplastic syndrome of HCC that was treated successfully. This case indicates that paraneoplastic syndrome reflected tumor progression and that serum levels of both EPO and T-cho might be used as tumor markers.

Hexagonal boron nitride nanoparticles were successfully prepared by laser ablation in liquid. A nanosecond laser at the second harmonic of Nd:YAG was used for the preparation, and they were evaluated by scanning electron microscopy, X-ray diffraction, and dynamic light scattering. Increasing the laser fluence reduced the secondary particle size, yielding particles of 379 nm. The hightemperature laser process did not cause any phase change, and hexagonal boron nitride nanoparticles were obtained. Decreasing the amount of sodium hydroxide solution added to the solvent of laser ablation reduced the amount of agglomeration and reduced the secondary particle size, yielding nanoparticles of 189 nm. The zeta potential of the prepared nanoparticle was -62 mV and the primary particle diameter was 25 nm. [ABSTRACT FROM AUTHOR]