Clinical efficacy and safety of selexipag in children and young adults with idiopathic and heritable pulmonary arterial hypertension.

OBJECTIVE: This study aimed to investigate the safety, tolerability, and efficacy of selexipag in children and young adults with idiopathic and heritable pulmonary arterial hypertension. METHODS: This retrospective cohort study included clinical data from five children and six young adults with pulmonary arterial hypertension receiving selexipag as add-on therapy or as a transition from beraprost sodium or epoprostenol infusion therapy. Clinical efficacy was evaluated by measuring improvement in clinical variables from baseline, including hemodynamic parameters. RESULTS: Of the 11 patients, 6 were switched from beraprost sodium to selexipag and one paediatric patient transitioned from epoprostenol to selexipag. The median maintenance dose of selexipag in children was 80 µg/kg/day. In nine patients undergoing repeat catheterisation, statistically significant improvements were observed after the initiation of selexipag in terms of mean pulmonary arterial pressure (p < 0.01), pulmonary vascular resistance index (p < 0.05), and cardiac index (p < 0.01). None of the patients had clinical worsening after selexipag during follow-up, but one young adult patient discontinued treatment due to severe headache. The most common side effect profiles were headache, nausea, abdominal pain, jaw pain, myalgia, and diarrhoea. CONCLUSIONS: Selexipag may have a favourable safety profile and potential efficacy in children and young adults with pulmonary arterial hypertension.

Clinical differences between children and adults with idiopathic and heritable pulmonary arterial hypertension.

BACKGROUND: Although previous studies have demonstrated that paediatric pulmonary arterial hypertension remains distinct from that in adults, there are limited studies evaluating a direct comparison between children and adults. The aim of this head-to-head comparison study was to compare the gender, haemodynamic parameters, and prognosis between paediatric and adult pulmonary arterial hypertension. METHODS AND RESULTS: We retrospectively assessed the clinical differences in 40 childhood-onset (under 20 years old) patients and 40 adult-onset patients with idiopathic and heritable pulmonary arterial hypertension who were followed up at two centres. There was no female predominance among patients with childhood-onset pulmonary arterial hypertension (child female: 42.5%, adult female: 80%). The percent of New York Heart Association functional class IV in adult-onset pulmonary arterial hypertension tended to be higher than those in childhood-onset pulmonary arterial hypertension (22.5 and 10%, respectively), although children had worse haemodynamic parameters at diagnosis (mean pulmonary artery pressure (children versus adults); median 65 mmHg versus 49 mmHg, p < 0.001). There was no significant difference in the event-free survival rate between the two groups (95% vs. 85%) during the follow-up period (median, 96 months; range, 1-120 months). CONCLUSIONS: Although paediatric pulmonary arterial hypertension patients had worse haemodynamic parameters at diagnosis than adults, children survived as long as adults with appropriate therapeutic strategies.

Polarization-insensitive local-oscillator-carrier loopback modulation for a cost-effective and high-port-count wavelength-routing optical switch.

A wavelength-routing optical switch uses a wavelength-tunable laser at each input port, and this transmitter implements output port selection by tuning the wavelength that is associated with each output port. With coherent transmission, loopback modulation of a local oscillator (LO) carrier generated at the output port can eliminate the need for a wavelength-tunable laser. However, loopback modulation can be unstable since the power fluctuates because fiber traversal by the light creates polarization rotation. Here, we propose a simple polarization-alignment circuit and verify its effectiveness in creating a high-port-count optical switch system. The proposed circuit consists of passive components and aligns the polarization state of the supplied LO carrier to be linearly polarized along the x-direction of a TE-input dual-polarization (DP) IQ modulator. The circuit is shown to yield stable modulation with Q-variation of less than 0.8 dB, regardless of any birefringence along the transmission path. The proposal's effectiveness is verified in optical switch system experiments with DP-QPSK signals; 1,856 × 1,856 switch scale is achieved with loopback modulation.

A case of renal cell carcinoma with late recurrence in the bilateral hilar lymph nodes twenty years after surgery.

Renal cell carcinoma (RCC) is a common malignancy with a high recurrence rate. However, brain and bilateral hilar lymph node (BHL) relapse is rare. A 65-year-old man with a chief complaint of hemosputum visited the primary care clinic. Computed tomography revealed BHL enlargement. Histopathological examination of biopsy specimens from the left lingular bronchus revealed RCC. This finding was similar to that of a left nephrectomy specimen of RCC observed 20 years ago. If patients have a medical history of RCC, physicians should consider the possibility of RCC recurrence, regardless of the number of years relapsed postoperatively.

A case of squamous cell lung cancer treated with anamorelin in combination with a multidisciplinary collaborative approach for treating cancer cachexia.

Anamorelin (ANA) is approved for treating cancer cachexia (CCX) in Japan. We report the case of a 69-year-old man with stage IVB squamous cell lung cancer complicated by CCX, having a 13.6% weight loss in 6 months. After chemotherapy was initiated, his weight was further reduced. Therefore, we started ANA combined with a treatment approach by a multidisciplinary collaboration, including nutritionists and physical therapists. After initiation of ANA, the body weight, appetite, psoas muscle index, and physical functions rapidly improved during chemotherapy. ANA administration combined with a multidisciplinary collaboration approach can be an effective supportive therapy against CCX during chemotherapy.

Design and verification of a LO bank enabled by fixed-wavelength lasers and fast tunable silicon ring filters for creating large scale optical switches.

The fast and widely tunable wavelength bank is a key enabler in creating wavelength-routing optical switches that do not use fast wavelength tunable lasers. A cost-effective design criterion needs to be developed before it can be applied to intra data center networks. In this paper, we develop a systematic method for designing a wavelength bank that yields high port-count and fast wavelength-routing optical switches for intra data center application. The wavelength bank is created with fixed-wavelength laser sources and wavelength-tunable filters with rapid wavelength selectivity. To optimize the optical switching system that uses the wavelength bank for supplying local oscillator (LO) lights for coherent detection, various parameters are analyzed, including effective bandwidth, laser output power, loss distribution, splitter port count, and optical amplifier gain. We carry out numerical simulations for optimizing the tradeoff between system performance and cost. To verify the designed wavelength bank, a silicon ring filter is newly fabricated with an average fiber-to-fiber insertion loss of 5.3 dB over a 22-nm bandwidth. Using 256-Gb/s DP-QPSK signals, experiments demonstrate a 1,024×1,024 optical switch that uses a fabricated silicon ring filter. The effectiveness of the scalable and fast-tunable LO bank is verified by achieving 262.1-Tb/s switch throughput with switching time under 18 µs.

Medicine at mass gatherings: current progress of preparedness of emergency medical services and disaster medical response during 2020 Tokyo Olympic and Paralympic Games from the perspective of the Academic Consortium (AC2020).

Mass gatherings are events characterized by "the concentration of people at a specific location for a specific purpose over a set period of time that have the potential to strain the planning and response resources of the host country or community." Previous reports showed that, as a result of the concentration of people in the limited area, injury and illness occurred due to several factors. The response plan should aim to provide timely medical care to the patients and to reduce the burden on emergency hospitals, and to maintain a daily emergency medical services system for residents of the local area. Although a mass gathering event will place a significant burden on the local health-care system, it can provide the opportunity for long-term benefits of public health-care and improvement of daily medical service systems after the end of the event. The next Olympic and Paralympic Games will be held in Tokyo, during which mass gatherings will occur on a daily basis in the context of the coronavirus disease (COVID-19) epidemic. The Academic Consortium on Emergency Medical Services and Disaster Medical Response Plan during the Tokyo Olympic and Paralympic Games in 2020 (AC2020) was launched 2016, consisting of 28 academic societies in Japan, it has released statements based on assessments of medical risk and publishing guidelines and manuals on its website. This paper outlines the issues and countermeasures for emergency and disaster medical care related to the holding of this big event, focusing on the activities of the academic consortium.