Language：Japanese   Publisher：Respiratory Investigation  

Background: This study evaluated trends in the cumulative incidence of airflow limitation (AFL) and lung function decline over 17 years in two independent cohorts. Furthermore, this study focused on the possible effects of legal restrictions that came into effect in 2006 to prevent unwanted exposure to passive smoke on lung function decline in Japan. Methods: Cohort 1 (1994–2011) and Cohort 2 (2002–2019) included 590 and 1012 participants, respectively. All participants were free of AFL at baseline, and they had no personal relationships each other. The association between the longitudinal category of smoking status and the cumulative incidence of AFL was assessed using logistic regression analysis. The annual FEV<inf>1</inf> decline was compared between Cohort 1 and 2, as well as before 2006 (pre) and after 2006 (post) within both cohorts. Results: After adjusting for confounding factors, the odds ratio (OR) for developing AFL increased with smoking status and intensity in both Cohort 1 and 2. The annual FEV<inf>1</inf> decline in Cohort 2 was significantly slower than that in Cohort 1 for persistent never smokers. The annual decline in FEV<inf>1</inf> after 2006 (post) was significantly slower than that before 2006 (pre) in persistent never smokers in both Cohort 1 and 2. Conclusions: Smoking remains a strong risk factor for the development of AFL and lung function decline in Japan. This study suggests that changes in the smoking environment may have preventative effects on lung function decline, especially in never smokers.

DOI： 10.1016/j.resinv.2025.06.019

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Language：Japanese   Publisher：Lancet Respiratory Medicine  

Background: Balloon pulmonary angioplasty (BPA) and medical therapy are the most common treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH), but their combined use has not been sufficiently studied. This study aimed to evaluate the efficacy during an exercise test and the safety of the continuation of riociguat post-BPA in patients with inoperable CTEPH and normalised haemodynamic variables. Methods: This multicentre, double-blind, randomised, controlled, phase 4 trial was conducted at four high-volume CTEPH centres in Japan. Patients aged 18–85 years with a confirmed diagnosis of inoperable, WHO functional class II–IV CTEPH, and a resting mean pulmonary arterial pressure (mPAP) of 25 mm Hg or higher and pulmonary vascular resistance of 3·0 Wood units or higher on right-heart catheterisation, received riociguat and BPA at screening. Screened patients with a resting cardiac index of less than 5·0 L/min per m² and mPAP of less than 25 mm Hg 3 months after the final BPA procedure were randomly assigned (1:1) either to receive placebo three times daily for 16 weeks or to continue taking riociguat three times daily for 16 weeks at a dose established during the screening period, which varied per patient from 1·0 mg to 2·5 mg. Randomisation was done via an online system with a block size of 8 based on the allocation factors of medical institution and riociguat dose at allocation; patients and investigators were masked to treatment assignment. The primary endpoint was the change in peak cardiac index calculated using the direct Fick method during a cardiopulmonary exercise test (CPET) from baseline to week 16, and was measured in the full analysis set (ie, all randomly assigned patients apart from those who had withdrawn consent, had severe protocol violations, or had never taken the active drug or placebo) in all patients with available data. The safety analysis included all patients who had taken at least one dose of the active drug or placebo. The trial was registered with the Japan Registry of Clinical Trials (jRCTs041200052) and ClinicalTrials.gov (NCT04600492) and is now closed. Findings: Between Nov 25, 2020, and May 16, 2023, 74 patients (including 58 female patients and 16 male patients) were enrolled and randomly assigned, 36 to the riociguat-discontinuing group and 38 to the riociguat-continuing group. 32 patients in the riociguat-discontinuing group and 34 in the riociguat-continuing group were included in the primary analysis after exclusion of patients with missing data. The adjusted mean of peak cardiac index during CPET changed from baseline to week 16 by −1·11 L/min per m² (95% CI −2·14 to −0·09) in the riociguat-discontinuing group and by −0·03 L/min per m² (−1·04 to 0·99) in the riociguat-continuing group (intergroup difference 1·09 L/min per m² [95% CI 0·20–1·97]; p=0·017). Non-serious adverse events occurred in ten (28%) patients in the riociguat-discontinuing group and 14 (38%) patients in the riociguat-continuing group (p=0·36). No deaths were observed. Interpretation: Our findings suggested that the continuation of riociguat post-BPA might attenuate the worsening of exercise intolerance without increasing the risk of adverse events; discontinuation might require more careful consideration. These results should be explored in long-term studies. Funding: Bayer Yakuhin, Bayer, and Merck Sharp & Dohme.

DOI： 10.1016/S2213-2600(25)00127-4

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Language：Japanese   Publisher：Ijc Heart and Vasculature  

Background: Invasive treatments for chronic thromboembolic pulmonary hypertension (CTEPH) include pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA). Both PEA and BPA demonstrate significant benefits for symptom relief, hemodynamic improvement, and overall patient outcomes. However, evidence of their efficacy in older adults remains limited. This study aimed to evaluate treatment efficacy—including exercise tolerance, hemodynamics, functional status—and clinical outcomes in CTEPH patients aged ≥70 years who underwent PEA or BPA. Methods: Among 1,527 patients in the CTEPH AntiCoagulants Registry, we selected those aged 70 years or older, including 25 who underwent PEA during follow-up and 210 who received BPA. We evaluated the World Health Organization functional class (WHO FC), a measure of subjective symptoms, along with exercise capacity, hemodynamic parameters, and clinical outcomes. Results: At baseline, BPA patients were older (P = 0.003) and tended to have lower body weight (P = 0.069) than those undergoing PEA. At a median follow-up of ∼2 years, BPA significantly improved six-minute walk distance and cardiac index, and reduced mean pulmonary artery pressure and pulmonary vascular resistance, with effects comparable to PEA after adjusting for age, sex, and body mass index (all adjusted P > 0.10). However, improvement in WHO FC was more frequent with BPA and became significant after covariate adjustment (adjusted P = 0.035). Adverse event rates were infrequent and similar between the two groups (all P > 0.10). Conclusions: In adults aged ≥ 70 years with CTEPH, BPA improved hemodynamics, exercise capacity, and outcomes comparable to PEA, but with greater WHO FC improvement. Further randomized trials in older adults are warranted. Clinical trial registration number: UMIN Clinical Trials Registry (identifier UMIN 000033784).

DOI： 10.1016/j.ijcha.2025.101751

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Language：Japanese   Publisher：Cardiovascular Intervention and Therapeutics  

Advances in chronic thromboembolic pulmonary hypertension (CTEPH) treatment have improved prognosis, shifting focus towards symptom management. This study aimed to identify factors influencing the World Health Organization functional class (WHO-FC) in CTEPH patients. The CTEPH AC registry is a prospective, multicenter database from 35 Japanese institutions, analyzing data from August 2018 to July 2023. We examined factors associated with achieving WHO-FC I and WHO-FC changes over time in 1,270 patients. Significant factors for WHO-FC I achievement included male sex (odds ratio: 1.86, p = 0.019), age (0.98, p = 0.007), pulmonary vasodilator use (0.51, p = 0.001), post-balloon pulmonary angioplasty (BPA) (1.93, p = 0.010), lower mean pulmonary arterial pressure (0.94, p = 0.004), and lower pulmonary vascular resistance (PVR) (0.78, p = 0.006). Multivariate analysis showed that WHO-FC improvement correlated with male sex, baseline PVR, and BPA during follow-up. WHO-FC deterioration was associated with cancer, history of pulmonary endarterectomy and/or BPA at registration, bleeding risks, and thyroid disease or hormone therapy. BPA implementation is closely linked to symptomatic improvement and achieving WHO-FC I, while symptom worsening is often associated with patient-specific, difficult-to-control conditions.

DOI： 10.1007/s12928-025-01095-9

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Language：Japanese   Publisher：Journal of Cardiology Cases  

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease characterized by pulmonary hypertension (PH), resulting from stenosis or occlusion of the pulmonary arteries owing to an organic thrombus that obstructs blood flow in the pulmonary arteries. The prognosis for untreated patients is poor; however, it has improved significantly with the advent of treatments such as PH-targeted medical therapy and pulmonary balloon angioplasty, in addition to pulmonary endarterectomy. Nevertheless, the exact mechanisms underlying this disease remain unknown. Recently, a close association between coronavirus disease 2019 (COVID-19) and thrombosis has been detected, with the risk of venous thrombosis complications increasing after COVID-19 infection; however, few studies have reported on the association between COVID-19 and CTEPH. Herein, we present the case of a young man who developed CTEPH after a mild COVID-19 infection, despite the lack of an obvious thrombophilic predisposition. We conclude that if a patient develops chronic shortness of breath symptoms after a COVID-19 infection, it is important to investigate not only the COVID-19 sequelae, but also the presence of other diseases such as pulmonary artery thrombosis. Learning objective: Coronavirus disease 2019 (COVID-19) infection frequently causes abnormal blood coagulation and is closely related to thrombosis. Although pulmonary embolism is a frequent complication of venous thrombosis, few studies have reported an association between COVID-19 and chronic thromboembolic pulmonary hypertension (CTEPH). Our patient developed CTEPH after COVID-19 infection. It is important to examine organic abnormalities, before diagnosing persistent dyspnea as a COVID-19 sequela.

DOI： 10.1016/j.jccase.2025.01.001

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Language：English  

DOI： 10.2185/jrm.2024-027

PubMed

Language：Japanese   Publisher：Gastrointestinal Endoscopy  

Background and Aims Endoscopic papillectomy (EP) is a minimally invasive treatment for ampullary epithelial tumors. However, clinical outcomes and predictors of recurrence remain unclear. Methods We conducted a multicenter, retrospective cohort study at 22 tertiary centers in Japan, including patients who underwent EP between January 2009 and December 2020. The primary outcome was cumulative recurrence in patients with histologically confirmed adenoma or adenocarcinoma. Recurrence predictors were evaluated using Cox proportional hazards model. Results Of 875 patients, 798 had confirmed ampullary epithelial tumor, and 776 were included in the long-term outcome analysis. The 3-year cumulative recurrence rate was 20%. Indeterminate (Rx) and positive (R1) resection margins were independently associated with higher recurrence compared with negative margins (R0), with hazard ratios of 2.30 (95% CI, 1.44-3.68) and 2.95 (95% CI, 1.85-4.71), respectively. R0 resection was achieved in 52.1% (416/798) of patients, with a 3-year cumulative recurrence rate of 11% (95% CI, 7.6%-15%). Conclusions Recurrence after EP for ampullary epithelial tumors is common, even after R0 resection. These findings highlight the need for long-term surveillance following EP.

DOI： 10.1016/j.gie.2025.10.037

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Language：Japanese   Publisher：Diagnostics  

Background: Quality indicators (QIs) are used to standardize care and improve outcomes in patients with pulmonary arterial hypertension (PAH). It is important that QIs are validated within specific healthcare contexts. Therefore, this study aimed to validate QIs for PAH management in Japan using a modified Delphi consensus method. Methods: QI candidates were identified from published European QIs and clinical practice guidelines. An expert panel of 11 PAH specialists from diverse Japanese institutions anonymously rated the 36 initial QI candidates in two rounds using a nine-point appropriateness scale. Results: In the first round, 35 QIs received a median score of ≥7 points. A panel discussion was held between rounds to address the single low-scored QI, biomarker modifications, and invasive examinations, resulting in 36 modified QIs. In the second round, all modified QIs received median scores of ≥7 points and were judged to be valid as the final Japanese set of QIs. Conclusions: The findings of this study validated a set of QIs for PAH management tailored to the Japanese healthcare context. These QIs can be used to standardize care, identify areas for improvement, and ultimately enhance outcomes for Japanese patients with PAH.

DOI： 10.3390/diagnostics14232656

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Language：Japanese   Publisher：Journal of Heart and Lung Transplantation  

Background: The management of chronic thromboembolic pulmonary hypertension (CTEPH) has advanced significantly in recent years, thereby improving patient prognosis. However, the impact of cancer on the outcomes of patients with CTEPH under current treatment remains unclear. This study aimed to investigate the prevalence of cancer in patients with CTEPH and determine how comorbid cancer affects their prognosis and clinical course. Methods: Data from an ongoing Japanese prospective cohort study were analyzed. Prevalence and primary cancer sites were evaluated. The association of a history of cancer with a composite endpoint, including all-cause death, lung transplantation, and worsening of CTEPH, as well as venous thromboembolism and bleeding events, was assessed. Results: Of the 1,270 patients in the cohort, 134 (10.6%) had a history of cancer, with the most common primary sites being the breast in women and the prostate in men. The incidence of composite outcome and all-cause death was higher in those with a history of cancer (p < 0.001, log-rank test). In the Cox proportional hazard model, age- and sex-adjusted hazard ratios for the composite outcome and all-cause death were 2.69 (95% confidence interval, 1.48–4.89, p = 0.001) and 4.25 (95% confidence interval, 1.98–9.10, p < 0.001), respectively, for patients with a history of cancer. No significant differences in venous thromboembolism and bleeding events were observed between patients with and those without a history of cancer. Conclusions: A history of cancer, with a prevalence of 10.6%, is an independent risk factor for mortality in patients with CTEPH undergoing the currently recommended treatment.

DOI： 10.1016/j.healun.2024.10.022

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Language：Japanese   Publisher：(一社)日本心臓病学会  

Language：Japanese   Publisher：Pediatric Neurology  

Background: Six percent of patients with Leigh syndrome (LS) present with infantile epileptic spasms syndrome (IESS). However, treatment strategies for IESS with LS remain unclear. This retrospective study aimed to evaluate the efficacy and safety of treatment strategies in patients with IESS complicated by LS and Leigh-like syndrome (LLS). Methods: We distributed questionnaires to 750 facilities in Japan, and the clinical data of 21 patients from 15 hospitals were collected. The data comprised treatment strategies, including adrenocorticotropic hormone (ACTH) therapy, ketogenic diet (KD) therapy, and antiseizure medications (ASMs); effectiveness of each treatment; and the adverse events. Results: The median age at LS and LLS diagnosis was 7 months (range: 0 to 50), whereas that at the onset of epileptic spasms was 7 (range: 3 to 20). LS was diagnosed in 17 patients and LLS in four patients. Seven, two, five, and seven patients received ACTH + ASMs, ACTH + KD + ASMs, KD + ASMs, and ASMs only, respectively. Four (44%) of nine patients treated with ACTH and one (14%) of seven patients treated with KD achieved electroclinical remission within one month of treatment. No patients treated with only ASMs achieved electroclinical remission. Seven patients (33%) achieved electroclinical remission by the last follow-up. Adverse events were reported in four patients treated with ACTH, none treated with KD therapy, and eight treated with ASMs. Conclusion: ACTH therapy shows the best efficacy and rapid action in patients with IESS complicated by LS and LLS. The effectiveness of KD therapy and ASMs in this study was insufficient.

DOI： 10.1016/j.pediatrneurol.2024.05.007

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Language：Japanese   Publisher：Jacc Asia  

Background: The contemporary outcome of balloon pulmonary angioplasty (BPA) and pulmonary endarterectomy (PEA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) are unclear. Objectives: This study aimed to clarify the characteristics and outcomes of CTEPH patients treated with BPA and PEA in Japan. Methods: Among 1,270 participants enrolled between 2018 and 2023 in the CTEPH AC (Chronic Thromboembolic Pulmonary Hypertension Anticoagulant) registry, a Japanese nationwide CTEPH registry, 369 treatment-naive patients (BPA strategy: n = 313; PEA strategy: n = 56) and 690 on-treatment patients (BPA strategy: n = 561; PEA strategy: n = 129) were classified according to the presence of prior reperfusion therapy. Morbidity and mortality events (all-cause death, rescue mechanical reperfusion therapy, and/or initiation of parenteral pulmonary vasodilators), pulmonary hemodynamics, exercise tolerance, and relevant laboratory test results were evaluated. Results: The BPA strategy was chosen in older patients than the PEA strategy (mean age, BPA vs PEA: 66.5 ± 12.6 years vs 62.5 ± 11.8 years; P = 0.028). Median follow-up period was 615 (Q1-Q3: 311-997) days in treatment-naive patients and 1,136 (Q1-Q3: 684-1,300) days in on-treatment patients. BPA strategy had as acceptable morbidity and mortality as PEA strategy (5-year morbidity and mortality event rate, BPA vs PEA: 10.2% [95% CI: 5.2%-19.5%] vs 16.1% [95% CI: 4.3%-50.6%] in treatment-naive patients; 9.7% [95% CI: 6.7%-13.8%] vs 6.9% [95% CI: 2.7%-17.3%] in on-treatment patients), with greater improvement of renal function; glomerular filtration rate in propensity score-matched population (difference between change: 4.9 [95% CI: 0.5-9.3] mL/min/1.73 m²; P = 0.030). Conclusions: BPA strategy was more frequently chosen in older patients compared with PEA strategy and showed acceptable outcomes for efficacy with greater advantage for improvement in renal function. (Multicenter registry of chronic thromboembolic pulmonary hypertension in Japan; UMIN000033784)

DOI： 10.1016/j.jacasi.2024.05.007

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Language：Japanese   Publisher：Pancreatology  

Background/objectives: The association between autoimmune pancreatitis (AIP) and pancreatic cancer (PC) remains controversial. This study aimed to clarify the long-term prognosis and risk of malignancies in AIP patients in Japan. Methods: We conducted a multicenter retrospective cohort study on 1364 patients with type 1 AIP from 20 institutions in Japan. We calculated the standardized incidence ratio (SIR) for malignancies compared to that in the general population. We analyzed factors associated with overall survival, pancreatic exocrine insufficiency, diabetes mellitus, and osteoporosis. Results: The SIR for all malignancies was increased (1.21 [95 % confidence interval: 1.05–1.41]) in patients with AIP. Among all malignancies, the SIR was highest for PC (3.22 [1.99–5.13]) and increased within 2 years and after 5 years of AIP diagnosis. Steroid use for ≥6 months and ≥50 months increased the risk of subsequent development of diabetes mellitus and osteoporosis, respectively. Age ≥65 years at AIP diagnosis (hazard ratio [HR] = 3.73) and the development of malignancies (HR = 2.63), including PC (HR = 7.81), were associated with a poor prognosis, whereas maintenance steroid therapy was associated with a better prognosis (HR = 0.35) in the multivariate analysis. Maintenance steroid therapy was associated with a better prognosis even after propensity score matching for age and sex. Conclusions: Patients with AIP are at increased risk of developing malignancy, especially PC. PC is a critical prognostic factor for patients with AIP. Although maintenance steroid therapy negatively impacts diabetes mellitus and osteoporosis, it is associated with decreased cancer risk and improved overall survival.

DOI： 10.1016/j.pan.2024.01.008

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Language：Japanese   Publisher：Water Research  

In this study, a long-term operation of 2,747 days was conducted to evaluate the performance of the upflow anaerobic sludge blanket (UASB) reactor and investigated the degradation mechanisms of high-organic loading phenol wastewater. During the reactor operation, the maximum chemical oxygen demand (COD) removal rate of 6.1 ± 0.6 kg/m³/day under 1,680 mg/L phenol concentration was achieved in the mesophilic UASB reactor. After a significant change in the operating temperature from 24.0 ± 4.1 °C to 35.9 ± 0.6 °C, frequent observations of floating and washout of the bloated granular sludge (novel types of the bulking phenomenon) were made in the UASB reactor, suggesting that the change in operating temperature could be a trigger for the bulking phenomenon. Through the metagenomic analysis, phenol degradation mechanisms were predicted that phenol was converted to 4-hydroxybenzoate via two possible routes by Syntrophorhabdaceae and Pelotomaculaceae bacteria. Furthermore, the degradation of 4-hydroxybenzoate to benzoyl-CoA was carried out by members of Syntrophorhabdaceae and Smithellaceae. In the bulking sludge, a predominant presence of Nanobdellota, belonging to DPANN archaea, was detected. The metagenome-assembled genome of the Nanobdellota lacks many biosynthetic pathways and has several genes for the symbiotic lifestyle such as trimeric autotransporter adhesin-related protein. Furthermore, the Nanobdellota have significant correlations with several methanogenic archaea that are predominantly present in the UASB reactor. Considering the results of this study, the predominant Nanobdellota may negatively affect the growth of the methanogens through the parasitic lifestyle and change the balance of microbial interactions in the granular sludge ecosystem.

DOI： 10.1016/j.watres.2024.121271

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Language：English   Publisher：(一社)日本循環器学会  

Language：Japanese   Publisher：Respiratory Medicine Case Reports  

A 65-year-old man with dyspnea and hemoptysis presented with a right upper lobe mass associated with enlarged mediastinal lymph nodes and bilateral pulmonary nodules on chest computed tomography (CT), suspected lung cancer. Bronchial and CT-guided biopsies revealed poorly differentiated carcinoma. His condition deteriorated rapidly before a definitive diagnosis could be made. Autopsy revealed primary mediastinal choriocarcinoma. Primary mediastinal choriocarcinomas are rare, difficult to diagnose early and have a poor prognosis. In patients with a tumor expanding across the lung and mediastinum and exhibiting pathologic findings of a pooly differentiated carcinoma, we should consider choriocarcinoma, evaluating the serum β-human chorionic gonadotropin levels.

DOI： 10.1016/j.rmcr.2024.102037

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Language：Japanese   Publisher：Immunological Medicine  

We started a registry for cases of immunoglobulin (Ig)G4-related disease (IgG4-RD) in December 2019 to clarify the clinical profile of IgG4-RD. In this study, clinical information from 854 cases registered by February 16, 2024 was analyzed from multiple perspectives. Diagnosis of IgG4-RD was made in 808 cases, comprising 638 definite, 38 probable, and 132 possible. The mean ± SD age at time of enrollment of the 808 cases was 67.9 ± 11.3 years, with 68.8% being male. The pancreas was the most frequently affected organ (49.8%), followed by the submandibular glands (46.2%) and lacrimal glands (30.6%). This study reconfirmed the pancreas and head-and-neck region as major affected areas in IgG4-RD. Clinically, submandibular adenitis and autoimmune pancreatitis often occur together in the same patient, but no association between the two organs was observed in our analysis. Regarding diagnosis, the comprehensive diagnostic criteria were most commonly used (63.6%). Storiform fibrosis and phlebitis obliterans were detected at different frequencies in different organs. In summary, this registry study identified clinical, imaging, hematologic, and pathologic findings in 808 Japanese patients with IgG4-RD. The frequency of affected organs and their characteristic pathological findings will be particularly useful for future practice.

DOI： 10.1080/25785826.2024.2430812

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Language：Japanese   Publisher：European Respiratory Journal  

Background Peripheral pulmonary artery stenosis (PPS) refers to stenosis of the pulmonary artery from the trunk to the peripheral arteries. Although paediatric PPS is well described, the clinical characteristics of adult-onset idiopathic PPS have not been established. Our objectives in this study were to characterise the disease profile of adult-onset PPS. Methods We collected data in Japanese centres. This cohort included patients who underwent pulmonary angiography (PAG) and excluded patients with chronic thromboembolic pulmonary hypertension or Takayasu arteritis. Patient backgrounds, right heart catheterisation (RHC) findings, imaging findings and treatment profiles were collected. Results 44 patients (median (interquartile range) age 39 (29–57) years; 29 females (65.9%)) with PPS were enrolled from 20 centres. In PAG, stenosis of segmental and peripheral pulmonary arteries was observed in 41 (93.2%) and 36 patients (81.8%), respectively. 35 patients (79.5%) received medications approved for pulmonary arterial hypertension (PAH) and 22 patients (50.0%) received combination therapy. 25 patients (56.8%) underwent transcatheter pulmonary angioplasty. RHC data showed improvements in both mean pulmonary arterial pressure (44 versus 40 mmHg; p<0.001) and pulmonary vascular resistance (760 versus 514 dyn·s·cm⁻⁵; p<0.001) from baseline to final follow-up. The 3-, 5- and 10-year survival rates of patients with PPS were 97.5% (95% CI 83.5–99.6%), 89.0% (95% CI 68.9–96.4%) and 67.0% (95% CI 41.4–83.3%), respectively. Conclusions In this study, patients with adult-onset idiopathic PPS presented with segmental and peripheral pulmonary artery stenosis. Although patients had severe pulmonary hypertension at baseline, they showed a favourable treatment response to PAH drugs combined with transcatheter pulmonary angioplasty.

DOI： 10.1183/13993003.00763-2023

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Language：Japanese   Publisher：(一社)日本心臓病学会  

Language：Japanese   Publisher：Journal of Cardiology Cases  

Pulmonary artery hypertension associated with human immunodeficiency virus infection (PAH-HIV) is known to be caused by HIV infection. Antiretroviral therapy and PAH-specific drugs improve the prognosis of patients with PAH-HIV, but the pathophysiology of PAH-HIV remains unclear. We report a case of PAH-HIV treated with upfront combination therapy including subcutaneous injection of treprostinil. One year after treatment initiation, the patient's PAH improved significantly. However, it worsened over time due to reduced efficacy of subcutaneous injection of treprostinil. Learning objective: The etiology and pathophysiology of pulmonary artery hypertension associated with human immunodeficiency virus infection (PAH-HIV) remain unclear, and there are few case reports of PAH-HIV in Japan because the HIV prevalence is low. We encountered a case of PAH-HIV in which the efficacy of subcutaneous treprostinil injection was attenuated. It was unclear whether the reduced efficacy was associated with the pathophysiology of PAH-HIV. When PAH control deteriorates, early alteration of medication choice and administration route is important.

DOI： 10.1016/j.jccase.2023.04.008

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Language：Japanese   Publisher：BMJ Open  

Objectives Balloon pulmonary angioplasty (BPA) and medical therapy, such as soluble guanylate cyclase stimulators, are recommended treatments for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy (PEA). However, monotherapy with BPA or medical therapy cannot always eliminate symptoms such as exertional dyspnoea. Thus, this study aims to clarify the efficacy of continuous treatment with riociguat in inoperable CTEPH patients with normalised haemodynamics after BPA. Methods and analysis This is a double-blind, multicentre, randomised, placebo-controlled trial. Participants with CTEPH who are ineligible for PEA will receive riociguat followed by BPA. Subsequently, participants will be randomised (1:1) into either riociguat continuing or discontinuing groups and will be observed for 16 weeks after randomisation. The primary endpoint will be the change in peak cardiac index (CI) during the cardiopulmonary exercise test. In the primary analysis, the least square mean differences and 95% CIs for the change in peak CI at 16 weeks between the groups will be estimated by a linear mixed-effects model with baseline value as a covariate, treatment group as a fixed effect and study institution as a random effect. Ethics and dissemination National Hospital Organisation Review Board for Clinical Trials (Nagoya) and each participating institution approved this study and its protocols. Written informed consent will be obtained from all participants. The results will be disseminated at medical conferences and in journal publications. Registration details Japan Registry of Clinical Trials: jRCT no. 041200052. ClinicalTrials.gov by National Library of Medicine Registry ID: NCT04600492. Trial registration number NCT04600492.

DOI： 10.1136/bmjopen-2023-072241

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Language：Japanese   Publisher：Journal of Cardiology  

Background: Currently, pulmonary hypertension-targeted therapy has been shown to improve the survival of patients with pulmonary artery hypertension (PAH). However, the importance of early diagnosis has not been investigated. Therefore, this study aimed to investigate whether a delayed diagnosis of PAH is associated with its prognosis. Methods and results: A total of 66 consecutive untreated patients were diagnosed with PAH from January 2008 to December 2021 at the Kagoshima University Hospital. The time from symptom onset to diagnosis correlated with brain natriuretic peptide levels (p < 0.001), right ventricle (RV) Tei index (p < 0.001), and the tricuspid annular plane systolic excursion/systolic pulmonary artery pressure ratio (p = 0.003). These findings suggest that in patients with PAH, RV function declines with increasing time from symptom onset to diagnosis. Furthermore, older patients with PAH appeared to have a longer time from symptom onset to diagnosis. Next, patients were divided into delayed diagnosis (>3 months) and early diagnosis (≤3 months) groups based on the time from symptom onset to diagnosis. Patients were categorized into three groups according to the European Society of Cardiology (or the European Respiratory Society) risk stratification guidelines. Patients diagnosed with PAH within 3 months of symptom onset were significantly in the low- or intermediate-risk groups (p < 0.001). A Kaplan-Meier analysis revealed that the cumulative event-free rate was significantly lower (p < 0.01) in the delayed diagnosis group than in the early diagnosis group. A delayed diagnosis was significantly associated with a worse outcome than an early diagnosis, after adjusting for different sets of confounding factors. Conclusions: A delayed PAH diagnosis is associated with a poor prognosis. Early diagnosis of PAH may lead to a low-risk treatment. Furthermore, older patients need more careful screening for PAH.

DOI： 10.1016/j.jjcc.2023.08.004

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Language：Japanese   Publisher：日本肺高血圧・肺循環学会・日本小児肺循環研究会  

Language：Japanese   Publisher：Engineering  

A microbial community structure of granules harvested from an anaerobic sludge blanket reactor treating phenolic wastewater was investigated using fluorescence in situ hybridization (FISH) and clone library construction. Clones of Syntrophorhabdaceae and Cryptanaerobacter were observed to be responsible for phenol degradation. For accurate taxonomic assignment of Cryptanaerobacter clones, phylogenetic analysis using nearly full-length 16S ribosomal RNA (rRNA) gene sequences was necessary. Three oligonucleotide probes were designed to detect the following three taxonomic groups: Syntrophorhabdaceae, Cryptanaerobacter, and Syntrophus. FISH analysis of thin sections of anaerobic granules showed a random distribution of bacteria and archaea. However, a well-defined distribution of Syntrophorhabdaceae, Cryptanaerobacter, and Syntrophus was observed. Cryptanaerobacter and Syntrophus were found on the outer layer of the granules and were closely associated with each other, while Syntrophorhabdaceae was located in the deeper part of the granules. Such specific distribution of the bacteria is most likely due to their metabolic association and affinity for the substrate. Phenol degradation in the granular sludge was observed to be carried out in the following way. First, Cryptanaerobacter converts phenol to benzoate, which is then degraded by Syntrophus into acetate. This syntrophic degradation of phenol occurs near the surface of the granule, where the phenol concentration is high. In the deeper part of the granule, where the phenol concentration is lower, Syntrophorhabdaceae degrades phenol into acetate. We observed that Syntrophorhabdaceae is less likely to produce benzoate as an intermediate to feed the neighboring organisms, which contradicts the theories presented by previous studies.

DOI： 10.1016/j.eng.2021.05.012

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Language：Japanese   Publisher：(株)文光堂  

Language：Japanese   Publisher：Journal of Cardiology  

Background: Mitochondria are dynamic organelles that undergo fission or fusion. These mitochondrial dynamics are reported to be associated with pulmonary hypertension (PH). PH is divided into 5 groups, including pulmonary artery hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), based on its pathogenesis. However, it is still unknown whether and how miRNAs related to mitochondrial dynamics (MD) affect PAH and CTEPH. Methods: We investigated patients who underwent right heart catheterization between October 2016 and January 2019. Out of 34 PH patients, 12 were diagnosed with PAH, and 22 were diagnosed with CTEPH. In addition, there were 30 patients diagnosed with left heart disease. We enrolled the 34 PH patients as the PH group and 30 left heart disease patients as the control group. Results: Among MD-related miRNAs, the circulating levels of miR-140-3p were higher, and those of miR-485-5p were lower in the PH group than in the control group (p < 0.01), suggesting that miRNAs inducing mitochondrial fission are related to PH. The miR-140-3p levels in the PAH and CTEPH groups were higher than those in the control group (p < 0.01). The levels of miR-140-3p and miR-485-5p in the PAH group correlated with pulmonary vascular resistance (r = 0.582, p = 0.046) and cardiac index (r = −0.36, p = 0.04), respectively. The miR-485-5p levels in the CTEPH group correlated with right atrium pressure (r = −0.456, p = 0.049). Conclusion: MD-related miRNAs levels change to induce fission and are closely related to the hemodynamics of PAH and CTEPH.

DOI： 10.1016/j.jjcc.2021.03.008

Scopus

PubMed

Language：Japanese   Publisher：Progress of Theoretical and Experimental Physics  

The Laser Interferometer Gravitational-wave Observatory Scientific Collaboration and Virgo Collaboration (LVC) sent out 56 gravitational-wave (GW) notices during the third observing run (O3). The Japanese Collaboration for Gravitational wave ElectroMagnetic follow-up (J-GEM) performed optical and near-infrared observations to identify and observe an electromagnetic (EM) counterpart. We constructed a web-based system that enabled us to obtain and share information on candidate host galaxies for the counterpart, and the status of our observations. Candidate host galaxies were selected from the GLADE catalog with a weight based on the 3D GW localization map provided by LVC. We conducted galaxy-targeted and wide-field blind surveys, real-time data analysis, and visual inspection of observed galaxies. We performed galaxy-targeted follow-ups to 23 GW events during O3, and the maximum probability covered by our observations reached 9.8%. Among these, we successfully started observations for 10 GW events within 0.5 days after the detection. This result demonstrates that our follow-up observation has the potential to constrain EM radiation models for a merger of binary neutron stars at a distance of up to ∼100 Mpc with a probability area of ≤ 500 deg 2.

DOI： 10.1093/ptep/ptab007

Scopus

Language：Japanese   Publisher：日本肺高血圧・肺循環学会・日本小児肺循環研究会  

DOI： 10.1253/circrep.CR-20-0133

PubMed

Language：Japanese   Publisher：(一社)日本老年医学会  

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1253/circrep.CR-20-0046

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1253/circrep.CR-19-0087

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1007/s00380-019-01373-6

Language：Japanese   Publisher：(一社)日本心臓病学会  

Language：English   Publishing type：Research paper (scientific journal)  

Language：Japanese   Publisher：(公社)日本医学放射線学会  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：International Heart Journal  

DOI： 10.1536/ihj.18-149

Scopus

PubMed

Language：Japanese   Publisher：(一社)日本心臓病学会  

Language：Japanese   Publisher：(一社)日本血液学会-東京事務局  

54歳女。易疲労感を主訴とした。甲状腺がんの術後経過観察中に肝脾腫を認め、近医婦人科で子宮筋腫と貧血を指摘された。紹介受診時の各種検査で多発ニューロパチー、血清VEGF上昇、IgA-λ型M蛋白、肝脾腫、下肢浮腫、内分泌異常、皮膚の色素沈着を認めたことからpolyneuropathy、organomegaly、endocrinopathy、M-protein and skin changes症候群と診断した。当初、大量化学療法併用自家造血幹細胞移植を計画していたが、治療前に肺高血圧症が判明した。lenalidomide・dexamethazone療法を先行し、3コース施行後の右心カテーテル検査で肺高血圧症の改善を確認してから大量化学療法併用自家造血幹細胞移植を行った。

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1536/ihj.16-629

Language：Japanese   Publisher：(一社)日本心臓病学会  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Article, review, commentary, editorial, etc. (scientific journal)  

DOI： 10.1016/j.radcr.2020.05.036

Scopus

PubMed

Publisher：The Japanese Society of Hematology  

<p>POEMS syndrome is often complicated by pulmonary hypertension. The standard therapy for patients with POEMS syndrome is high-dose chemotherapy followed by autologous stem cell transplantation. However, the safety of high-dose chemotherapy for patients complicated with pulmonary hypertension remains unclear, and the optimal therapy for these patients is yet to be establishment. Herein, we report the case of a 54-year-old woman with POEMS syndrome accompanied by pulmonary hypertension. We successfully and safely performed lenalidomide and dexamethasone (Ld) therapy followed by high-dose chemotherapy and autologous stem cell transplantation, which improved her pulmonary hypertension. Thus, Ld can be considered as safe and effective for pulmonary hypertension with POEMS syndrome.</p>

DOI： 10.11406/rinketsu.59.489

PubMed

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肺高血圧診療における地域連携の重要性.

肺高血圧症のトータルケア.

当院でのPH治療における地域医療連携活動と薬物治療.

当院におけるセレキシパグ使用症例

肺高血圧症の診断と治療～病診連携の実際～.

The patient mental health care in PAH treatment.

肺疾患合併PAHからみるリオシグアトの効果.

肺高血圧症のトータルケア.

トレプロスチニルの使用経験.

肺高血圧症の最近の治療と鹿児島での取り組み.

Audience： Guardians