Refractory phenotype of -sensitized asthma with bronchiectasis and allergic bronchopulmonary aspergillosis.

Background: Sensitization to mucus plugs, and bacterial colonization may coexist and relate to a refractory phenotype during follow-up in asthma with bronchiectasis and allergic bronchopulmonary aspergillosis (ABPA).

Objective: This study aimed to clarify the features of -sensitized refractory asthma with bronchiectasis and determine the refractory phenotype in this population and ABPA.

Methods: This study included cases of the oldest available -specific IgE data and chest computed tomography images from a nationwide survey of refractory asthma with bronchiectasis.

IgG4-related pleural disease diagnosed by thoracoscopic pleural biopsy: A case report.

Here we report a rare case of immunoglobulin G4 (IgG4)-related pleural disease diagnosed using a thoracoscopic pleural biopsy. A 66-year-old man was admitted to our hospital with right-dominant bilateral pleural effusions and gradually worsening dyspnoea. Chest radiographs revealed right-dominant pleural effusions, while chest computed tomography showed bilateral pleural effusions without parenchymal lesions.

Serum VEGF-A levels on admission in COVID-19 patients correlate with SP-D and neutrophils, reflecting disease severity: A prospective study.

Background And Objective: The coronavirus disease 2019 (COVID-19) pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has resulted in significant global morbidity and mortality. This study aimed to investigate the clinical significance of serum vascular endothelial growth factor A (VEGF-A) in COVID-19 patients and its association with disease severity and pulmonary injury.

Methods: We prospectively collected data from 71 hospitalized COVID-19 patients between June 2020 and January 2021.

Practical guide for the diagnosis and management of primary ciliary dyskinesia.

Objective: Primary ciliary dyskinesia (PCD) is a relatively rare genetic disorder that affects approximately 1 in 20,000 people. Approximately 50 genes are currently known to cause PCD. In light of differences in causative genes and the medical system in Japan compared with other countries, a practical guide was needed for the diagnosis and management of Japanese PCD patients.

LAG3 is an independent prognostic biomarker and potential target for immune checkpoint inhibitors in malignant pleural mesothelioma: a retrospective study.

Background: Lymphocyte-activation gene 3 (LAG3) is an immune checkpoint receptor; novel LAG3 immune checkpoint inhibitors (ICIs) exhibit therapeutic activity in melanoma. The role of LAG3and ICIs of LAG3 are unknown in malignant pleural mesothelioma (MPM). This study aimed to uncover the prognostic landscape of LAG3 in multiple cancers and investigate the potential of using LAG3 as an ICIs target in patients with MPM.

Nationwide survey of refractory asthma with bronchiectasis by inflammatory subtypes.

Rationale: Bronchiectasis and bronchiolitis are differential diagnoses of asthma; moreover, they are factors associated with worse asthma control.

Objective: We determined clinical courses of bronchiectasis/bronchiolitis-complicated asthma by inflammatory subtypes as well as factors affecting them.

Methods: We conducted a survey of refractory asthma with non-cystic fibrosis bronchiectasis/bronchiolitis in Japan.

A case of lung intravascular large B cell lymphoma developed with respiratory failure rescued by corticosteroid prior to definite diagnosis.

A 56-year-old man complained progressive dyspnea, fatigue and fever for one month. His chest CT exhibited faint ground-glass opacities, and the levels of serum LDH and soluble interleukin 2 receptor were markedly elevated. Positron emission tomography (PET) showed high uptake of 18-fluoro deoxy glucose (FDG) only on both lungs.

Analysis of the diagnosis of Japanese patients with primary ciliary dyskinesia using a conditional reprogramming culture.

Background: Primary ciliary dyskinesia (PCD) is diagnosed through multiple methods, including transmission electron microscopy (TEM), a high-speed video microscopy analysis (HSVA), immunofluorescence (IF), and genetic testing. A primary cell culture has been recommended to avoid the misdiagnosis of secondary ciliary dyskinesia derived from infection or inflammation and improve diagnostic accuracy. However, primary cells fail to differentiate into ciliated cells through repeated passages.

Tryptase and IL-33 in Bronchoalveolar Lavage Fluid May Predict the Types of Eosinophilic Pneumonia and Disease Recurrence.

Introduction: Eosinophilic pneumonia (EP) is characterized by a marked accumulation of eosinophils in the lungs and blood. Eosinophils and mast cells play an important role in the pathogenesis of EP via release of biomarkers such as tryptase and interleukin (IL)-33. However, the potential role of these biomarkers is not fully understood.

Analysis of the clinical features of Japanese patients with primary ciliary dyskinesia.

Objective: Primary ciliary dyskinesia (PCD) is a rare hereditary disease. Most reports of PCD in Japan are case reports, and clinical analysis has not been performed. Differences in the causative genes might affect the clinical features in different ethnic groups.

Clinical characteristics and treatment outcomes of severe asthma patients with a history of multiple biologic drugs use.

Background: Asthma control has been shown to improve after clinical use of molecular-targeted biologic drugs. Although most patients have shown favorable responses to biologic drugs, some individuals need to switch to another biologic drug. To date, limited data are available regarding patients who received multiple biologic drugs.

Less airway inflammation and goblet cell metaplasia in an IL-33-induced asthma model of leptin-deficient obese mice.

Background: Obesity-associated asthma is a phenotype of severe asthma. Late-onset, non-eosinophilic and female-dominant phenotype is highly symptomatic and difficult to treat. Leptin, an adipokine, exerts an immunomodulatory effect.

Characteristics of patients with severe asthma who experienced treatment failure with omalizumab.

Background: Omalizumab, an anti-IgE antibody, has been widely used in many countries, including Japan. However, some patients do not respond to omalizumab, and the cause of treatment failure has not been fully elucidated.

Objective: This study aimed to evaluate the characteristics of adult asthma patients who failed to achieve disease control with omalizumab in a real-world setting.

Multifaceted analysis of Japanese cases of primary ciliary dyskinesia: Value of immunofluorescence for ciliary protein detection in patients with DNAH5 and DNAH11 mutations.

Multifaceted analysis is recommended for the diagnosis of primary ciliary dyskinesia (PCD). A 31-year-old woman had situs inversus, bronchiectasis, family history of PCD, and compound heterozygous mutations in DNAH5. Her cilia were immotile.

Polymerase-chain reaction testing to prevent hospital-acquired severe acute respiratory syndrome coronavirus 2 infection in Shinjuku, an epicenter in Tokyo: The Tokyo Women's Medical University model.

Hospital-acquired severe acute respiratory virus coronavirus 2 (SARS-CoV-2) infection is a healthcare challenge. We hypothesized that polymerase chain reaction testing of symptomatic triaged outpatients and all inpatients before hospitalization in Shinjuku, a coronavirus disease 2019 (COVID-19) epicenter in Tokyo, using the Tokyo Women's Medical University (TMWU) model would be feasible and efficient at preventing COVID-19. This retrospective study enrolled 2981 patients from March to May 2020.

A case of pulmonary infarction induced by undiagnosed HIV.

A 25-year-old Chinese man visited our institution due to fever and left chest pain. A chest CT showed infiltrative shadows with pleural effusion. Despite antibiotics treatment, his symptoms gradually worsened.

A case of lymphangioleiomyomatosis with diffuse large B-cell lymphoma: Usefulness of FDG-PET.

Lymphangioleiomyomatosis (LAM) is characterized by cystic lung disease, abdominal tumor and involvement of the axial lymph nodes. We report a very rare case of LAM with malignant lymphoma. A 51-year-old female had medical history of recurrent pneumothorax and nephrectomy for a left renal angiomyolipoma and was diagnosed with LAM by video-assisted thoracoscopic surgery at the age of 30.

Coexistence of diffuse panbronchiolitis and sarcoidosis revealed during splenectomy: a case report.

Background: Sarcoidosis is a systemic granulomatous disease caused by CD4+ cell-dominant inflammation. Meanwhile, diffuse panbronchiolitis is a chronic inflammatory respiratory disease predominantly caused by CD8+ lymphocytes and neutrophils. Herein, we report a rare case of sarcoidosis in which the clinical presentation had become evident as diffuse panbronchiolitis after splenectomy for sarcoidosis.

Copy number variation in DRC1 is the major cause of primary ciliary dyskinesia in the Japanese population.

Background: Primary ciliary dyskinesia (PCD) is a rare genetic disorder caused by functional impairment of cilia throughout the body. The involvement of copy number variation (CNV) in the development of PCD is largely unknown.

Methods: We examined 93 Japanese patients with clinically suspected PCD from 84 unrelated families.

PD-L1, FGFR1, PIK3CA, PTEN, and p16 expression in pulmonary emphysema and chronic obstructive pulmonary disease with resected lung squamous cell carcinoma.

Background: Emphysema and chronic obstructive pulmonary disease (COPD) are well known independent risk factors for lung cancer. However, the developmental mechanisms between emphysema/COPD and lung cancer remain unknown. The purpose of this study was to evaluate PD-L1, FGFR1, PIK3CA, PTEN, and p16 expression in squamous cell carcinoma (SCC) associated with emphysema/COPD.

Comparison of tumor cell numbers and 22C3 PD-L1 expression between cryobiopsy and transbronchial biopsy with endobronchial ultrasonography-guide sheath for lung cancer.

Background: We previously reported cryobiopsy (Cryo) with endobronchial ultrasonography-guide sheath (EBUS-GS) for peripheral pulmonary lesions (PPLs) provides significantly larger tissues than transbronchial biopsy (TBB) and provides high quantity and quality DNA for gene analysis by next generation sequencing. However, the tumor cell yields and programmed death ligand 1 (PD-L1) expression between each approach have not been compared. Here, we assessed the tumor cell numbers and PD-L1 expression for Cryo with EBUS-GS for PPLs and TBB in patients with lung cancer.

A Japanese Case of Primary Ciliary Dyskinesia with DNAH5 Mutations.

A 33-year-old woman presented with a productive cough from childhood. She had suffered from repeated bacterial pneumonia. Her clinical and imaging findings revealed chronic sinusitis, bronchiectasis and situs inversus.