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.

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.

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.

The Japanese respiratory society guidelines for the management of cough and sputum (digest edition).

Cough and sputum are common complaints at outpatient visits. In this digest version, we provide a general overview of these two symptoms and discuss the management of acute (up to three weeks) and prolonged/chronic cough (longer than three weeks). Flowcharts are provided, along with a step-by-step explanation of their diagnosis and management.

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.

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.

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.

Cryobiopsy with endobronchial ultrasonography using a guide sheath for peripheral pulmonary lesions and DNA analysis by next generation sequencing and rapid on-site evaluation.

Background: The purpose of this study was to evaluate the diagnostic accuracy of Cryo with endobronchial ultrasonography using a guide sheath (EBUS-GS) for peripheral pulmonary lesions (PPLs) to assess the volume of specimen, determine DNA sequencing analysis, and evaluate the utility of rapid on-site evaluation (ROSE).

Methods: Out of 30 patients assessed for eligibility, 23 were enrolled in this prospective study. The histological diagnostic yield of Cryo was evaluated and the volume was compared to that of trans-bronchial biopsy (TBB).

Clarithromycin suppresses IL-13-induced goblet cell metaplasia via the TMEM16A-dependent pathway in guinea pig airway epithelial cells.

Background: Transmembrane protein 16A (TMEM16A) is associated with mucus secretion and ion transport in asthma. Clarithromycin (CAM) is reported to inhibit IL-13-induced goblet cell metaplasia. However, the effect of CAM on TMEM16A function and expression remains unclear.

Coexistence of diffuse panbronchiolitis and asthma: reciprocity of neutrophilic and eosinophilic inflammation.

Diffuse panbronchiolitis (DPB) and asthma are obstructive airway diseases, the former being characterized by Th1-type and the latter by Th2-type airway inflammation. Differential diagnosis is often a problem, but coexistence has rarely been reported. A 76-year-old man with asthma was admitted to our hospital because of one-month history of dyspnoea on exertion with bilateral diffuse granular shadows.

Effect of tiotropium on mucus hypersecretion and airway clearance in patients with COPD.

Background: Increased sputum production is an important feature of COPD, in which a large amount of secretions stagnated in the respiratory lumen may aggravate airflow limitation, impair airway mucociliary transport, and cause recurrent respiratory infection and, hence, acute exacerbations of the diseases. There is evidence that airway mucus hypersecretion is associated with the severity and prognosis of COPD, but the symptoms are generally difficult to treat.

Methods: In an open, non-controlled study, we examined the effect of the anticholinergic agent tiotropium on airway mucus hypersecretion in 22 COPD patients.

Primary ciliary dyskinesia with complex abnormalities including cleavage of B-subfibers.

A 25-year-old Japanese woman suffered from repeated respiratory tract infections. Because of her characteristic medical history and imaging findings, we suspected primary ciliary dyskinesia (PCD) and performed a transbronchial biopsy. The biopsy revealed complex abnormalities of the ciliary structure including cleavage of the B-subfibers observed by transmission electron microscopy analysis and the complete loss of ciliary motion by video analysis.

Pulmonary Paragonimiasis: The Detection of a Worm Migration Track as a Diagnostic Clue for Uncertain Eosinophilic Pleural Effusion.

A 38-year-old woman with sustained right chest pain was referred to our hospital. She showed pleural effusion and peripheral blood eosinophilia. Thoracentesis revealed eosinophilic pleural effusion in which the smear, culture and cytological examinations were all negative.

Budesonide/formoterol maintenance and reliever therapy in moderate-to-severe asthma: effects on eosinophilic airway inflammation.

Although the budesonide and formoterol in a single inhaler for maintenance and reliever therapy has been evaluated in recent studies, the effects on eosinophilic airway inflammation remain uncertain. The purpose of this study was to compare the efficacy, including anti-inflammatory effects, of as-needed budesonide/formoterol with salbutamol in Japanese patients with moderate-to-severe asthma. Patients with asthma using an inhaled corticosteroid plus a long-acting beta2-agonist as a controller and at least one asthma exacerbation in the previous 12 months were randomized to budesonide/formoterol maintenance therapy (160/4.

Niflumic acid inhibits goblet cell degranulation in a guinea pig asthma model.

Background: Human Ca(2+)-activated Cl ion channel 1 (hCLCA1) is expressed in goblet cell hyperplasia in the airway of asthmatics, and murine CLCA3 is associated with antigen-sensitized and IL-13-induced goblet cell metaplasia in mice. However, the role of CLCA in goblet cell degranulation is not fully investigated. Niflumic acid (NFA), a relatively specific CLCA inhibitor, inhibits goblet cell metaplasia, but the effect of NFA on goblet cell degranulation has not been determined in an asthma model.

Double-stranded RNA and TGF-alpha promote MUC5AC induction in respiratory cells.

Viral infection is a major trigger for exacerbation of asthma and induces overproduction of mucins. We investigated whether dsRNA could amplify the induction of mucin by TGF-alpha in human bronchial epithelial cells, as well as the molecular mechanisms regulating MUC5AC expression. Human pulmonary mucoepidermoid carcinoma (NCI-H292) cells and normal human bronchial epithelial cells were exposed to polyinosinic-cytidyric acid (poly(I:C)) and TGF-alpha.

Elimination of IL-13 reverses established goblet cell metaplasia into ciliated epithelia in airway epithelial cell culture.

Background: Interleukin (IL)-13 induces goblet cell metaplasia and plays an important role in mucus hypersecretion in asthma. We previously reported that IL-13 induced goblet cell differentiation along with less ciliated cell differentiation in guinea pig tracheal epithelial cells in vitro. In this study, we asked whether elimination of IL-13 could reverse the established goblet cell metaplasia into ciliated epithelia.

Ultrafine carbon black particles stimulate proliferation of human airway epithelium via EGF receptor-mediated signaling pathway.

Exposure to ambient ultrafine particles induces airway inflammatory reactions and tissue remodeling. In this experiment, to determine whether ultrafine carbon black (ufCB) affects proliferation of airway epithelium and, if so, what the mechanism of action is, we studied human primary bronchial epithelial cell cultures. Incubation of cells in the serum-free medium with ufCB increased incorporations of [(3)H]thymidine and [(3)H]leucine into cells in a time- and dose-dependent manner.

Air exposure causes oxidative stress in cultured bovine tracheal epithelial cells and produces a change in cellular glutathione systems.

An air-liquid interface culture system accelerates cell differentiation and growth in airway epithelial cells. In this study, the authors investigated whether glutathione (GSH) affects cell growth in an air-liquid interface culture. Various components of the cellular GSH system were measured and the number of cells was counted after conversion from an immersed feeding to an air-liquid interface.