Clinical and Pathophysiological Features of High-altitude Pulmonary Edema in the Japanese Population: A Review of Studies on High-altitude Pulmonary Edema in Japan.

High-altitude pulmonary edema (HAPE) is a life-threatening, noncardiogenic pulmonary edema that occurs in unacclimatized individuals rapidly ascending to high altitudes above 2,500 m above sea level. Until the entity of HAPE was first identified in a case report published in Japan in 1966, the symptoms of severe dyspnea or coma occurring in climbers of the Japan Alps were incorrectly attributed to pneumonia or congestive heart failure. The Shinshu University Hospital serves as the central facility for rescuing and treating patients with HAPE in the region.

Twenty-year Follow-up of the First Bilateral Living-donor Lobar Lung Transplantation in Japan.

Patients with end-stage lung disease can undergo living-donor lobar lung transplantation (LDLLT), with survival rates improving every year. We herein report the 20-year follow-up findings of the first patient who underwent LDLLT in Japan. A 24-year-old woman with primary ciliary dyskinesia became ventilator-dependent after severe respiratory failure and right-sided heart failure following repeated respiratory infections.

Serum soluble interleukin-2 receptor as a biomarker in immunoglobulin G4-related disease.

Objectives: Serum soluble interleukin-2 (IL-2) receptor (sIL-2R) might reflect disease activity in immunoglobulin G4-related disease (IgG4-RD). We aimed to elucidate the clinical significance of blood markers, including sIL-2R, in patients with IgG4-RD.

Methods: We enrolled 59 patients with IgG4-RD and investigated the association between blood markers (white blood cells, C-reactive protein, sIL-2R, IgG, IgG4, IgE, total hemolytic complement), and clinical indices.

Cytokine profiles in the BAL fluid of IgG-related respiratory disease compared with sarcoidosis.

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Cardiac hypertrophy is exacerbated in aged mice lacking the osteoprotegerin gene.

Aims: Osteoprotegerin (OPG) may play a role in the progression of cardiac hypertrophy and heart failure. However, its pathophysiological role in changes in cardiac structure and function with ageing remains to be elucidated.

Methods And Results: We conducted experiments using 2.

Late-onset rejection of a unilateral donor lung with vascular C4d deposition in bilateral living-donor lobar lung transplantation: an autopsy case report.

A 37-year-old woman had undergone bilateral living-donor lobar lung transplantation 11 years previously for idiopathic pulmonary arterial hypertension. Her father donated the right lobe and her brother donated the left lobe. She subsequently developed progressively worsening respiratory dysfunction due to pneumonia.

Biological properties of adrenomedullin conjugated with polyethylene glycol.

Adrenomedullin (AM) is a vasodilator peptide with pleiotropic effects, including cardiovascular protection and anti-inflammation. Because of these beneficial effects, AM appears to be a promising therapeutic tool for human diseases, while intravenous injection of AM stimulates sympathetic nerve activity due to short-acting potent vasodilation, resulting in increased heart rate and renin secretion. To lessen these acute reactions, we conjugated the N-terminal of human AM peptide with polyethylene glycol (PEG), and examined the biological properties of PEGylated AM in the present study.

Thrombomodulin for acute exacerbations of idiopathic pulmonary fibrosis: a proof of concept study.

Introduction: The mortality of acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is high. Anticoagulation therapy (recombinant human soluble thrombomodulin (rhTM)) is recognized as a potential new strategy for treating disseminated intravascular coagulation in Japan. This preliminary study was to evaluate whether the coagulation factors increase or decrease in AE-IPF-patients, and whether the additional administration of rhTM for AE-IPF-patients has any beneficial effects on inflammatory mediators and activated coagulation.

The potential efficacy of noninvasive ventilation with administration of a neutrophil elastase inhibitor for acute respiratory distress syndrome.

Purpose: Noninvasive ventilation (NIV) can reduce the need for invasive mechanical ventilation. The aim of this investigation was to determine whether the combination of NIV with administration of a neutrophil elastase inhibitor could improve outcome and respiratory conditions in acute respiratory distress syndrome (ARDS)-patients, according to the Berlin definition.

Methods: ARDS-patients were treated with NIV and a neutrophil elastase inhibitor.

Viral infections in patients with an acute exacerbation of idiopathic interstitial pneumonia.

Background: Patients with slowly progressive idiopathic interstitial pneumonia, including idiopathic pulmonary fibrosis, often deteriorate, thus suggesting that the clinical course may be unpredictable. Such episodes are termed acute exacerbation of idiopathic interstitial pneumonia. The etiology of an acute exacerbation of idiopathic interstitial pneumonia is unknown.

Hydrogen peroxide content and pH of expired breath condensate from patients with asthma and COPD.

Background: Oxidative stress is implicated in the pathogenesis of asthma and chronic obstructive pulmonary disease (COPD). Analysis of the expired breath condensate (EBC) has been suggested to provide non-invasive inflammatory markers that reflect oxidative stress in the airways.

Objective: The present study attempts to elucidate whether the hydrogen peroxide (H2O2) levels and pH values in EBC may be useful as biomarkers of the activity or severity of asthma and COPD.

Polymorphisms of the tissue inhibitor of metalloproteinase 3 gene are associated with resistance to high-altitude pulmonary edema (HAPE) in a Japanese population: a case control study using polymorphic microsatellite markers.

Introduction: High-altitude pulmonary edema (HAPE) is a hypoxia-induced, life-threatening, high permeability type of edema attributable to pulmonary capillary stress failure. Genome-wide association analysis is necessary to better understand how genetics influence the outcome of HAPE.

Materials And Methods: DNA samples were collected from 53 subjects susceptible to HAPE (HAPE-s) and 67 elite Alpinists resistant to HAPE (HAPE-r).

Comparative analysis of PET findings and clinical outcome in patients with primary mediastinal seminoma.

Background: Primary mediastinal seminoma is a rare neoplasm. Cisplatin-based chemotherapy is the standard treatment, but management of post-chemotherapy seminoma residuals is still controversial. We encountered four cases of primary mediastinal seminoma and reviewed the clinical characteristics and outcomes, focusing on tumor size and F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) findings after chemotherapy.

Sivelestat prevents cytoskeletal rearrangements in neutrophils resulting from lung re-expansion following one-lung ventilation during thoracic surgery.

Patients undergoing lobectomy are at risk of developing acute lung injury resulting from one-lung ventilation (OLV) during surgery. We investigated the morphological and functional behavior of neutrophils in patients who underwent lobectomy and assessed the ability of sivelestat to inhibit neutrophil activity. This was a blinded randomized study.

Annual changes in pulmonary function in combined pulmonary fibrosis and emphysema: over a 5-year follow-up.

Background: Combined pulmonary fibrosis and emphysema (CPFE) is a unique disorder that has been previously described, and the distinct features of CPFE in comparison with chronic obstructive pulmonary disease (COPD) have been reported. However, the yearly dynamics of pulmonary function parameters in CPFE patients compared with those in COPD patients have not yet been reported.

Methods: We retrospectively enrolled patients with CPFE and COPD who had undergone pulmonary function tests more than five times during a follow-up period of more than five years.

Clinical characteristics and outcomes of patients with small cell lung cancer detected by CT screening.

The present study was performed to evaluate the clinical characteristics and survival outcomes in patients with small cell lung cancer (SCLC) detected by low-dose computed tomography (CT). We retrospectively reviewed clinical records of patients with SCLC treated at our hospital between 1997 and 2011 and selected patients with SCLC detected by CT screening. We identified 12 patients (male/female 11/1; mean age 67.

Primary malignant sarcomatoid mesothelioma in the pericardium.

Primary malignant pericardial mesothelioma is an exceptionally rare tumor, and making an antemortem diagnosis of this disease is notoriously difficult. We herein report the case of a 61-year-old woman with pericardial mesothelioma who presented with shortness of breath and peripheral edema of the lower limbs. Chest computed tomography (CT) showed an anterior mass and thickened pericardium with multiple pericardial nodules.

Additive efficacy of short-acting bronchodilators on dynamic hyperinflation and exercise tolerance in stable COPD patients treated with long-acting bronchodilators.

The purpose of this study was to clarify the additive efficacy of short-acting β(2)-agonists (SABA) or muscarinic antagonists (SAMA) on dynamic hyperinflation and exercise tolerance in patients with chronic obstructive pulmonary disease (COPD) who had been treated with long-acting bronchodilators. Thirty-two patients with stable COPD who had been treated with long-acting bronchodilators, including long-acting muscarinic antagonists (LAMA), were examined by pulmonary function tests, dynamic hyperinflation evaluated by the method of step-wise metronome-paced incremental hyperventilation, and the incremental shuttle walking test before and after inhalation of SABA or SAMA. The additive efficacy of the two drugs was analyzed.