Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases.

Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased quality of life and, potentially, early death. Idiopathic pulmonary fibrosis may be regarded as a model for other progressive-fibrosing ILDs. Here we focus on other ILDs that may present a progressive-fibrosing phenotype, namely idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, connective tissue disease-associated ILDs (e.g. rheumatoid arthritis-related ILD), fibrotic chronic hypersensitivity pneumonitis, fibrotic chronic sarcoidosis and ILDs related to other occupational exposures. Differential diagnosis of these ILDs can be challenging, and requires detailed consideration of clinical, radiological and histopathological features. Accurate and early diagnosis is crucial to ensure that patients are treated optimally.

Superoxide dismutase 3 dysregulation in a murine model of neonatal lung injury.

Bronchopulmonary dysplasia (BPD), a common chronic respiratory disease that occurs after premature birth, is believed to be secondary to oxidative damage from hyperoxia and inflammation, which leads to impaired alveolar formation and chronic lung dysfunction. We hypothesized that extracellular superoxide dismutase (SOD)3, an antioxidant uniquely targeted to the extracellular matrix (ECM) and alveolar fluid, might have a different response (down-regulation) to hyperoxic injury and recovery in room air (RA), thereby contributing to the persistent airspace injury and inflammation. We used a murine BPD model using postnatal hyperoxia (O2) (4 or 5 d) followed by short-term recovery (14 d) in RA, which mimics the durable effects after injury during alveolar development. This was associated with significantly increased mRNA expression for antioxidant genes mediated by nuclear factor erythroid 2-related factor (Nrf2) in the O2 (n = 4) versus RA group (n = 5). SOD3, an Nrf2-independent antioxidant, was significantly reduced in the O2-exposed mice compared with RA. Immunohistochemistry revealed decreased and disrupted SOD3 deposition in the alveolar ECM of O2-exposed mice. Furthermore, this distinct hyperoxic antioxidant and injury profile was reproducible in murine lung epithelial 12 cells exposed to O2. Overexpression of SOD3 rescued the injury measures in the O2-exposed cells. We establish that reduced SOD3 expression correlates with alveolar injury measures in the recovered neonatal hyperoxic lung, and SOD3 overexpression attenuates hyperoxic injury in an alveolar epithelial cell line. Such findings suggest a candidate mechanism for the pathogenesis of BPD that may lead to targeted interventions.

[Benign tracheal stenosis treated by T-tube in a patient with autism; report of a case].

A female with autism, aged over 40 years, who had been hospitalized in a nursing home, developed descending necrotizing mediastinitis requiring tracheostomy. Subsequently, tracheal stenosis was observed. She was referred to our hospital. T-tube therapy was selected, and there has been no recurrence during the 3-year follow-up. We report a patient in whom a T-tube was useful for treating benign tracheal stenosis in the presence of autism.

[Surgical fixation for flail chest].

Flail chest occurs by blunt chest trauma and is associated with pulmonary contusion, atelectasis, pneumothorax, hemothorax, and respiratory failure. Because of its severity, it may need internal pneumatic stabilization or surgical fixation. Some patients do not need the internal stabilization and are observed conservatively. Some of these patients, however, increase the flail after palliating the pain and getting up. These patients show inefficient ventilation and surgical fixation is needed. The operation should be performed after the improvement of pulmonary contusion. In this paper, we presented 2 patients who showed such course and clarified the surgical methodology.

[A case of a perforated duodenal ulcer coincident with a gastric carcinoma].

A 44-year-old male patient under treatment for a duodenal ulcer for the past 9 years visited our clinic because of a sudden pain in the epigastric region. He was diagnosed as having a perforated duodenal ulcer, so an emergency laparotomy was performed. In this operation, the coexistence of a gastric carcinoma was clarified, and a subtotal gastrectomy with an R2 dissection was done at the same time. The coexistent gastric carcinoma was localized to a size of 20 x 12 mm within the gastric proper mucosa. The infiltrated range of the cancer was as wide as 120 x 80 mm, extending to the proper tunica muscularis. This case was seen as manifesting a relative early stage of scirrhous carcinoma.