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The Clinical and Research Utility of Bronchoscopy in Cystic Fibrosis

Bronchoscopy is used in the management of cystic fibrosis (CF) for multiple reasons, of which sampling respiratory secretions has been the most frequent. The evidence behind the role of bronchoscopy for routine sampling of respiratory secretions to determine clinical management is lacking, although there remain well-accepted indications for bronchoscopy in research and follow-up of advanced therapies in the CF population. We review the clinical and research utility of bronchoscopy in patients with CF and outline promising research uses of this tool that can contribute to recent advances in understanding and treating CF. …

Unilateral Multicystic Pulmonary Disease in a Young Adult: An Unusual Diagnosis

Congenital pulmonary airway malformation (CPAM) is an uncommon developmental hamartomatous anomaly caused by abnormal branching of immature bronchioles with replacement of the lung by cysts/disorganized tissue. CPAMs are usually lobar or sublobar in extent, and ∼90% of lesions are identified within the first 2 years of life. CPAM occupying an entire lung, with a delayed adulthood presentation, is extremely rare; only 4 such occurrences have been previously described. We present a case of a 20-year-old woman diagnosed with unilateral entire lung CPAM on the basis of radiologic findings that was surgically resected via video-assisted thoracoscopic surgery. …

Subtle But Clinically Significant: A Review of Antibody Deficiencies in Adults

Primary immunodeficiency diseases are becoming more frequently diagnosed in the adult population. Although infections are the most common symptoms, patients can also present with sequelae of autoimmune and noninfectious pulmonary diseases, such as interstitial lung disease and granulomatous disease. Pulmonologists are likely to encounter patients with various and, at times, subtle symptoms suggestive of immunodeficiency. In this review article, we discuss the presentation, evaluation, and management of 4 antibody deficiencies: common variable immunodeficiency, selective immunoglobulin A deficiency, specific antibody deficiency, and immunoglobulin G subclass deficiency. …

Noninvasive Oxygenation Strategies For Acute Exacerbation of Interstitial Lung Disease: A Retrospective Single-center Study and a Review of the Literature

Acute exacerbation of interstitial lung disease (AE-ILD) causes severe respiratory failure so that the patients would often require mechanical ventilation. Mechanical ventilation may improve survival in selected patients with AE-ILD; however, in-hospital mortality rate is so high in patients who need mechanical ventilation that the use of mechanical ventilation is weakly recommended. Although there is no fixed strategy with regard to selection of oxygen devices, a palliative approach to how to spend the end-of-life periods is often needed. Although noninvasive ventilation may be used to avoid intubation, high-flow nasal cannula (HFNC) was developed in recent years and has been feasible and useful for acute respiratory failure of various causes. In terms of the quality of life, moreover, HFNC systems are associated with patient comfort and tolerance and more ability for oral intake of nutrition and less occurrence of cognitive dysfunction or coma. HFNC has broad utility, and it is likely that usage will increase more and more in the future. Limited data of its use in AE-ILD are available, and further study will be necessary. …

Neurological Emergencies in the Intensive Care Unit

Neurological emergencies present a unique challenge in the intensive care unit, requiring rapid evaluation and treatment. There is a complex interplay in critically ill patients, wherein illness affects the brain and the brain can, in turn, affect the illness, making diagnosis and management challenging. In this review, we discuss the diagnosis and management of common neurological issues that may be encountered in the intensive care unit, including elevated intracranial pressure, traumatic brain injury, ischemic stroke, intracerebral hemorrhage, subarachnoid hemorrhage, status epilepticus, neurological complications of critical illness, neuromuscular respiratory failure, and neurological complications in the immunocompromised. …

Cough and Nasal Pain: Putting Together an Uncommon Diagnosis

The pulmonary vasculitides include a wide spectrum of disorders that have in common inflammation and destruction of pulmonary blood vessels. Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is the most common of the antineutrophil cytoplasmic antibody–associated small-vessel vasculitides, and affects a number of organ systems. GPA affects a wide age range, although it typically presents in middle adulthood. The classic triad of GPA—upper airway involvement (sinusitis, otitis media, mucosal ulcerations, airway inflammation), lower respiratory tract disease, and glomerulonephritis—is not always evident at presentation. Furthermore, limited forms of GPA occur, in which the expected upper airway and renal involvement may be lacking. The imaging manifestations of GPA commonly include multiple, bilateral nodules or masses, frequently with cavitation or necrosis. Diffuse lung opacity due to alveolar hemorrhage, or tracheobronchial inflammation that may result in stenosis, may be encountered. Systemic GPA may be diagnosed when cytoplasmic antineutrophil cytoplasmic antibody/anti-proteinase 3 antibodies are detected in the proper clinical context, but, often, the diagnosis of GPA relies on recognition of a combination of common clinical, laboratory, and imaging findings with typical histopathologic changes noted from a biopsy of the affected tissue. A combination of a corticosteroid and immunomodulatory agents is used to induce and maintain remission in patients with GPA. Although potentially fatal if untreated, immunosuppressive therapy has dramatically improved survivorship in patients with GPA. …