Radiographic Distribution as a Diagnostic Clue in Pulmonary Disease.

Because some disease processes produce radiographic abnormalities that occur in characteristic distributions in the chest, classifying the position and appearance of these suggestive features and the underlying diseases provides a tool by which diagnostic accuracy might be improved. The goal of this review is to offer to the chest clinician a taxonomy of these disease entities that can produce characteristic chest radiographic distributions. These radiographic distributions often reflect anatomic or physiologic conditions that drive the radiographic appearance; for example, foramen of Morgagni diaphragmatic hernias most commonly present in the right ventral chest, consistent with the anatomic location of the diaphragmatic foramen. This taxonomy includes 3 distributional categories: (1) upper versus lower lung zone-predominant processes, (2) central versus peripheral processes, and (3) processes with distinctive focal locations, eg, "photonegative appearance" as in chronic eosinophilic pneumonia. It is hoped that this taxonomy aids the chest clinician in generating and streamlining a differential diagnosis and in ascertaining the specific cause of diseases with radiographic abnormalities.

Computed tomography findings in bronchial asthma: quantification of air trapping and correlation with pulmonary function tests.

BACKGROUND: High-resolution computed tomography (HRCT) scans can help identify subsets of asthma patients who show rapid decline in lung function. PURPOSE: To evaluate high-resolution computed tomography (HRCT) findings in adult patients with asthma, obtain quantitative measurements of air trapping on expiratory scans, and correlate the findings with pulmonary function tests (PFTs). MATERIAL AND METHODS: Thirty adults with asthma with persistent-mild, persistent-moderate, and persistent-severe categories as per standard clinical guidelines were evaluated with inspiratory and expiratory HRCT for various imaging features of bronchial asthma. Expiratory HRCT scan were used to quantify degree of air-trapping, and their values quantified as "pixel index," using a special "density' mask" software. Complete spirometry and body plethysmography were performed on each patient within 0-2 days of HRCT scans. HRCT findings were correlated with the clinical severity groups and the CT pixel indices (PI) were correlated with the PFT results using correlation coefficients and linear regression analysis. RESULTS: The inspiratory CT findings did not correlate with increase in disease severity. Expiratory scans accurately quantified areas of air trapping. CT-PI correlated well with PFT values indicative of airway obstruction and airflow limitation, which helped differentiate patients with asthma with increasing severity from those with milder forms of the disease. CONCLUSION: Expiratory thin-section CT is a useful objective method to quantify air-trapping in people with asthma. The air-trapping score measured in "pixel indices" correlates well with PFT results and can successfully identify patients with severe asthma. Further studies are needed to confirm if this parameter can serve as a potential marker for airway remodeling and declining lung function.

Acute Dyspnea After Lung Transplantation: A Twisted Tale.

CASE PRESENTATION: A 67-year-old woman developed sudden-onset severe dyspnea 24 h after a bilateral sequential lung transplant for COPD. She had an uneventful surgery performed on cardiopulmonary bypass support. She required two units of packed RBCs as well as fresh frozen plasma during the surgery. She was successfully extubated within 12 h of surgery. Her primary graft dysfunction score was grade 2, 1, and 1 at 0, 12, and 24 h, respectively. Approximately 24 h after the procedure, she complained of increasing shortness of breath without fever, chills, cough, or pleuritic chest pain.

Recurrence of primary disease following lung transplantation.

Lung transplant has become definitive treatment for patients with several end-stage lung diseases. Since the first attempted lung transplantation in 1963, survival has significantly improved due to advancement in immunosuppression, organ procurement, ex vivo lung perfusion, surgical techniques, prevention of chronic lung allograft dysfunction and bridging to transplant using extracorporeal membrane oxygenation. Despite a steady increase in number of lung transplantations each year, there is still a huge gap between demand and supply of organs available, and work continues to select recipients with potential for best outcomes. According to review of the literature, there are some rare primary diseases that may recur following transplantation. As the number of lung transplants increase, we continue to identify disease processes at highest risk for recurrence, thus shaping our future approaches. While the aim of lung transplantation is improving survival and quality of life, choosing the best recipients is crucial due to a shortage of donated organs. Here we discuss the common disease processes that recur and highlight its impact on overall outcome following lung transplantation.

A confounding bronchopulmonary situs abnormality after bilateral lung transplantation.

A 36-year-old female with a past medical history of primary ciliary dyskinesia and bilateral lung transplantation was noted to have a rare and confounding postsurgical anatomy acquired as a result of transplantation. Bronchoscopy and computed tomography showed isomeric main bronchi with a tri-lobed right lung, a bi-lobed left lung and dextrocardia. This rare phenomenon can be observed in lung transplant recipients with situs ambiguous morphology of their native lungs, who receive donor lungs with normal situs solitus morphology. To the best of our knowledge, ours is the first reported case of such a composite bronchopulmonary situs abnormality. Careful review of bronchial anatomy should be done with the help of CT imaging prior to undertaking any bronchial interventions in these subset of patients with bronchial isomerism and bilateral lung transplantation.

Congenital Left Atrial Aneurysms.

Online supplemental material is available for this article.

Recycling plastic: diagnosis and management of plastic bronchitis among adults.

Plastic bronchitis is a rare, underdiagnosed and potentially fatal condition. It is characterised by the formation and expectoration of branching gelatinous plugs that assume the shape of the airways. These airway plugs differ from the allergic mucin that characterises allergic bronchopulmonary aspergillosis and mucoid impaction of the bronchi. Plastic bronchitis is most often encountered in the paediatric population following corrective cardiac surgery, such as the Fontan procedure. It also occurs in adults. Plastic bronchitis in adults is rare, heterogeneous in its aetiology, and can lead to respiratory distress or even life-threatening airway obstruction. Plastic bronchitis in adulthood should not be overlooked, particularly in patients with chronic inflammatory lung diseases. This review presents current understanding of the presentation, aetiology, pathogenesis, pathology and management of plastic bronchitis in adults.

A case of hypereosinophilic syndrome with STAT5b N642H mutation.

Hypereosinophilia is defined as persistent eosinophilia (>1.5 × 109/L). Hypereosinophilic syndrome (HES) is a term used to describe a group of disorders characterized by sustained hypereosinophilia associated with end-organ damage. Based on underlying molecular mechanism of eosinophilia, there are different subtypes of HES. Diagnosis of HES subtype can be challenging, especially in the absence of overt lymphoid/myeloid neoplasms or discernable secondary causes. Long-term outpatient follow-up with periodic complete blood count and repeated bone marrow biopsy may be needed to monitor disease activity. Somatic signal transducer and activation transcription 5b (STAT5b) N642H mutation was recently found to be associated with myeloid neoplasms with eosinophilia. We report a case of HES who presented with pulmonary embolism and acute eosinophilic pneumonia, found to have recurrent STAT5b N642H mutation by next-generation sequencing, suggesting possible underlying myeloid neoplasm.

A Unique Case of Combined Nodular and Tracheobronchial Amyloidosis.

Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of misfolded proteins that can affect either systemically or locally confined to one system. Pulmonary amyloidosis is rare and can be classified into three forms according to the anatomic site of involvement: nodular pulmonary amyloidosis, tracheobronchial amyloidosis and diffuse alveolar-septal amyloidosis. The former two usually represent localized amyloid disease and the latter represents systemic disease. Typically lung parenchymal and tracheobronchial amyloidosis do not present together in localized forms of pulmonary amyloidosis. Here we report a unique case of localized pulmonary immunoglobulin light-chain amyloidosis, manifested as both parenchymal nodules and tracheobronchial amyloid deposition.