Lung transplantation outcomes of patients with interstitial pneumonia with autoimmune features: a single center retrospective cohort study.

Objective: Interstitial pneumonia with autoimmune features (IPAF) describes patients with interstitial lung disease (ILD) and autoimmune features without meeting criteria for a specific rheumatic disease. No longitudinal data exist on post-transplant outcomes in IPAF patients. We compared baseline demographics, pre-transplant characteristics, and post-transplant outcomes between IPAF and idiopathic pulmonary fibrosis (IPF) patients undergoing double lung transplantation.

Mycobacterium abscessus Complex Infections Among Lung Transplant Recipients: A Multicenter Retrospective Cohort Study in Canada.

Background: Mycobacterium abscessus complex is known to cause life-threatening disease in lung transplantation (LT) recipients (LTRs). Therefore, many centers consider the presence of M. abscessus as a relative contraindication to LT.

Impact of recipient age on mortality among Cytomegalovirus (CMV)-seronegative lung transplant recipients with CMV-seropositive donors.

Background: Cytomegalovirus (CMV)-seronegative lung transplant recipients (LTRs) with seropositive donors (CMV D+/R-) have the highest mortality of all CMV serostatuses. Due to immunosenescence and other factors, we hypothesized CMV D+/R- status might disproportionately impact older LTRs. Thus, we investigated whether recipient age modified the relationship between donor CMV status and mortality among CMV-seronegative LTRs.

Outcomes following heart or bilateral-lung transplantation from donors who died of drug toxicity in British Columbia, Canada.

Introduction: The illicit drug toxicity (overdose) crisis has worsened across Canada; between 2016 and 2021, more than 28,000 individuals have died of drug toxicity. Organ donation from persons who experience drug toxicity death (DTD) has increased in recent years. This study examines whether survival after heart or bilateral-lung transplantation differed by donor cause of death.

Does community size or commute time affect severity of illness at diagnosis or quality of care in a centralized care model of pulmonary hypertension?

Background: Centralized care models are often used for rare diseases like pulmonary hypertension (PH). It is unknown how living in a rural or remote area influences outcomes.

Methods: We identified all patients from our PH database who carried a diagnosis of WHO Group 1 or WHO Group 4 PH.

Evolving Paradigms in the Treatment of Atrial Septal Defects With Pulmonary Arterial Hypertension.

Atrial septal defects are one of the most frequently diagnosed congenital heart defects in adulthood. The presence of concurrent moderate or severe pulmonary arterial hypertension without Eisenmenger syndrome at the time of diagnosis can make for a challenging clinical scenario. There is continually evolving literature to determine the ideal approach to this subset of patients.

Brief Education Improves Proper Metered-Dose Inhaler Use.

Background: Inhaled β-agonists are the cornerstone of acute treatment for asthma and chronic lung disease. Upon emergency department (ED) discharge, patients optimally receive prescriptions for metered-dose inhalers (MDIs) with instructions on their proper use. Yet prior studies suggest that ED personnel have limited knowledge of proper MDI techniques.

Lessons from lung transplantation: Cause for redefining the pathophysiology of pulmonary hypertension in gaucher disease.

Background: Gaucher disease type 1 (GD1) is a lysosomal storage disease rarely resulting in end stage pulmonary hypertension (PH) and interstitial lung disease. There have only been two previous case reports of patients with GD1 receiving lung transplants.

Case Presentation: We report a case of successful bilateral sequential lung transplantation in a patient with end-stage GD1-related PH.

Clinical and hemodynamic factors in predicting response to fluid challenge during right heart catheterization.

Fluid challenge during right heart catheterization has been used for unmasking pulmonary hypertension (PH) related to left-sided heart disease. We evaluated the clinical and hemodynamic factors affecting the response to fluid challenge and investigated the role of fluid challenge in the classification and management of PH patients. We reviewed the charts of 67 patients who underwent fluid challenge with a baseline pulmonary arterial wedge pressure (PAWP) of ≤ 18 mmHg.

Successful lung transplantation in an HIV seropositive patient with desquamative interstitial pneumonia: a case report.

Background: Until recently, lung transplantation was not considered in patients with human immunodeficiency virus (HIV). HIV seropositive patients with suppressed viral loads can now expect long-term survival with the advent of highly active antiretroviral therapies (HAART); however, HIV remains a relative contraindication to lung transplantation. We describe, to our knowledge, the first HIV seropositive lung transplant recipient in Canada.

Whole exome sequencing analysis in severe chronic obstructive pulmonary disease.

Chronic obstructive pulmonary disease (COPD), one of the leading causes of death worldwide, is substantially influenced by genetic factors. Alpha-1 antitrypsin deficiency demonstrates that rare coding variants of large effect can influence COPD susceptibility. To identify additional rare coding variants in patients with severe COPD, we conducted whole exome sequencing analysis in 2543 subjects from two family-based studies (Boston Early-Onset COPD Study and International COPD Genetics Network) and one case-control study (COPDGene).

Comparison of thermodilution and indirect Fick cardiac outputs in pulmonary hypertension.

Background: The accurate measurement of cardiac output (CO) is required in patients with pulmonary hypertension (PH).While both the thermodilution (TDCO) and indirect Fick (IFCO) methods are commonly used, there is little data comparing them in patients with PH.

Methods: We performed a retrospective analysis of patients evaluated at our center.

Organizing Pneumonia in an Adult With Chronic Recurrent Noninfectious Osteomyelitis: A Case Report and Literature Review.

Chronic recurrent multifocal osteomyelitis (CRMO) is an idiopathic inflammatory disorder primarily of children and adolescents that is characterized by multifocal nonpyogenic relapsing and remitting inflammatory bone lesions. Pulmonary abnormalities are rarely associated with CRMO, with two reported cases of consolidation on chest CT that occurred in children. We present a case of organizing pneumonia in an adult patient with CRMO.

Family physician access to specialist advice by telephone: Reduction in unnecessary specialist consultations and emergency department visits.

Problem Addressed: Timely access to specialist care is an important issue for patients with mild to moderate symptoms, and wait times for referrals are currently quite long.

Objective Of Program: To provide FPs with quick telephone access to other specialists for treatment advice for patients with nonserious conditions that they would otherwise refer to specialist care.

Program Description: The RACE (Rapid Access to Consultative Expertise) program is a telephone hot-line providing FPs and nurse practitioners in the Vancouver, BC, area with timely access to specialist consultations.

Genetic Association and Risk Scores in a Chronic Obstructive Pulmonary Disease Meta-analysis of 16,707 Subjects.

The heritability of chronic obstructive pulmonary disease (COPD) cannot be fully explained by recognized genetic risk factors identified as achieving genome-wide significance. In addition, the combined contribution of genetic variation to COPD risk has not been fully explored. We sought to determine: (1) whether studies of variants from previous studies of COPD or lung function in a larger sample could identify additional associated variants, particularly for severe COPD; and (2) the impact of genetic risk scores on COPD.

Exome Array Analysis Identifies a Common Variant in IL27 Associated with Chronic Obstructive Pulmonary Disease.

Rationale: Chronic obstructive pulmonary disease (COPD) susceptibility is in part related to genetic variants. Most genetic studies have been focused on genome-wide common variants without a specific focus on coding variants, but common and rare coding variants may also affect COPD susceptibility.

Objectives: To identify coding variants associated with COPD.

Unclassifiable interstitial lung disease: an unresolved diagnostic dilemma.

Interstitial lung disease (ILD) classification requires a multidisciplinary review that includes input from an ILD clinician, chest radiologist, and lung pathologist. We report a case of ILD that remained unclassifiable due to discordant clinical, radiological, and pathological findings despite a thorough evaluation that included examination of explanted lung tissue. This case demonstrates that ILD can remain unclassifiable even with a complete evaluation and illustrates one approach to the management of such patients.

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Medical and surgical management for pulmonary arterial hypertension.

Prior to the availability of the pulmonary arterial hypertension (PAH)-specific therapy, PAH was a dreadful disease with a very poor prognosis. Better understanding of the complex pathobiology of PAH has led to a major therapeutic evolution. International regulatory agencies have approved many specific drugs with different pharmacologic pathways and routes of administration.

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: General management for pulmonary hypertension.

Treatment of pulmonary hypertension (PH) patients is challenging and should only be initiated after a comprehensive diagnostic evaluation. Such treatment should ideally be done in specialized centers with full capability for hemodynamic measurements, having access to a broad range of PAH therapies, and adequate experience in the management of critically ill patients. The following discussion is intended to review the general measures and the non-specific (supportive) therapy used in managing PH patients, while the specific therapy will be discussed in a subsequent different article.

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: 2014 updates.

The Saudi Association for Pulmonary Hypertension (previously called Saudi Advisory Group for Pulmonary Hypertension) has published the first Saudi Guidelines on Diagnosis and Treatment of Pulmonary Arterial Hypertension back in 2008.[1] That guideline was very detailed and extensive and reviewed most aspects of pulmonary hypertension (PH). One of the disadvantages of such detailed guidelines is the difficulty that some of the readers who just want to get a quick guidance or looking for a specific piece of information might face.