Canadian respiratory journal : journal of the Canadian Thoracic Society
-
Pulmonary hypertension (PH) in patients with connective tissue disease (CTD) can occur in isolation or concomitantly with interstitial lung disease (ILD). Targeted therapies for PH can mitigate clinical deterioration in CTD patients with isolated PH; however, the effect of these therapies in CTD patients with PH and ILD (CTD-PH-ILD) are poorly characterized. ⋯ Treatment using PH-specific therapies in patients with CTD, PH and ILD was well tolerated. Further studies to investigate the efficacy of PH-specific therapies in CTD-PH-ILD patients are warranted.
-
Chronic obstructive pulmonary disease (COPD) is a complex, multisystem disorder that often results in exacerbations requiring emergency department (ED) management. Following an exacerbation and discharge from the ED, reassessment and management adjustment with a health care provider are recommended to re-establish control of the disease. ⋯ More than 30% of COPD ED visits resulted in admission; regional variation was significant. Moreover, discharged patients experienced delayed follow-up and often required repeat ED visits. Interventions to improve reassessment and reduce COPD-related repeat ED visits should be explored.
-
A large kindred of familial pulmonary fibrosis is reported. Six members from the first two generations of this particular kindred were described more than 40 years previously; six more individuals from the third and fourth generations have also been evaluated. The proband, now 23 years of age, has mild disease; the other 11 documented affected family members all died from their disease at an average age of 37 years (range 25 to 50 years). ⋯ However, the initial radiographic pattern in many of these individuals was upper lobe and nodular and, along with the young age, was atypical for idiopathic pulmonary fibrosis. Several genetic abnormalities have been associated with familial pulmonary fibrosis. The present study examined the genes coding for surfactant protein-C, ATPbinding cassette protein A3 and telomerase, and found no abnormalities.
-
Diagnosed obstructive sleep apnea (OSA) affects 2% to 7% of middle-age persons worldwide and represents a substantial health care burden. The gold standard for treating OSA in adults is continuous positive airway pressure (CPAP) therapy. Compliance with this treatment is especially important in OSA patients experiencing concomitant acute and chronic disease or illness, and those undergoing procedures associated with sedation, analgesia and anesthesia. ⋯ Only a small percentage of hospitalized OSA patients were documented as having received CPAP therapy during their stay. Issues relating to the accuracy, specificity and completeness of the Canadian Institute for Health Information's hospital Discharge Abstract Database specific to OSA and its management were identified. Practices pertaining to the reporting, coding and management of hospitalized adult OSA patients warrant further investigation and research.
-
Review Case Reports
Rare lung diseases III: pulmonary Langerhans' cell histiocytosis.
Pulmonary Langerhans' cell histiocytosis (PLCH) is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. The current review discusses the presenting features and relevant diagnostic testing and treatment options for PLCH in the context of a clinical case. ⋯ A major theme of the current series of articles on rare lung diseases has been the translation of insights gained from fundamental research to the clinic. Accordingly, the understanding of dendritic cell biology in this disease has led to important advances in the care of patients with PLCH.