BACKGROUND:In cystic fibrosis (CF), omalizumab has been used for difficult-to-treat asthma and allergic bronchopulmonary aspergillosis (ABPA) but safety and efficacy data are limited for this population.METHODS:We assessed patients receiving omalizumab for asthma or ABPA in the Toronto adult CF center between 2005 and 2017. We evaluated treatment safety and efficacy by analyzing changes in FEV1% predicted (FEV1pp) max value, slope and variability captured by the area under the curve (AUC), the cumulative dose of systemic corticosteroids (SCS), use of intravenous (IV) antibiotics and hospitalization days before omalizumab and up to 1 year after treatment initiation. Linear mixed effects model was used for FEV1pp slope and the trapezoidal rule for FEV1pp AUC.RESULTS:Twenty-seven CF patients received omalizumab, 16 (59.3%) for asthma and 11 (40.7%) for ABPA. No significant omalizumab-related adverse effects were observed. In the asthmatic group, the max value of FEV1pp improved on omalizumab and the cumulative dose of SCS decreased. In the ABPA group, the rate of FEV1pp decline (slope) and the variability of FEV1pp (AUC) improved on omalizumab. In ABPA patients, the cumulative SCS dose was not significantly different but 4 (36%) patients decreased their SCS dose by >50% compared to baseline. Days on IV antibiotics and hospital days did not differ significantly before and while on omalizumab therapy.CONCLUSIONS:In adult CF patients with difficult-to-treat asthma or ABPA, omalizumab should be considered. Larger studies are needed to identify patient characteristics that may predict response to omalizumab.

A 35-year-old healthy African-American woman presented with a 4-month history of gradual loss of vision in the right eye from an optic neuropathy. MRI of the orbits with gadolinium showed isolated thickening and enhancement of the right optic nerve sheath. Chest x-ray and CT-scan of the chest were performed and showed bilateral hilar and mediastinal lymphadenopathy. This was suggestive of sarcoidosis, and the diagnosis was confirmed with histopathology. The patient promptly recovered vision with high-dose corticosteroids; the thickening of the optic nerve sheath also regressed. Isolated optic nerve sheath thickening from sarcoidosis is rare and may mimic compressive optic neuropathies such as optic nerve sheath meningiomas. A systemic evaluation for systemic inflammatory etiologies should be considered in such cases.

Patient registries provide clinicians, patients and families with the ability to track important health outcomes at a population, cystic fibrosis (CF) center, and patient level. International quality improvement (QI) work driven by registries has been effective at improving the health and the care delivered to the individual patient. In this review, we examine the role CF registries have played in the QI process over the years and discuss the inherent strengths and limitations that exist when using registry data for this purpose.

Choosing Wisely is a campaign that aims to help clinicians and patients engage in conversations regarding unnecessary tests and treatments, in order to improve quality of care and reduce waste in healthcare. Specialty societies are asked to develop lists of commonly used tests and treatments that are not supported by evidence and/or could expose patients to unnecessary harm. The Canadian Thoracic Society appointed a 5-member Choosing Wisely Task Force to develop this list. After establishing evidence-based criteria for recommendation selection and prioritization, they generated an initial list of candidate recommendations from: 1) existing respiratory-related US and Canadian Choosing Wisely recommendations; 2) Canadian Medical Association (CMA) Patient-Oriented Evidence that Matters (POEMs (TM)) rated by >= 10% of CMA respondents to: "... help to avoid unnecessary or inappropriate treatment, diagnostic procedures, preventative interventions or a referral..."; and 3) additional suggestions by CTS content experts. The list was serially reduced through voting by members of the Canadian Respiratory Guidelines Committee and the Task Force in three electronic Delphi processes and by members of the CTS in an online poll (members were also asked to suggest additional recommendations). Evidence reviews were performed for the top 10 recommendations. This resulted in the following CTS Choosing Wisely Top 6 List: 1) Don't initiate long-term maintenance inhalers in stable patients with suspected COPD if they have not had confirmation of post-bronchodilator airflow obstruction with spirometry; 2) Don't perform CT screening for lung cancer among patients at low risk for lung cancer; 3) Don't perform chest computed tomography (CT angiography) or ventilation-perfusion scanning to evaluate for possible pulmonary embolism in patients with a low clinical probability and negative results of a highly sensitive D-dimer assay; 4) Don't treat adult cough with antibiotics even if it lasts more than 1 week, unless bacterial pneumonia is suspected (mean viral cough duration is 18 days); 5) Don't initiate medications for asthma (e.g., inhalers, leukotriene receptor antagonists, or other) in patients >= 6 years old who have not had confirmation of reversible airflow limitation with spirometry, and in its absence, a positive methacholine or exercise challenge test, or sufficient peak expiratory flow variability; and 6) Don't use antibiotics for acute asthma exacerbations without clear signs of bacterial infection. This list was developed through a rigorous and novel process and addresses overuse in different areas of respiratory medicine in Canada. It can provide a starting point for a systematic implementation process targeting clinicians and patients, to the benefit of patients and the healthcare system in general.

Purpose of review Survival for patients with cystic fibrosis (CF) continues to improve. The proportion of CF patients over the age of 18 years is nearly 50%, and care providers will need to better understand this patient population. Despite these improvements, young females continue to have a worse prognosis and lower median survival compared with their male counterparts. Contributing factors to the difference in survival remain uncertain. Recent findings The ‘gender gap’ remains an area of controversy. Recent data suggest that it still exists, though exact reasons remain unclear. For those patients diagnosed in adulthood, outcomes are also improving. Some evidence suggests persistence of the gender gap. Other data suggest a reversal of this effect. Additional work and study are needed. Summary This review finds supporting evidence for persistence of the gender gap and outlines the effect of age and sex on survival in CF. The majority of patients with CF will now be adults; thus, care providers must be aware of the impact this will have on ongoing patient management.

Pulmonary sequestration is described as a dysplastic mass of lung tissue that lacks communication with the tracheobronchial tree and receives systemic rather than pulmonary arterial blood supply. Two distinct classifications, intralobar and extralobar, have been described. The present article discusses the etiology, clinical and radiographic features of pulmonary sequestration as well as the management of this condition when it is discovered incidentally.

Purpose of reviewSurvival for patients with cystic fibrosis (CF) continues to improve. The proportion of CF patients over the age of 18 years is nearly 50%, and care providers will need to better understand this patient population. Despite these improvements, young females continue to have a worse prognosis and lower median survival compared with their male counterparts. Contributing factors to the difference in survival remain uncertain.Recent findingsThe gender gap' remains an area of controversy. Recent data suggest that it still exists, though exact reasons remain unclear. For those patients diagnosed in adulthood, outcomes are also improving. Some evidence suggests persistence of the gender gap. Other data suggest a reversal of this effect. Additional work and study are needed.SummaryThis review finds supporting evidence for persistence of the gender gap and outlines the effect of age and sex on survival in CF. The majority of patients with CF will now be adults; thus, care providers must be aware of the impact this will have on ongoing patient management.

Healthcare quality has received sustained attention since the release of To Err is Human by the US Institute of Medicine in late 1999.1 This report captured widespread interest with the oft-quoted estimate that medical errors annually cause 44 000–98 000 deaths in US hospitals alone. This period also coincided with publication of ‘An organisation with a memory’,2 which described the scale and nature of serious failures in the UK National Health Service. A widely accepted definition describes quality as the degree to which health services for individuals and populations increase the likelihood of desired health outcomes and are consistent with current professional knowledge.3 4 This definition further characterised quality in terms of six dimensions: safety, effectiveness, patient centeredness, timeliness, efficiency and equitability. Numerous studies document major shortcomings in each of these dimensions across a range of clinical settings.3 One illustrative study5 showed that only 55% of Americans with chronic medical conditions received basic aspects of acute, chronic and preventive care.5 For example, only 50% of patients with asthma received chronic inhaled corticosteroids and a similarly low percentage of patients with chronic obstructive pulmonary disease (COPD) received influenza vaccination. These major shortfalls in effective healthcare do not simply reflect access issues, as comparable data from Canada (with universal public healthcare) show that only 56% of patients with COPD had undergone spirometry as recommended by guidelines and only 34% received guideline-concordant treatment.6 Given that COPD will become the third leading cause of death by 20307 and represents the one common cause of death for which mortality rates continue to climb, we must improve adherence to evidence-based aspects of COPD management.8 Quality improvement (QI) is a science9 and includes numerous distinct strategies for changing patient and provider behaviour, as well …

Résumé Objectif Décrire un cas d’embolie graisseuse survenu suite à une chirurgie élective de relâchement de contracture du tendon chez un patient souffrant de dystrophie myotonique, et souligner l’importance de la prise en compte de ce syndrome dans le diagnostic différentiel de dysfonctionnement neuro-cognitif postopératoire aigu. Éléments cliniques Un homme de 34 ans souffrant de dystrophie myotonique a été opéré sans incident sous anesthésie régionale pour relâcher la contracture d’un tendon. Immédiatement après l’opération, des complications neurologiques et respiratoires sont apparues, nécessitant une admission aux soins intensifs. Le patient a manifesté la triade clinique classique associée à l’embolie graisseuse : hypoxémie, déficit neurologique et pétéchies. Un diagnostic d’embolie graisseuse a été établi mais, malgré un traitement à base de liquides intraveineux et d’inotropes, le patient est décédé. Il n’y avait pas de shunt intracardiaque établi, ce qui aurait suggéré qu’un shunt intrapulmonaire physiologique aurait pu être responsable du développement de manifestations systémiques d’embolie graisseuse. Conclusions Le dysfonctionnement neurologique postopératoire est un état difficile ayant de nombreuses causes possibles. Toutes les étiologies possibles, y compris l’embolie graisseuse, doivent être prises en compte dans le diagnostic différentiel et dans la prise en charge postopératoire des patients qui développent un déficit neurologique aigu postopératoire et de l’hypoxémie.

PURPOSE:To describe a case of fat embolism syndrome (FES) following elective tendon contracture release in a patient with myotonic dystrophy, to highlight the importance of considering this entity in the differential diagnosis of acute postoperative neurocognitive dysfunction.CLINICAL FEATURES:A 34-yr-old man with myotonic dystrophy underwent uneventful tendon contracture release under regional anesthesia. In the immediate postoperative period, neurological and respiratory complications developed, requiring intensive care support. The patient showed the classical clinical triad of hypoxemia, neurological impairment and a petechial rash associated with the FES. A diagnosis of FES was made and, despite therapy including fluid and inotropic support, the patient succumbed to the condition. There was no demonstrated intracardiac shunt, suggesting a physiological intrapulmonary shunt was responsible for the development of systemic manifestations of FES.CONCLUSIONS:Postoperative neurological dysfunction is a difficult condition with numerous possible causes. All possible etiologies, including FES, need to be considered in the differential diagnosis and postoperative management of patients developing acute postoperative neurological impairment and hypoxemia.

Purpose: To describe a case of fat embolism syndrome (FES) following elective tendon contracture release in a patient with myotonic dystrophy, to highlight the importance of considering this entity in the differential diagnosis of acute postoperative neurocognitive dysfunction. Clinical features: A 34-yr-old man with myotonic dystrophy underwent uneventful tendon contracture release under regional anesthesia. In the immediate postoperative period, neurological and respiratory complications developed, requiring intensive care support. The patient showed the classical clinical triad of hypoxemia, neurological impairment and a petechial rash associated with the FES. A diagnosis of FES was made and, despite therapy including fluid and inotropic support, the patient succumbed to the condition. There was no demonstrated intracardiac shunt, suggesting a physiological intrapulmonary shunt was responsible for the development of systemic manifestations of FES. Conclusions: Postoperative neurological dysfunction is a difficult condition with numerous possible causes. All possible etiologies, including FES, need to be considered in the differential diagnosis and postoperative management of patients developing acute postoperative neurological impairment and hypoxemia.

The Case: A 70-year-old man came to the emergency department with a complaint of dysphagia of acute onset. He reported a similar episode a week earlier that had resolved without treatment. His history included mild cognitive impairment, a remote-left-hip arthroplasty, hip and knee osteoarthritis,