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.
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.
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.
Using registry data to improve quality of care McIntyre, Kieran; Bertrand, Dominique Pougheon; Rault, Gilles
Journal of cystic fibrosis,
September 2018, 2018-Sep, 2018-09-00, 20180901, Volume:
17, Issue:
5
Journal Article
Peer reviewed
Open access
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.
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.
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.
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.
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.
•In adult CF patients with severe ABPA or asthma, the safety and efficacy of omalizumab are not known.•Omalizumab was well tolerated and no significant drug-related adverse effects were observed.•In CF-asthma, the max FEV1pp improved on omalizumab and the cumulative SCS dose decreased.•In ABPA, the rate of FEV1pp decline (slope) and the variability of FEV1pp improved on omalizumab.
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™) 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.
An adult patient with new-onset dysphagia McIntyre, Kieran; McVaugh-Smock, Simon; Mourad, Ophyr
CMAJ. Canadian Medical Association journal,
2006-Nov-07, 2006-11-07, 20061107, Volume:
175, Issue:
10
Journal Article
Peer reviewed
Open access
The thymus is probably involved in the pathogenesis of MG;1 thymic tumours should therefore be excluded. Between 30% and 60% of patients with epithelial thymic tumours (thymoma) have MG and, in turn, ...some 10%-15% of people with MG have thymoma. Their relationship has yet to be fully elucidated, although the initial steps in the pathogenesis of MG in most cases take place within abnormal thymic microenvironments, whether inflammatory or neoplastic. In about 90% of patients, no cause can be identified. The classical presentation includes a myasthenic "snarl" and a voice that sounds nasal because of bulbar and facial muscle weakness (Box 2; Table l). Dysphagia is the only manifestation of MG that has been well documented:2 about 20% of cases involve the bulbar muscles. MG can be diagnosed by means of serology, nerve conduction studies with SFEMG, or the classic Tensilon test. Since about 85% of patients with generalized disease will have antibodies to the acetylcholine receptor, a positive result is diagnostic for MG; unfortunately, this test is not routinely available. This case demonstrates the importance of considering neurotransmitter diseases in the approach to new-onset focal weakness that involves the bulbar musculature. MG was diagnosed in our patient, who presented purely with swallowing abnormalities, via videofluorography during a Tensilon test.3 This novel diagnostic approach should be considered for patients with suspected bulbar MG.
Drug-related hepatotoxicity McDonnell, Marie E; Braverman, Lewis E
The New England journal of medicine,
2006-May-18, Volume:
354, Issue:
20
Journal Article
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