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INTRODUCTION: Common variable immunodeficiency (CVID) is a primary immune disorder characterised by impaired B cell differentiation and hypogammaglobulinaemia. It predisposes to encapsulated bacterial infections, commonly affecting the respiratory tract. 8%-20% of CVID patients have a granulomatous form. Granulomatous CVID may share clinical features with sarcoidosis, namely persistent cough, breathlessness, fatigue, fever, and arthralgia. Non caseating granulomata in lymph node biopsies and elevated angiotensin converting enzyme levels occur in both conditions. Differentiation between the two conditions is essential to allow management with immunoglobulin replacement and avoidance of excessive corticosteroids. METHOD: Patients with a diagnosis of \"sarcoidosis\" recorded in chest clinic letters between April 2009 and September 2010 were identified. Clinic letters, blood tests, histology and imaging were reviewed. RESULTS: 175 patients with a diagnosis of sarcoidosis were identified. 80% had lymphadenopathy and 62% pulmonary involvement. Dermatological, ocular, rheumatological and other involvement was found in 19%, 17%, 16% and 9% respectively. 24% had potential features of CVID, such as recurrent chest infections, sinusitis, bronchiectasis or inflammatory bowel disease. Total globulin had been measured in 172/175 (98%) patients. 45/172 (26%) had elevated total globulin; 3/172 (2%)had low total globulin levels. Immunoglobulins had been measured in 106/175 (61%). Immunoglobulins had been measured in: 20/28 (71%) of those with recurrent sinusitis or lower respiratory tract infections; 20/25 (80%) of those with bronchiectasis; and8/8 (100%) of those with both recurrent chest infections and bronchiectasis. Four patients had persistently low IgM, two had persistently low IgA and one had persistently low IgG. No single patient had polyclonal hypogammaglobulinaemia, but levels were not measured in 11/42 (26%) with potential features of CVID. CONCLUSION: Our study identified a cohort of patients with a diagnosis of sarcoidosis and features associated with CVID. We found that immunoglobulins were not being routinely measured during the work-up of patients with sarcoidosis as recommended by the Map of Medicine. Recent review of 28 local CVID patients identified two who were initially misdiagnosed with sarcoidosis. In one case this misdiagnosis persisted for 8 years. Granulomatous CVID is uncommon, but respiratory physicians should ensure that their routine work-up for sarcoidosis excludes this treatable condition.

IntroductionThere is a national target to recruit 100,000 patients with COVID-19 to the PRIEST study. Use of a research proforma to collect the required data risked Emergency Department (ED) physicians collecting this dataset at the expense of their more usual clinical history. Use of paper admission forms which could not be taken from COVID-19 ‘dirty’ to ‘clean’ areas either resulted in work duplication to transcribe information written in the ‘dirty’ zone or potential recall error/omission when staff completed paperwork later in the ‘clean’ zone.MethodsThe solution (in a hospital which still uses paper notes) was an electronic admissions document, completed in the dirty zone and printed out in the clean zone. The use of Visual Basic (VB) allowed insertion of scripts to automate data extraction for research and quality improvement use. Microsoft Word was preferred because of staff familiarity, combined with better data governance than cloud solutions such as Google Forms and better legibility/formatting than research data collection tools such as REDCap.ResultsVB enhanced Word documents offer similar benefits for management of patients in COVID-19 treatment areas and for subsequent follow up. The electronic forms were received well by staff, as they allowed for clerking and PRIEST to happen simultaneously. Clinical staff remarked that the form also acted as a prompt when clerking, which reduced the time taken to carry out subsequent patient encounters. Research teams found the form much easier to process, as it negated the need to quarantine notes prior to assessment and allowed for data to be extracted immediately into an Excel spreadsheet.ConclusionThe use of VB not only increased staff safety, but it also allowed researchers to contemporaneously process data. Whilst a mixed paper and electronic notes system is less than perfect, the adjunct of the virtual form was well received during the COVID-19 pandemic. Future expansion of VB use as an adjunct to medical clerking will be considered and has already been piloted in the collection of data for COVID-19 patients admitted to our hospital.

IntroductionRecurrent pulmonary exacerbations lead to progressive lung damage in cystic fibrosis (CF). Inhaled medications (mucoactive agents and antibiotics) help prevent exacerbations, but objectively measured adherence is low. We investigated whether a multi-component (complex) self-management intervention to support adherence would reduce exacerbation rates over 12 months.MethodsBetween October 2017 and May 2018, adults with CF (aged ≥16 years; 19 UK centres) were randomised to the intervention (data-logging nebulisers, a digital platform and behavioural change sessions with trained clinical interventionists) or usual care (data-logging nebulisers). Outcomes included pulmonary exacerbations (primary outcome), objectively measured adherence, body mass index (BMI), lung function (FEV1) and Cystic Fibrosis Questionnaire-Revised (CFQ-R). Analyses were by intent to treat over 12 months.ResultsAmong intervention (n=304) and usual care (n=303) participants (51% female, median age 31 years), 88% completed 12-month follow-up. Mean exacerbation rate was 1.63/year with intervention and 1.77/year with usual care (adjusted ratio 0.96; 95% CI 0.83 to 1.12; p=0.64). Adjusted mean differences (95% CI) were in favour of the intervention versus usual care for objectively measured adherence (9.5% (8.6% to 10.4%)) and BMI (0.3 (0.1 to 0.6) kg/m2), with no difference for %FEV1 (1.4 (−0.2 to 3.0)). Seven CFQ-R subscales showed no between-group difference, but treatment burden reduced for the intervention (3.9 (1.2 to 6.7) points). No intervention-related serious adverse events occurred.ConclusionsWhile pulmonary exacerbations and FEV1 did not show statistically significant differences, the intervention achieved higher objectively measured adherence versus usual care. The adherence difference might be inadequate to influence exacerbations, though higher BMI and lower perceived CF treatment burden were observed.

JAMA 2005 ; 293 : 581 -8 This longitudinal study correlates acquisition of non-mucoid and mucoid phenotypes of Pseudomonas aeruginosa with respiratory symptoms, quantitative chest radiography, pulmonary function tests, and anti-pseudomonal antibodies in cystic fibrosis (CF).

La première exploration minéralogique organisée par les Européens, dans ce qui est aujourd'hui le Canada, a été menée par Martin FROBISHER au cours de ses trois expéditions à l'île de Baffin (1576, 1577 et 1578).

The first organized mineral exploration by Europeans in what is now Canada was led by Martin FROBISHER in his 3 expeditions to Baffin Island (1576, 1577 and 1578).

According to a study conducted by Maron et al. Clinical presentation of HCM exacerbation in the ED may have a diverse array of non-specific symptoms such as syncope, hypotension, chest pain, shortness of breath, arrhythmias, heart failure and in worst cases, sudden cardiac death. Conversely, agents which preserve or augment afterload will help to decrease the degree of outflow obstruction. [...]pure alpha-agonists are the preferred vasopressors in patients with HCM and outflow obstruction who experience decompensation [11,19]. Disopryramide should not be administered in the absence of beta blockers or CCB among HCM patients with atrial fibrillation due to its propensity to increase AV nodal conduction and, therefore, ventricular rate [20].4 Conclusion In the busy community hospital ED settings, the emergency physician must act quickly to intervene and rapidly manage acutely decompensating HCM patients.

Gangliosides occur in much greater amounts in clean isolated neurons and in the neuropil teased from immediately around the neuron cell body and dendrites than in isolated clumps of glial cells. Since the zone of neuropil adjacent to neurons is richest in terminal axons and synaptic endings, these findings indicate a specific concentration of these sialoglycolipids in synaptic membranes.