Canadian respiratory journal : journal of the Canadian Thoracic Society
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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.
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Despite evidence supporting the role of noninvasive ventilation (NIV) in diverse populations, few publications describe how NIV is used in clinical practice. ⋯ The present study illustrates how NIV is used in clinical practice and confirms that NIV initiation has increased over time.
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Lemierre's syndrome is a rare clinical syndrome defined as orophyngeal sepsis, thrombophlebitis of the internal jugular vein and septic thombo-emboli. It is typically encountered in young, immunocompetent individuals, with a mean incident age of 20 years. ⋯ The defined treatment course is at least six weeks of antibiotics, with the role of anticoagulation being unclear. The present article documents a case of Lemierre's syndrome complicated by acute renal failure and loculated pleural effusion in an otherwise healthy 16-year-old patient.
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Pulmonary vein stenosis is a rare cause of pulmonary hypertension, with variable onset and presentation. One or more of the four pulmonary veins can be primarily or secondarily affected. A five-month-old girl presented with respiratory distress, lethargy and cyanosis requiring intubation. ⋯ Pulmonary vein stenosis is a rare cause of pulmonary hypertension, and is associated with significant morbidity and mortality. Surgical intervention may be of benefit in selected cases. It stands to reason that any treatment will have the best chance of success if completed before the pulmonary hypertension becomes fixed. Early diagnosis depends on a high index of clinical suspicion.
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A key mandate of the Canadian Thoracic Society (CTS) is to promote evidence-based respiratory care through clinical practice guidelines (CPGs). To improve the quality and validity of the production, dissemination and implementation of its CPGs, the CTS has revised its guideline process and has created the Canadian Respiratory Guidelines Committee to oversee this process. ⋯ The CTS has also committed to develop guidelines in new areas, an annual guideline review cycle, and a new formal process for dissemination and implementation. Ultimately, it is anticipated that these changes will have a significant impact on the quality of care and clinical outcomes of individuals suffering from respiratory diseases across Canada.