Immunoglobulin (Ig) G4-related disease (also known as &#x2018;IgG4-related sclerosing disease&#x2019;, &#x2018;IgG4-related systemic disease&#x2019; or &#x2018;hyper-IgG4-disease&#x2019;) is a recently recognized systemic fibroinflammatory disease associated with IgG4-positive plasma cells in tissue lesions. IgG4-related disease was initially described as autoimmune pancreatitis, but it is now known to affect virtually any organ. The authors describe a patient presenting with multi-organ manifestations, including airway inflammation mimicking asthma, pulmonary parenchymal infiltrates, intrathoracic lymphadenopathy, submandibular gland swelling and a kidney mass.

Hiroshi Sekiguchi

Ryohei Horie

Timothy R Aksamit

Eunhee S Yi

Jay H Ryu

Immunoglobulin G4-Related Disease Mimicking Asthma

Jacob Gelberg

David Ryan Stather

Pulmonary Manifestations of Inflammatory Bowel Disease

Emilia &#x15a;wietlik

Anna Doboszy&#x144;ska

Dr Richard Vincent Hodder

Peter Par&#xe9;

Jules Arthur Peter Par&#xe9;

BACKGROUND: Almost all patients with Duchenne muscular dystrophy (DMD) eventually develop respiratory failure. Once 24 h ventilation is required, either due to incomplete effectiveness of nocturnal noninvasive ventilation (NIV) or bulbar weakness, it is common practice to recommend invasive tracheostomy ventilation; however, noninvasive daytime mouthpiece ventilation (MPV) as an addition to nocturnal mask ventilation is also an alternative.METHODS: The authors&#x2019; experience with 12 DMD patients who used 24 h NIV with mask NIV at night and MPV during daytime hours is reported.RESULTS: The mean (&#xb1; SD) age and vital capacity (VC) at initiation of nocturnal (only) NIV subjects were 17.8&#xb1;3.5 years and 0.90&#xb1;0.40 L (21&#x25; predicted), respectively; and, at the time of MPV, 19.8&#xb1;3.4 years and 0.57 L (13.2&#x25; predicted), respectively. In clinical practice, carbon dioxide (CO2) levels were measured using different methods: arterial blood gas analysis, transcutaneous partial pressure of CO2 and, predominantly, by end-tidal CO2. While the results suggested improved CO2 levels, these were not frequently confirmed by arterial blood gas measurement. The mean survival on 24 h NIV has been 5.7 years (range 0.17 to 12 years). Of the 12 patients, two deaths occurred after 3.75 and four years, respectively, on MPV; the remaining patients continue on 24 h NIV (range two months to 12 years; mean 5.3 years; median 3.5 years).CONCLUSIONS: Twenty-four hour NIV should be considered a safe alternative for patients with DMD because its use may obviate the need for tracheostomy in patients with chronic respiratory failure requiring more than nocturnal ventilation alone.

Douglas A McKim

Nadia Griller

Carole LeBlanc

Andrew Woolnough

Judy King

Twenty-Four Hour Noninvasive Ventilation in Duchenne Muscular Dystrophy: A Safe Alternative to Tracheostomy

BACKGROUND: Menthol (l-menthol) is a naturally-occurring cold receptor agonist commonly used to provide symptomatic relief for upper airway congestion. Menthol can also reduce the sensation of dyspnea. It is unclear whether the physiological action of menthol in dyspnea reduction is through its cold receptor agonist effect or whether associated mechanical changes occur in the upper airway.OBJECTIVE: To determine whether menthol inhalation alters upper airway resistance in humans.METHODS: A randomized, sham-controlled, single-blinded crossover study of inhaled menthol on upper airway resistance during semirecumbent quiet breathing in healthy subjects was conducted. Ten healthy participants (eight female) with a mean (&#xb1; SD) age of 21&#xb1;1.6 years completed the study.RESULTS: Nasal resistance before testing was similar on both occasions. No differences were found in respiratory frequency (mean &#xb1; SEM) (menthol 17.0&#xb1;1.1 cmH2O/L/s; sham 16.9&#xb1;0.9 cmH2O/L/s), minute ventilation (menthol 7.7&#xb1;0.5 cmH2O/L/s; sham 7.9&#xb1;0.5 cmH2O/L/s) or total inspiratory time/total breath time (menthol 0.4&#xb1;0.1 cmH2O/L/s; sham 0.4&#xb1;0.1 cmH2O/L/s). The upper airway resistance was similar during menthol (3.47&#xb1;0.32 cmH2O/L/s) and sham (3.27&#xb1;0.28 cmH2O/L/s) (P=0.33) inhalation.CONCLUSION: Inhalation of menthol does not alter upper airway resistance in awake human subjects.

Effie J Pereira

Lauren Sim

Helen S Driver

Chris M Parker

Michael F Fitzpatrick

The effect of inhaled menthol on upper airway resistance in humans: A randomized controlled crossover study

Diffuse pulmonary lymphangiomatosis (DPL) is a rare disease characterized by infiltration of the lung, pleura and mediastinum with thin-walled lymphangiomas. DPL can result in mass effect from infiltrative disease, restrictive and obstructive pulmonary physiology, chylous effusions and respiratory failure. The present article discusses clinical, radiographic and pathological features, and treatment options for DPL.

Kunal C Kadakia

Sandeep M Patel

Andrew H Limper

Diffuse Pulmonary Lymphangiomatosis

Many common diseases affecting the airways are characterized by airway inflammation. The measurement of this inflammation has a significant role in the management of these diseases. Quantitative sputum cell counts provide a measurement of the type and severity of inflammation present. Sputum cell counts are used in routine clinical practice in some centres but their use is not widespread. The present study used a standardized questionnaire to determine both patients&#x2019; and physicians&#x2019; attitudes toward the use of sputum cell counts. The use of sputum cell counts was well accepted by patients and physicians. Ninety per cent of patients were satisfied with the test. Sixty per cent of family physicians were satisfied with the test and 80&#x25; were in favour of it being funded by the government. The authors recommend more widespread use of sputum cell counts to guide the management of airway diseases.

Liesel D&#x2019;silva

Helen Neighbour

Amiram Gafni

Katherine Radford

Frederick E Hargreave

Parameswaran Nair

Quantitative Sputum Cell Counts to Monitor Bronchitis: A Qualitative Study of Physician and Patient Perspectives

BACKGROUND: Loud snoring is a common symptom in the general population. The evidence-based literature indicates that snoring may be associated with sleep fragmentation and sleep apnea, which may affect cognitive function and predispose to occupational injury. High rates of occupational injury occur on farms and may be related to personal and health factors. Thus, loud snoring may not be a trivial symptom and should be considered as important in medical assessments.METHODS: A prospective cohort study was conducted in Saskatchewan. Baseline questionnaires were completed for 5502 individuals by representatives from 2390 farms. Sleep patterns at baseline were categorized as the following: no reported sleep disorders; physician-diagnosed sleep apnea (treatment unknown); and loud snoring. Survival analyses were used to relate sleep patterns with subsequent injury.RESULTS: A total of 6.7&#x25; (369 of 5502) of participants reported a possible sleep disorder. Of these, 69.4&#x25; (256 of 369) reported loud snoring only. Loud snoring was only associated with a consistent increase in risk (eg, HR 1.45 [95 CI 1.07 to 1.99 for work-related injury]) for five farm injury outcomes. Relationships between physician-diagnosed sleep apnea and time to first injury were not significant, presumably because a diagnosis of sleep apnea implied treatment for sleep apnea.DISCUSSION: Sleep disorders are an important potential risk factor for occupational injury on farms. Substantial proportions of farm residents report loud snoring and this is related to subsequent injury. Some of these cases may represent sleep fragmentation or undiagnosed obstructive sleep apnea. Identification and clinical management of sleep disorders related to snoring should be part of health assessments conducted by physicians.

James A Dosman

Louise Hagel

Robert Skomro

Xiaoqun Sun

Andrew G Day

William Pickett

Loud Snoring is A Risk Factor for Occupational Injury in Farmers

John Granton

The Times They are a Changin&#x2019;

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Canadian Respiratory Journal provides a multidisciplinary forum for research in all areas of respiratory medicine. The journal publishes research related to asthma, allergy, COPD, non-invasive ventilation, therapeutic intervention etc.

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Chief Editor, Dr Alice M Turner, is based at the University of Birmingham, UK. Her main research interests are the clinical aspects of alpha 1 antitrypsin deficiency and chronic obstructive pulmonary disease.

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Canadian Respiratory Journal - Table of contents 2013

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Canadian Respiratory Journal provides a multidisciplinary forum for research in all areas of respiratory medicine. The journal publishes research related to asthma, allergy, COPD, non-invasive ventilation, therapeutic intervention etc.

Canadian Respiratory Journal