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Non-invasive ventilation (NIV) is an evidence based management of acidotic, hypercapnic exacerbations of COPD. Previous national and international audits of clinical practice have shown variation against guideline standards with significant delays in initiating NIV.

We aimed to map the clinical pathway to better understand delays and reduce the door-to-NIV time to less than 3 hours for all patients with acidotic, hypercapnic exacerbations of COPD requiring this intervention, by mandating the use of a guideline based educational management proforma.

The proforma was introduced at 7 acute hospitals in North London and Essex and initiated at admission of the patient. It was used to record the clinical pathway and patient outcomes until the point of discharge or death. Data for 138 patients were collected. 48% of patients commenced NIV within 3 hours with no reduction in door-to-mask time during the study period. Delays in starting NIV were due to: time taken for review by the medical team (101 minutes) and time taken for NIV to be started once a decision had been made (49 minutes). There were significant differences in door-to-NIV decision and mask times between differing respiratory on-call systems, p < 0.05).

The introduction of the proforma had no effect on door-to-mask times over the study period. Main reasons for delay were related to timely access to medical staff and to NIV equipment; however, a marked variation in practice within these hospitals was been noted, with a 9-5 respiratory on-call system associated with shorter NIV initiation times.

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OBJECTIVE: To review the work of one tertiary paediatric palliative care service in facilitating planned withdrawal of ventilatory support outside the intensive care setting, with the purpose of developing local guidance for practice.

METHODS: Retrospective 10-year (2003-2012) case note review of intensive care patients whose parents elected to withdraw ventilation in another setting. Demographic and clinical data revealed common themes and specific incidents relevant to local guideline development.

RESULTS: 18 children (aged 2 weeks to 16 years) were considered. Three died prior to transfer. Transfer locations included home (5), hospice (8) and other (2). Primary pathologies included malignant, neurological, renal and respiratory diseases. Collaborative working was evidenced in the review including multidisciplinary team meetings with the palliative care team prior to discharge. Planning included development of symptom management plans and emergency care plans in the event of longer than anticipated survival. Transfer of children and management of extubations demonstrated the benefits of planning and recognition that unexpected events occur despite detailed planning. We identified the need for local written guidance supporting healthcare professionals planning and undertaking extubation outside the intensive care setting, addressing the following phases: (i) introduction of withdrawal, (ii) preparation pretransfer, (iii) extubation, (iv) care postextubation and (v) care postdeath.

CONCLUSIONS: Planned withdrawal of ventilatory support outside the intensive care setting is challenging and resource intensive. The development of local collaborations and guidance can enable parents of children dependent on intensive care to consider a preferred place of death for their child, which may be outside the intensive care unit.

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Aspiration is a common but underrecognized clinicopathologic entity, with varied radiographic manifestations. Aspiration represents a spectrum of diseases, including diffuse aspiration bronchiolitis, aspiration pneumonitis, airway obstruction by foreign body, exogenous lipoid pneumonia, interstitial fibrosis, and aspiration pneumonia with or without lung abscess formation.

Many patients who aspirate do not present with disease, suggesting that pathophysiology is related to a variety of factors, including decreased levels of consciousness, dysphagia, impaired mucociliary clearance, composition of aspirate, and impaired host defenses. In this pictorial essay, we will review the different types of aspiration lung diseases, focusing on their imaging features and differential diagnosis.

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Neuromuscular diseases represent a heterogeneous group of disorders of the muscle, nerve or neuromuscular junction. The respiratory muscles are rarely spared in neuromuscular diseases even if the type of muscle involvement, severity and time course greatly varies among the different diseases. Diagnosis of respiratory muscle weakness is crucial because of the importance of respiratory morbidity and mortality.

Presently, routine respiratory evaluation is based on non-invasive volitional tests, such as the measurement of lung volumes, spirometry and the maximal static pressures, which may be difficult or impossible to obtain in some young children.

Other tools or parameters are thus needed to assess the respiratory muscle weakness and its consequences in young children. The measurement of oesogastric pressures can be helpful as they allow the diagnosis and quantification of paradoxical breathing, as well as the assessment of the strength of the inspiratory and expiratory muscles by means of the oesophageal pressure during a maximal sniff and of the gastric pressure during a maximal cough. Sleep assessment should also be part of the respiratory evaluation of children with neuromuscular disease with at least the recording of nocturnal gas exchange if polysomnography is not possible or unavailable.

This improvement in the assessment of respiratory muscle performance may increase our understanding of the respiratory pathophysiology of the different neuromuscular diseases, improve patient care, and guide research and innovative therapies by identifying and validating respiratory parameters.

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