Articles: patients.
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WHAT IS ALREADY KNOWN ON THIS TOPIC: Application of continuous positive airway pressure (CPAP) for patients with acute respiratory distress reduces the need for tracheal intubation and mechanical ventilation. Case series have demonstrated CPAP's feasibility in out-of-hospital settings. ⋯ WHAT THIS STUDY ADDS TO OUR KNOWLEDGE: Within the CPAP group, the rate of intubation was 30% less than the usual care group, and mortality was 20% lower. HOW THIS MIGHT CHANGE CLINICAL PRACTICE: Emergency medical services systems and their medical directors should consider making CPAP available as part of the treatment for out-of-hospital severe respiratory distress patients, particularly in systems with long transport times.
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Delirium is a complex neuropsychiatric syndrome presenting primarily with disturbances of cognition, perception and sensorium, alertness, sleep/wake cycle, and psychomotor behavior in the context of a medical etiology. The presentation can be quite variable among patients and even within a given patient because of its waxing and waning course. This variability and overlap with other psychiatric syndromes has led to substantial underrecognition and undertreatment in clinical settings. ⋯ Similarly, research is warranted that focuses on preventing delirium, potentially by identifying susceptible patients and intervening early. It is particularly challenging to devise cost-effective interventions for preventing and identifying delirium early in its course, given the rapid pace and resource limitations in inpatient and intensive care settings, and current data do not clearly indicate that such systems have proven benefit. Still, the indisputable health and financial costs of delirium indicate that prevention and identification should be a high priority.
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Ornithine transcarbamylase (OTC) deficiency is an X-linked urea cycle defect. While hemizygous males typically present with hyperammonemic coma in infancy, reports of rare late-onset presentations exist, with poor outcomes in males up to 58 years old. Relatives with mutations identical to affected patients often remain asymptomatic, and it is likely that environmental and genetic factors influence disease penetrance and expression. ⋯ This case illustrates that OTC deficiency can cause acute, severe hyperammonemia in a previously healthy adult and that the P225T mutation can be associated with late-onset OTC deficiency. We speculate that exposure to organic chemicals might have contributed to the onset of symptoms in this patient. This case also emphasizes that persistent hyperammonemia may cause irreversible neurologic damage and that after the diagnosis of hyperammonemia is established in an acutely ill patient, certain diagnostic tests should be performed to differentiate between urea cycle disorders and other causes of hyperammonemic encephalopathy.