Therapy of patients with chronic respiratory failure is mainly directed at minimizing symptoms in order to improve, or at least to prevent a deterioration of, patients' well-being. Under such circumstances, the perceived effect of therapies on patients' well-being and daily life represents the most important subjective outcome of treatment. Therefore, there is a need to provide a global estimate of health in patients on long term oxygen therapy or overnight home mechanical ventilation. The Maugeri Foundation Respiratory Failure Questionnaire (MRF28) is the first health status ("quality of life") questionnaire specifically developed for use in CRF and its items were selected to be applicable to patients with both obstructive and restrictive diseases. The Quality of Life Evaluation and Survival Study (QuESS) is a multinational study with the aim of re-evaluating the natural history of chronic respiratory failure in about 300 patients. To the authors knowledge, the Quality of Life Evaluation and Survival Study is the first study to evaluate the natural history of chronic respiratory failure in such a large number of subjects and with a complete set of data. In fact, both pathophysiologic and health status assessments will be made. Moreover, by collecting data on mortality, disease exacerbations and hospitalization, it will also be possible to verify the predictive ability of health status versus pathophysiology in terms of mortality and healthcare utilization.
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Hong Kong Med J
January 2025
Division of Nephrology, Department of Medicine, School of Clinical Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong SAR, China.
J Med Genet
January 2025
Division of Clinical and Metabolic Genetics, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Biallelic pathogenic variants in cause a fatal autosomal recessive multisystem disorder characterized by recurrent autoinflammation, hypomyelination, progressive neurodegeneration, microcephaly, failure to thrive, liver dysfunction, respiratory chain defects and accumulation of glycogen in skeletal muscle. No missense variants in have been reported to date.We report a 6-year-old boy with microcephaly, global developmental delays, lower limb spasticity with hyperreflexia, epilepsy, abnormal brain MRI, failure to thrive, recurrent fevers and transaminitis.
View Article and Find Full Text PDFResuscitation
January 2025
Division of Neurology, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA.
Aims: To determine which patient and cardiac arrest factors were associated with obtaining neuroimaging after in-hospital cardiac arrest, and among those patients who had neuroimaging, factors associated with which neuroimaging modality was obtained.
Methods: Retrospective cohort study of patients who survived in-hospital cardiac arrest (IHCA) and were enrolled in the ICU-RESUS trial (NCT02837497).
Results: We tabulated ultrasound (US), CT, and MRI frequency within 7 days following IHCA and identified patient and cardiac arrest factors associated with neuroimaging modalities utilized.
Heart Lung
January 2025
College of Nursing, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia; King Abdullah International Medical Research Center, Riyadh, Saudi Arabia; Ministry of the National Guard Health Affairs Riyadh, Saudi Arabia; Medical-Surgical Nursing Department, Faculty of Nursing, Cairo University, Cairo, Egypt.
Background: Endotracheal intubation and mechanical ventilation comprise common life support interventions for patients in intensive care units (ICUs). Premature or delayed extubation increases the risk of morbidity and mortality. Despite following weaning protocols, 10-20 % of patients fail extubation within 48 h.
View Article and Find Full Text PDFAm J Emerg Med
January 2025
Departments of Emergency Medicine and Critical Care Medicine, Stanford Health Care, 900 Welch Road, Palo Alto, CA 94304, USA.
Background: Critically ill ED patients on life support may undergo transition to comfort care as decided by the surrogate decision maker. When several hours are needed for loved ones to arrive and say farewell before initiating comfort care ("delayed comfort care"), these patients require prolonged ED stays or costly intensive care unit (ICU) admissions.
Methods: A novel ED observation unit (EDOU)-based delayed comfort care pathway for ED patients on invasive mechanical ventilation and/or vasopressors was created in 2013 at Stanford Hospital.
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