1 40 1 Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource January 2018 List Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. Citation List Month January 2018 List URL Address <a href="http://doi.org/10.1016/j.ejpn.2017.04.1310" target="_blank" rel="noreferrer">http://doi.org/10.1016/j.ejpn.2017.04.1310</a> Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Palliative care in children with spinal muscular atrophy type 1: How do they die? Results from a French multicentric study (National Hospital clinical Research Program) Publisher An entity responsible for making the resource available European Journal Of Paediatric Neurology Date A point or period of time associated with an event in the lifecycle of the resource 2017 Subject The topic of the resource clinical research; palliative therapy; Werdnig Hoffmann disease; 50-48-6 (amitriptyline); 52-26-6 (morphine); 57-27-2 (morphine); 549-18-8 (amitriptyline); Amitriptyline; benzodiazepine derivative; Child; Clinical Article; clinical practice; Diagnosis; Drug Therapy; Female; follow up; Human; infant; Intensive care unit; Interview; Male; Morphine; multicenter study; nasogastric tube; Noninvasive Ventilation; psychologist; quantitative analysis; Resuscitation; time of death Creator An entity primarily responsible for making the resource Hully M; Barnerias C; Vanesse S; Viallard ML; Desguerre I Description An account of the resource Objective: The national Hospital Clinical Research Program (PHRC) called Assessment of clinical practices of palliative care in children with Spinal Muscular Atrophy Type 1 (SMA 1) was conducted to depict palliative practices in that fatal disease, in which death up to now occurs few weeks or months after the diagnosis. We here report data about the conditions of death for the patients included. Methods: In this French multicentric study, patients were included from june 2012 to june 2016. Parents and physicians filled in a specific health book during the follow up, the physician in charge filled in a survey concerning the patient's management over the last 48 hours before death, then a semidirected interview of the parents was conducted by a trained psychologist 6 to 18 months after the child's death. We here report the quantitative analysis of data obtained from the survey about patient's management around death. Results: 38 patients were included in the study (17 centres), data were available for 36 dead patients. Median age at inclusion was 3 months (0,6-10,4), death occurred at a median 5,5 month of age (1,5-16,4), i.e a median follow-up of 2 months (0,2-12,8). 39% of patients died at home, 6% in an intensive care unit. At the time of death, patients received morphine (56%), benzodiazepines (39%), amitriptyline (39%). Treatments were given through a nasogastric tube (83%), and oxygenotherapy was delivered (76%). 6% patients received noninvasive ventilation at the time of death. No resuscitation recommendations had been prepared for most patients (97%), written in 85%, after a multidisciplinary meeting in most cases (79%). Conclusion: Our data confirm current knowledge about natural outcome in SMA 1, death occurring very soon after the diagnosis, claiming for an effective palliative management of the patients, including the involvement of parents in medical care at home. Identifier An unambiguous reference to the resource within a given context <a href="http://doi.org/10.1016/j.ejpn.2017.04.1310" target="_blank" rel="noreferrer">10.1016/j.ejpn.2017.04.1310</a> Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). 2017 50-48-6 (amitriptyline) 52-26-6 (morphine) 549-18-8 (amitriptyline) 57-27-2 (morphine) Amitriptyline Barnerias C benzodiazepine derivative Child Clinical Article Clinical Practice Clinical Research Desguerre I Diagnosis Drug Therapy European Journal of Paediatric Neurology Female Follow Up Hully M Human Infant Intensive Care Unit Interview January 2018 List Male Morphine Multicenter Study nasogastric tube Noninvasive Ventilation Palliative Therapy Psychologist quantitative analysis Resuscitation time of death Vanesse S Viallard ML Werdnig Hoffmann disease