{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T12:36:53Z","timestamp":1774442213308,"version":"3.50.1"},"reference-count":17,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,10,13]],"date-time":"2017-10-13T00:00:00Z","timestamp":1507852800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2018,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>This study described end\u2010of\u2010life care for children affected by spinal muscular atrophy type 1 (<jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>1), which is characterised by progressive muscle weakness and develops in the first six months of life.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively analysed 17 children (13 boys) who attended the University of Padua's paediatric palliative care centre in Italy from March 2000 to March 2015. All the children received supportive care without proactive respiratory intervention to prolong survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The median age at admission was 3.57 months, and the median age at death was 6.80 months. The most frequent symptoms were dyspnoea and pain. In the last 72 hours of life, 15\/17 children required more intense doses of morphine and, or, benzodiazepines for intractable dyspnoea and pain, but deep palliative sedation was not needed. Airway suction to manage secretions and nasogastric tubes was required in all cases. The place of death was previously planned by the parents in all cases \u2013 home, hospital or hospice \u2013 and 15\/17 deaths occurred in that place. We also interviewed 16 of the 17 parents after their child died.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our study found that symptom management and psychological support for families were the cornerstones of end\u2010of\u2010life care for children with <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>1.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.14086","type":"journal-article","created":{"date-parts":[[2017,9,23]],"date-time":"2017-09-23T15:02:46Z","timestamp":1506178966000},"page":"140-144","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Symptom management and psychological support for families are the cornerstones of end\u2010of\u2010life care for children with spinal muscular atrophy type 1"],"prefix":"10.1111","volume":"107","author":[{"given":"Chiara","family":"Di Pede","sequence":"first","affiliation":[{"name":"Department of Women's and Children's Health Pediatric Pain and Palliative Care Service University of Padua Padua Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caterina","family":"Agosto","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health Pediatric Pain and Palliative Care Service University of Padua Padua Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Valentina","family":"De Tommasi","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health Pediatric Pain and Palliative Care Service University of Padua Padua Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandra","family":"De Gregorio","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health Pediatric Pain and Palliative Care Service University of Padua Padua Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Franca","family":"Benini","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health Pediatric Pain and Palliative Care Service University of Padua Padua Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,10,13]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cppeds.2010.05.001"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mop.0000193266.86129.47"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.289.18.2387"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073807305788"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2012-2278"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/PHM.0b013e31804a8505"},{"key":"e_1_2_1_8_1","unstructured":"Veneto in numbers: statistics pocket guide: year 2007. Available at:http:\/\/www.regione.veneto.it."},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1186\/1824-7288-34-4"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(14)60746-7"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1440-1754.1999.00351.x"},{"key":"e_1_2_1_12_1","first-page":"1","article-title":"Palliative care in children with spinal muscular atrophy type I: what do they need?","volume":"24","author":"Garc\u00edaSalido A","year":"2014","journal-title":"Palliat Support Care"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.2009.178269"},{"key":"e_1_2_1_14_1","unstructured":"World Health Organization.WHO definition of palliative care. Available at:http:\/\/www.who.int\/cancer\/palliative\/definition\/en\/(accessed on March 15 2008)."},{"key":"e_1_2_1_15_1","first-page":"2","article-title":"IMPaCCT: standards for paediatric palliative care in Europe","volume":"14","author":"European Association of Palliative Care (EAPC) Taskforce","year":"2007","journal-title":"Eur J Pall Care"},{"key":"e_1_2_1_16_1","volume-title":"When children die: improving palliative and end\u2010of\u2010life care for children and their families","author":"Field MJ","year":"2003"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1177\/0269216308096527"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpainsymman.2007.12.017"}],"container-title":["Acta Paediatrica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fapa.14086","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/apa.14086","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T22:58:13Z","timestamp":1696028293000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/apa.14086"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,10,13]]},"references-count":17,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2018,1]]}},"alternative-id":["10.1111\/apa.14086"],"URL":"https:\/\/doi.org\/10.1111\/apa.14086","archive":["Portico"],"relation":{},"ISSN":["0803-5253","1651-2227"],"issn-type":[{"value":"0803-5253","type":"print"},{"value":"1651-2227","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,10,13]]},"assertion":[{"value":"2017-03-03","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2017-09-18","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2017-10-13","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}