{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T05:31:37Z","timestamp":1787031097954,"version":"build-2736575974"},"reference-count":15,"publisher":"SAGE Publications","issue":"2","license":[{"start":{"date-parts":[[2023,1,3]],"date-time":"2023-01-03T00:00:00Z","timestamp":1672704000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Respir Care"],"published-print":{"date-parts":[[2023,2]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Spinal muscular atrophy (SMA) is a neurodegenerative disease that results in progressive muscular atrophy and weakness. The primary cause of morbidity and mortality in these children is pulmonary disease due to poor airway clearance that leads to acute respiratory failure. There is a paucity of literature on the treatment of children with SMA and acute respiratory failure.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>We performed a retrospective chart review of pediatric patients with SMA type I or II admitted to the PICU in a tertiary-care children's hospital with acute respiratory failure who required mechanical ventilation and\/or aggressive airway clearance.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      The study included 300 unique encounters among 137 unique subjects. All the subjects received airway clearance at home before admission, and 257 encounters (85.7%) were supported with noninvasive ventilation (NIV) before admission. Sixty-eight subjects (49.6%) required endotracheal intubation on admission or at some point during their PICU stay. The median (interquartile range [IQR]) time to intubation was 0 (0\u201320) h, and the median (IQR) duration of mechanical ventilation was 2 (1\u20135) d on invasive mechanical ventilation with no statistical difference between type I and type II (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      = .89). Of those, 65 (91.1%) were successfully extubated to NIV on the first attempt, whereas 4 subjects (5.8%) required multiple extubation attempts and 3 subjects (4.4%) required subsequent tracheostomy. For the subjects who were intubated, both PICU and hospital length of stay were longer (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt; .001) when compared with the subjects managed by NIV alone. The subjects with SMA type I had a longer PICU length of stay, with a median (IQR) of 5 (3\u201311) d versus 4 (2\u20137) d (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      = .002). The hospital length of stay and duration of invasive mechanical ventilation were not statistically different (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      = .055 and\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      = .068, respectively).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>The subjects with SMA types I or II can be treated successfully with NIV and aggressive airway clearance during acute respiratory failure. Similarly, when intubation is required, successful extubation can be achieved with NIV transitional support combined with aggressive airway clearance maneuvers.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4187\/respcare.10353","type":"journal-article","created":{"date-parts":[[2023,1,3]],"date-time":"2023-01-03T11:55:58Z","timestamp":1672746958000},"page":"247-254","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":8,"title":["Management of Critically Ill Patients With Spinal Muscular Atrophy Admitted With Acute Respiratory Failure"],"prefix":"10.1177","volume":"68","author":[{"given":"Awni M","family":"Al-Subu","sequence":"first","affiliation":[{"name":"Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolyn K","family":"Adams","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Care, American Family Children's Hospital, The University of Wisconsin Hospitals and Clinics, Madison, Wisconsin."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scarlett A","family":"Dykstra","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Care, American Family Children's Hospital, The University of Wisconsin Hospitals and Clinics, Madison, Wisconsin."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miranda R","family":"Langkamp","sequence":"additional","affiliation":[{"name":"Pediatric Respiratory Care, American Family Children's Hospital, The University of Wisconsin Hospitals and Clinics, Madison, Wisconsin."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rhonda A","family":"Yngsdal-Krenz","sequence":"additional","affiliation":[{"name":"Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rand M","family":"Al Subu","sequence":"additional","affiliation":[{"name":"Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhumin","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Biostatistics and Medical Informatics, University of Wisconsin, Madison, Wisconsin."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kimberly N","family":"Baker","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiac Critical Care Medicine, The Heart Center at Nemours Children\u2019s Hospital, Nemours Children\u2019s Hospital, Orlando, Florida."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas B","family":"Brazelton","sequence":"additional","affiliation":[{"name":"Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2023,1,3]]},"reference":[{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.spen.2021.100878"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2020.593282"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2017.11.005"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1177\/0883073807305788"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000000741"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.phm.0000184097.17189.93"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2008-2952K"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2017.11.004"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2008-2952F"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1097\/PCC.0b013e3182388ad1"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1097\/PHM.0b013e31804a8505"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.117.4.1100"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.10110"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2021.10296"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.25899"}],"container-title":["Respiratory 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