{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T17:52:47Z","timestamp":1787593967164,"version":"build-2736575974"},"reference-count":32,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2022,3,28]],"date-time":"2022-03-28T00:00:00Z","timestamp":1648425600000},"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":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>The recent development of disease\u2010modifying treatments in spinal muscular atrophy (SMA) type 1 shifted these patients' management from palliative to proactive. The aim of this study was to assess patients' nocturnal gas exchanges before noninvasive ventilation (NIV) initiation and their clinical evolution to determine if capnia is a good criterion to decide when to introduce respiratory support.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Patients and Methods<\/jats:title>\n                    <jats:p>This multicentric retrospective study reports the respiratory management and evolution of 17 SMA type 1 children (10 females) for whom treatment with Nusinersen was initiated between 2016 and 2018.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Median [interquartile range\u2014IQR] age at diagnosis and at first Nusinersen injection was of 4 [3;8] and 4 [3;9] months, respectively. Patients were followed during 38 [24;44] months. Thirteen (76%) patients were started on NIV at a median [IQR] age of 12 [9;18] months. Repeated hospitalizations and intensive care unit admissions were needed for 11 of them. Blood gas and nocturnal gas exchange recordings performed before NIV initiation were always normal. 9\/13 X\u2010ray performed before NIV showed atelectasis and\/or acute lower respiratory tract infections. There was a significant decrease in the total number of hospital admissions between the first and second year of treatment (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.04).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This study shows that patients do not present with nocturnal hypoventilation before respiratory decompensations and NIV initiation, and suggests that a delay in NIV initiation might result in respiratory complications. There is a need for disease\u2010centered guidelines for the respiratory management of these patients, including NIV indications.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.25899","type":"journal-article","created":{"date-parts":[[2022,3,20]],"date-time":"2022-03-20T22:43:51Z","timestamp":1647816231000},"page":"1505-1512","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Respiratory management of spinal muscular atrophy type 1 patients treated with Nusinersen"],"prefix":"10.1002","volume":"57","author":[{"given":"Joris","family":"Menard","sequence":"first","affiliation":[{"name":"Department of Pediatric Pulmonology Armand Trousseau University Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreea M","family":"Seferian","sequence":"additional","affiliation":[{"name":"I\u2010Motion H\u00f4pital Armand Trousseau Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Fleurence","sequence":"additional","affiliation":[{"name":"ESEAN\u2010APF Health Center for Children and Adolescents Nantes France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Audrey","family":"Barzic","sequence":"additional","affiliation":[{"name":"Department of Pediatric Fondation Ildys Brest France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Binoche","sequence":"additional","affiliation":[{"name":"Pediatric Intensive Care Unit Department Centre Hospitalier Universitaire de Lille Lille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00e9raldine","family":"Labouret","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology and Allergology Children's Hospital Toulouse 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M","family":"Bieleu","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology and ICU, AP\u2010HP Universit\u00e9 Paris Saclay H\u00f4pital Raymond Poincar\u00e9 Garches France"},{"name":"Centre de R\u00e9f\u00e9rence des Maladies Neuromusculaires Garches\u2010Necker\u2010Mondor\u2010Hendaye (GNMH), Centre Nord\u2010 Est\u2010 Ile de France R\u00e9seau national des maladies neuromusculaires, FILNEMUS  France"},{"name":"European Reference Center Network (Euro\u2010NMD ERN)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Gomez Garcia de la Banda","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology and ICU, AP\u2010HP Universit\u00e9 Paris Saclay H\u00f4pital Raymond Poincar\u00e9 Garches France"},{"name":"Centre de R\u00e9f\u00e9rence des Maladies Neuromusculaires Garches\u2010Necker\u2010Mondor\u2010Hendaye (GNMH), Centre Nord\u2010 Est\u2010 Ile de France R\u00e9seau national des maladies neuromusculaires, FILNEMUS  France"},{"name":"European Reference Center Network (Euro\u2010NMD ERN)"},{"name":"URC APHP Paris\u2010Saclay 4 Institut de Myologie Paris 5 APHP Raymond Poincar\u00e9 Hospital Garche France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7026-7523","authenticated-orcid":false,"given":"Harriet","family":"Corvol","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology Armand Trousseau University Hospital Paris France"},{"name":"Sorbonne Universit\u00e9 Centre de Recherche Saint\u2010Antoine (CRSA) Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9270-4061","authenticated-orcid":false,"given":"Laurent","family":"Servais","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, MDUK Oxford Neuromuscular Center University of Oxford Oxford UK"},{"name":"Division of Child Neurology Reference Center for 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