{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T21:44:55Z","timestamp":1792878295395,"version":"4.5.0"},"reference-count":18,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2002,6,4]],"date-time":"2002-06-04T00:00:00Z","timestamp":1023148800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2002,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Our objectives were to describe survival, hospitalization, speech, and outcomes related to respirator needs for spinal muscular atrophy type 1 (SMA1) patients, using noninvasive or tracheostomy ventilation. From 65 SMA patients referred to our clinic since 1996, we chose 56 SMA1 patients who developed respiratory failure before age 2 years. Patients either had tracheostomy tubes (group A), or used noninvasive ventilation and assisted coughing; a previously reported extubation protocol (group B) was used as needed.<\/jats:p><jats:p>Sixteen patients underwent tracheostomy at 10.8\u2009\u00b1\u20095.0 months of age, 33 were in group B, and 7 others died without life\u2010support interventions. Compared to group B, group A patients had fewer hospitalizations until age 3 years, but more after age 5, and 15 of 16 lost all spontaneous breathing tolerance posttracheostomy and could not speak. One group A patient died at 16 months of age, and the others were 73.8\u2009\u00b1\u200957 months of age (the oldest was 19 years old). Two group B patients died at 6 and 13 months, respectively, whereas the other 31 were 41.8\u2009\u00b1\u200926.0 months (and up to 8.3 years) old. Three of 31 in group B required high\u2010span positive inspiratory pressure plus positive end\u2010expiratory pressure (PIP\u2009+\u2009PEEP) continuously with minimal tolerance for breathing on their own, and 4 could not communicate verbally.<\/jats:p><jats:p>In conclusion, SMA type 1 children can survive beyond 2 years of age when offered tracheostomy or noninvasive respiratory support. The latter is associated with fewer hospitalizations after age 5 years, freedom from daytime ventilator use, and the ability to speak. Pediatr Pulmonol. 2002; 34:16\u201322. \u00a9 2002 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ppul.10110","type":"journal-article","created":{"date-parts":[[2002,10,6]],"date-time":"2002-10-06T11:25:39Z","timestamp":1033903539000},"page":"16-22","source":"Crossref","is-referenced-by-count":184,"title":["Spinal muscular atrophy type 1: Management and outcomes"],"prefix":"10.1002","volume":"34","author":[{"given":"John R.","family":"Bach","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Scott","family":"Baird","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Plosky","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jose","family":"Navado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian","family":"Weaver","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2002,6,4]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-2008-1040858"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1090-3798(99)80012-9"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.117.4.1100"},{"key":"e_1_2_1_5_2","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1177\/088307388800300106","article-title":"Long\u2010term mechanical ventilation in infants with neuromuscular disease","volume":"3","author":"Iannaccone ST","year":"1988","journal-title":"J Child Neurol"},{"key":"e_1_2_1_6_2","first-page":"285","volume-title":"Pulmonary rehabilitation: the obstructive and paralytic conditions","author":"Bach JR","year":"1996"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1097\/00002060-199801000-00003"},{"key":"e_1_2_1_8_2","article-title":"Duchenne muscular dystrophy: prolongation of life by noninvasive respiratory muscle aids","author":"Gomez\u2010Merino E","journal-title":"Am J Phys Med Rehabil"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.110.6.1566"},{"key":"e_1_2_1_10_2","first-page":"91","volume-title":"Poliomyelitis: papers and discussions presented at the Third International Poliomyelitis Conference","author":"Hodes HL","year":"1955"},{"key":"e_1_2_1_11_2","first-page":"80","article-title":"Alveolar ventilation as a function of ventilatory support method","volume":"5","author":"Bach JR","year":"1995","journal-title":"Eur J Phys Med Rehabil"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.149.6.8004310"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1159\/000127928"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00624607"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1038\/sc.1975.28"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0300-9785(86)80097-7"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.85.3.363"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.104.5.1634"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8249(199903)45:3<337::AID-ANA9>3.0.CO;2-U"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fppul.10110","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.10110","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,18]],"date-time":"2023-10-18T06:31:19Z","timestamp":1697610679000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.10110"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2002,6,4]]},"references-count":18,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2002,7]]}},"alternative-id":["10.1002\/ppul.10110"],"URL":"https:\/\/doi.org\/10.1002\/ppul.10110","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2002,6,4]]}}}