{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T18:15:00Z","timestamp":1777918500625,"version":"3.51.4"},"reference-count":24,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2014,3,10]],"date-time":"2014-03-10T00:00:00Z","timestamp":1394409600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Anesthesia"],"published-print":{"date-parts":[[2014,5]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Mucopolysaccharidoses (<jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content>) are hereditary storage diseases; airway management typically worsens in these patients with the progression of the disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective(s)<\/jats:title><jats:p>To assess the incidence of perioperative complications in children with <jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content> and the impact of enzyme replacement therapy (<jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>) followed by hematopoietic stem cell transplantation (<jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The records of patients with <jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content> treated with <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> followed by <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>, who received anesthesia at the Wilhelmina Children's Hospital between 2003 and 2012, were reviewed. Data were collected on incidence of perioperative respiratory and cardiovascular complications and the impact of treatment and age.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Nineteen children with <jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content> were identified (including 17 Hurler patients), who received <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> treatment followed by <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>. Median age at start of treatment was 14 (range: 7\u201343) months. Patients were anesthetized 136 times. The incidence of respiratory and cardiovascular complications was 24% and 4%, respectively. Airway management by face mask was difficult in 7%. There were no problems with the laryngeal mask airway. Tracheal intubation was difficult in 25% and failed in 10%; using a video laryngoscope was most successful (89%), followed by classic laryngoscope (67%) and fiber\u2010optic scope (20%). Multivariate logistic regression analyses showed that the incidence of perioperative respiratory problems did not increase with age or decrease after start of treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Perioperative airway management was most successful using a laryngeal mask airway or video laryngoscope. Treatment with <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> followed by <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content> and patient age did not influence the incidence of perioperative respiratory problems.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pan.12370","type":"journal-article","created":{"date-parts":[[2014,3,10]],"date-time":"2014-03-10T13:32:45Z","timestamp":1394458365000},"page":"521-527","source":"Crossref","is-referenced-by-count":23,"title":["Perioperative complications in patients diagnosed with mucopolysaccharidosis and the impact of enzyme replacement therapy followed by hematopoietic stem cell transplantation at early age"],"prefix":"10.1111","volume":"24","author":[{"given":"Johanna H.A.M.","family":"Megens","sequence":"first","affiliation":[{"name":"Department of Anesthesiology Intensive Care and Emergency Medicine Wilhelmina Children's Hospital University Medical Centre Utrecht Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michel","family":"de Wit","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Intensive Care and Emergency Medicine Wilhelmina Children's Hospital University Medical Centre Utrecht Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter M.","family":"van Hasselt","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Metabolic diseases Wilhelmina Children's Hospital University Medical Centre Utrecht Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jaap Jan","family":"Boelens","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Blood and Marrow Transplantation Program Wilhelmina Children's Hospital University Medical Centre Utrecht Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Desiree B.M.","family":"van der 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