{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T12:32:56Z","timestamp":1763037176199,"version":"3.37.3"},"reference-count":25,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2018,3,25]],"date-time":"2018-03-25T00:00:00Z","timestamp":1521936000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Sectie Kinderanesthesiologie (Dutch Society for Pediatric Anesthesiology) of the Dutch Society for Anesthesiology"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Anesthesia"],"published-print":{"date-parts":[[2018,5]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Pompe disease is a rare metabolic disorder caused by a deficiency of the lysosomal enzyme acid \u03b1\u2010glucosidase. Glycogen accumulation damages skeletal, cardiac, and smooth muscles, causing a progressive and debilitating muscle weakness and cardiomyopathy. As life expectancy has much improved since the introduction of enzyme replacement therapy an increasing number of patients are referred for surgical procedures. Due to the potential cardiopulmonary complications, these patients form a high\u2010risk group for the anesthesiologist.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>In this study, we investigated the incidence of perioperative complications in children with Pompe disease treated in our hospital since the introduction of enzyme replacement therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Anesthetic and perioperative data of children with Pompe disease treated between 1999 and 2015 in the Erasmus <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content>\u2014Sophia Children's Hospital, University Medical Centre, Rotterdam, The Netherlands, were collected, retrospectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of the 65 children with Pompe disease, 34 patients underwent in total 77, mostly low\u2010risk, surgical procedures. Twenty\u2010one children had the classic infantile form and 13 had a nonclassic presentation of Pompe disease. In 13 (16.8%) procedures, 1 or more perioperative complications occurred. Perioperative desaturation was the main complication (12.9%), followed by arrhythmia (3.8%) and heart failure requiring diuretic treatment (2.6%). One child died 2\u00a0days postoperatively, but this was considered unrelated to the procedure.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Despite the potentially high anesthetic risk for children with Pompe disease under enzyme replacement therapy, the incidence of perioperative complications in our study was relatively low. Our data suggest that with proper precautionary measures and a critical choice of timing of the operation, general anesthesia in children with Pompe disease could be relatively safe nowadays.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pan.13361","type":"journal-article","created":{"date-parts":[[2018,3,25]],"date-time":"2018-03-25T17:37:36Z","timestamp":1521999456000},"page":"428-435","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Perioperative management of children with glycogen storage disease type <scp>II<\/scp>\u2014Pompe disease"],"prefix":"10.1111","volume":"28","author":[{"given":"Linelot","family":"Bosman","sequence":"first","affiliation":[{"name":"Department of Anesthesiology Erasmus MC University Medical Centre Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sanne E.","family":"Hoeks","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Erasmus MC University Medical Centre Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonia","family":"Gonz\u00e1lez Candel","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Erasmus MC\u2014Sophia Children's Hospital University Medical Centre Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hannerieke J. M.","family":"van den Hout","sequence":"additional","affiliation":[{"name":"Division of Metabolic Diseases and Genetics Department of Pediatrics Erasmus MC\u2014Sophia Children's Hospital University Medical Centre Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ans T.","family":"van der Ploeg","sequence":"additional","affiliation":[{"name":"Division of Metabolic Diseases and Genetics Department of Pediatrics Erasmus MC\u2014Sophia Children's Hospital University Medical Centre Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7867-971X","authenticated-orcid":false,"given":"Lonneke M.","family":"Staals","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Erasmus MC\u2014Sophia Children's Hospital University Medical Centre Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,3,25]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.281.3.249"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.112.2.332"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2005.11.033"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.gim.0000218152.87434.f3"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(00)02533-2"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000251268.41188.04"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9592.2007.02215.x"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2044.1986.tb13007.x"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9592.2004.01242.x"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00101-012-1998-0"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2008.07.013"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00020005"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000198776.53007.2c"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(08)61555-X"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-011-9404-7"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-7-88"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa033349"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.57.7.1290"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-011-9388-3"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(10)61193-2"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.gim.0000195896.04069.5f"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.113.5.e448"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-015-9912-y"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9592.2007.02196.x"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00540-010-0984-4"}],"container-title":["Pediatric Anesthesia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpan.13361","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pan.13361","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T14:20:13Z","timestamp":1695478813000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pan.13361"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,3,25]]},"references-count":25,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2018,5]]}},"alternative-id":["10.1111\/pan.13361"],"URL":"https:\/\/doi.org\/10.1111\/pan.13361","archive":["Portico"],"relation":{},"ISSN":["1155-5645","1460-9592"],"issn-type":[{"type":"print","value":"1155-5645"},{"type":"electronic","value":"1460-9592"}],"subject":[],"published":{"date-parts":[[2018,3,25]]},"assertion":[{"value":"2018-02-15","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-03-25","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}