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Uniform criteria for starting and stopping <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> in adult patients were developed and reported here.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Three consensus meetings were organized through the European Pompe Consortium, a network of experts from 11 European countries in the field of Pompe disease. A systematic review of the literature was undertaken to determine the effectiveness of <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> in adult patients on a range of clinical outcome measures and quality of life. A narrative synthesis is presented.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Consensus was reached on how the diagnosis of Pompe disease should be confirmed, when treatment should be started, reasons for stopping treatment and the use of <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> during pregnancy. This was based on expert opinion and supported by the literature. One clinical trial and 43 observational studies, covering a total of 586 individual adult patients, provided evidence of a beneficial effect of <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> at group level. At individual patient level, the response to treatment varied, but factors associated with a patient's response to <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> were not described in many studies. Eleven observational studies focused on more severely affected patients, suggesting that <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> can also be beneficial in these patients. There are no studies on the effects of <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> in pre\u2010symptomatic patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This is the first European consensus recommendation for starting and stopping <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> in adult patients with Pompe disease, based on the extensive experience of experts from different countries.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.13285","type":"journal-article","created":{"date-parts":[[2017,5,6]],"date-time":"2017-05-06T07:07:05Z","timestamp":1494054425000},"page":"768","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":136,"title":["European consensus for starting and stopping enzyme replacement therapy in adult patients with Pompe disease: a 10\u2010year experience"],"prefix":"10.1111","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3359-1324","authenticated-orcid":false,"given":"A. 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