{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T13:56:55Z","timestamp":1791899815060,"version":"4.3.4"},"reference-count":35,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,10,4]],"date-time":"2006-10-04T00:00:00Z","timestamp":1159920000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2007,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Adult\u2010onset acid maltase deficiency is an inherited lysosomal skeletal\u2010muscle disease characterized by progressive myopathy and respiratory failure, for which there is no known therapy. In an uncontrolled, prospective study, we evaluated whether adherence to high\u2010protein and low\u2010carbohydrate nutrition and exercise therapy (NET) can slow the progressive deterioration of muscle function in this disease. Thirty\u2010four patients have been treated with NET for periods of 2\u201310 years (mean 4.5 \u00b1 2.5). Pre\u2010NET rate of muscle function deterioration, as measured by the Walton scale, was compared to post\u2010NET rate. Twenty\u2010six patients were deemed to be consistently compliant with NET. Difference between pre\u2010NET slope of muscle function deterioration to that of post\u2010NET slope in compliant patients was \u22120.29 (95% CI \u22120.19, 0.39) (<jats:italic>P<\/jats:italic> &lt; 0.0001). We conclude that compliance with NET can slow deterioration of muscle function and improve the natural history of adult\u2010onset acid maltase deficiency. Muscle Nerve, 2006<\/jats:p>","DOI":"10.1002\/mus.20665","type":"journal-article","created":{"date-parts":[[2006,10,4]],"date-time":"2006-10-04T19:28:06Z","timestamp":1159990086000},"page":"70-77","source":"Crossref","is-referenced-by-count":102,"title":["Modification of the natural history of adult\u2010onset acid maltase deficiency by nutrition and exercise therapy"],"prefix":"10.1002","volume":"35","author":[{"given":"Alfred E.","family":"Slonim","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Bulone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teresia","family":"Goldberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Minikes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Efrat","family":"Slonim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Galanko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Martiniuk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,12,14]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1097\/00125817-200103000-00008"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/PL00014270"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.55.5.710"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1249\/00005768-198205000-00012"},{"key":"e_1_2_7_6_2","first-page":"648","volume-title":"Myology","author":"Brown DH","year":"1994"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1249\/00005768-199201000-00016"},{"key":"e_1_2_7_8_2","first-page":"1559","volume-title":"Myology","author":"Engel AG","year":"2004"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0248-8663(05)82200-3"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20807"},{"key":"e_1_2_7_11_2","first-page":"46","article-title":"Management of muscular dystrophy","volume":"63","author":"Gardner\u2010Medwin D","year":"1977","journal-title":"Physiotherapy"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1152\/jappl.1973.34.1.107"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh384"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2005-0559"},{"key":"e_1_2_7_15_2","series-title":"The metabolic and molecular bases of inherited diseases","first-page":"3389","author":"Hirschhorn R","year":"2001"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1152\/jappl.1986.60.2.562"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.49.9.1011"},{"key":"e_1_2_7_18_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.55.8.1122"},{"key":"e_1_2_7_19_2","first-page":"36","article-title":"Mechanisms of chaperone\u2010mediated autophagy","author":"Majeski AE","year":"2004","journal-title":"Int J Biochem Cell Biol"},{"key":"e_1_2_7_20_2","first-page":"328","article-title":"Acid maltase deficiency in an adult. 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