{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T07:50:07Z","timestamp":1790063407425,"version":"4.0.1"},"reference-count":29,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2017,7,10]],"date-time":"2017-07-10T00:00:00Z","timestamp":1499644800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2017,7,10]],"date-time":"2017-07-10T00:00:00Z","timestamp":1499644800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"publisher","award":["DK107900"],"award-info":[{"award-number":["DK107900"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100014093","name":"Galactosemia Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100014093","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2017,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>One of many vexing decisions faced by parents of an infant with classic galactosemia (CG) is how carefully to restrict non\u2010dairy galactose from their growing child's diet. Until recently, many experts recommended vigorous lifelong dietary restriction of milk and all high\u2010galactose dairy products as well as some non\u2010dairy sources of galactose such as legumes and specific fruits and vegetables. Recently, experts have begun to relax their recommendations. The new recommendations, that restrict only high galactose dairy products, were made in the face of uncertainty, however, because no sufficiently powered study had been reported testing for possible association between rigor of non\u2010dairy galactose restriction and severity of long\u2010term outcomes in CG. Here we describe the largest study of diet and outcomes in CG reported to date, conducted using information gathered from 231 patients with CG and 71 unaffected sibling controls. We compared rigor of dietary galactose restriction, measured using a 4\u2010point scale by a retrospective parent\u2010response survey, with outcomes including growth, adaptive behaviors, receipt of speech therapy, receipt of special educational services, and for girls and women, a plasma marker of ovarian function (AMH). Our results confirmed the expected differences between patients and controls, but among patients showed no significant association between rigor of non\u2010dairy galactose restriction in early childhood and any of the outcomes quantified. Indeed, some weak associations were seen suggesting that rigorous restriction of non\u2010dairy galactose may be deleterious rather than beneficial. Despite limitations, these findings support the ongoing trend toward diet liberalization with regard to non\u2010dairy sources of galactose for children and adults with classic galactosemia.<\/jats:p>","DOI":"10.1007\/s10545-017-0067-x","type":"journal-article","created":{"date-parts":[[2017,7,10]],"date-time":"2017-07-10T12:51:24Z","timestamp":1499691084000},"page":"813-821","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Rigor of non\u2010dairy galactose restriction in early childhood, measured by retrospective survey, does not associate with severity of five long\u2010term outcomes quantified in 231 children and adults with classic galactosemia"],"prefix":"10.1002","volume":"40","author":[{"given":"Allison B.","family":"Frederick","sequence":"first","affiliation":[{"name":"Department of Human Genetics Emory University School of Medicine 3rd Floor Whitehead Bldg., 615 Michael St Atlanta GA 30322 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David J.","family":"Cutler","sequence":"additional","affiliation":[{"name":"Department of Human Genetics Emory University School of Medicine 3rd Floor Whitehead Bldg., 615 Michael St Atlanta GA 30322 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith L.","family":"Fridovich\u2010Keil","sequence":"additional","affiliation":[{"name":"Department of Human Genetics Emory University School of Medicine 3rd Floor Whitehead Bldg., 615 Michael St Atlanta GA 30322 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,7,10]]},"reference":[{"key":"e_1_2_12_2_1","unstructured":"AcostaP YannicelliS(2001)Protocol 14 \u2013 Galactosemia nutritional support protocols 4th edn. 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