{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T02:55:41Z","timestamp":1761101741400},"reference-count":28,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[1989,7,20]],"date-time":"1989-07-20T00:00:00Z","timestamp":616896000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1990,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The biochemical and growth responses to dietary branched\u2010chain amino acid (BCAA) intake were studied in two children; one with a disorder of branched\u2010chain amino acid metabolism, maple syrup urine disease (MSUD) (McKusick 24860), and another with methylmalonic aciduria (MMA) (McKusick 25100). Biochemical control of MSUD focussed on plasma leucine levels while measurement of plasma ammonia levels was used in MMA. From 0 to 2.75 years both patients exhibited five episodes of toxicity. In each case toxicity was associated with dietary indiscretion or infection. The quantity of protein tolerated was always less in the MMA patient and was approximately 1 g\/kg\/day. From 1 to 2.75 years each patient's growth velocity approximated their predicted growth channel except during periods of toxicity. In both cases leucine intake, which gave normal growth without toxicity, was always judged to be less than the FAO\/WHO recommendations. The BCAA intake of the MMA patient was remarkably stable from 0.5 to 2.75 years and at 2 years of age isoleucine and valine intake approximated the FAO\/WHO recommendations. From 2 to 2.75 years BCAA intake (mg\/kg\/day) of the MSUD patient was less than that of the MMA patient and well below FAO\/WHO recommendations. Neuromotor development in both patients is normal.<\/jats:p>","DOI":"10.1007\/bf01799675","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T13:55:56Z","timestamp":1119362156000},"page":"125-136","source":"Crossref","is-referenced-by-count":8,"title":["Evaluation of branched\u2010chain amino acid intake in children with maple syrup urine disease and methylmalonic aciduria"],"prefix":"10.1002","volume":"13","author":[{"given":"H. G.","family":"Parsons","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine University of Calgary  3330 Hospital Drive NW Calgary Alberta T2N 1N4 Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. J.","family":"Carter","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine University of Calgary  3330 Hospital Drive NW Calgary Alberta T2N 1N4 Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Unrath","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine University of Calgary  3330 Hospital Drive NW Calgary Alberta T2N 1N4 Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F. F.","family":"Snyder","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine University of Calgary  3330 Hospital Drive NW Calgary Alberta T2N 1N4 Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1989,7,20]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"865","article-title":"Outcome of early and long\u2010term management of classical Maple Syrup Urine Disease","volume":"68","author":"Chow C. L.","year":"1981","journal-title":"Pediatrics"},{"key":"e_1_2_1_3_1","first-page":"1005","article-title":"Management of Maple Syrup Urine Disease in Canada","volume":"115","author":"Committee for Improvement of Hereditary Disease Management","year":"1975","journal-title":"Can. Med. Assoc. 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Technical Report Series No. 724 World Health Organization Geneva 1985."},{"key":"e_1_2_1_11_1","first-page":"1","volume-title":"Clinical Pediatrics and Adolescent Endocrinology","author":"Kaplan S. 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