{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T11:59:54Z","timestamp":1791892794618,"version":"4.3.4"},"reference-count":13,"publisher":"Wiley","license":[{"start":{"date-parts":[[2017,1,1]],"date-time":"2017-01-01T00:00:00Z","timestamp":1483228800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Case Reports in Dermatological Medicine"],"published-print":{"date-parts":[[2017]]},"abstract":"<jats:p><jats:italic>Introduction<\/jats:italic>. Maple syrup urine disease (MSUD) is an inborn error of branched chain amino acids (BCAAs) metabolism. We report an infant with MSUD who developed 2 episodes of cutaneous lesions as a result of isoleucine deficiency and zinc deficiency, respectively.<jats:italic> Case Presentation<\/jats:italic>. A 12-day-old male infant was presented with poor milk intake and lethargy. The diagnosis of MSUD was made based on clinical and biochemical data.<jats:italic> Management and Outcome<\/jats:italic>. Specific dietary restriction of BCAAs was given. Subsequently, natural protein was stopped as the patient developed hospital-acquired infections which resulted in an elevation of BCAAs. Acrodermatitis dysmetabolica developed and was confirmed to be from isoleucine deficiency. At the age of 6 months, the patient developed severe lethargy and was on natural protein exclusion for an extended period. Despite enteral supplementation of zinc sulfate, cutaneous manifestations due to zinc deficiency occurred.<jats:italic> Discussion<\/jats:italic>. Skin lesions in MSUD patients could arise from multiple causes. Nutritional deficiency including isoleucine and zinc deficiencies can occur and could complicate the treatment course as a result of malabsorption, even while on enteral supplementation. Parenteral nutrition should be considered and initiated accordingly. Clinical status, as well as BCAA levels, should be closely monitored in MSUD patients.<\/jats:p>","DOI":"10.1155\/2017\/3905658","type":"journal-article","created":{"date-parts":[[2017,10,25]],"date-time":"2017-10-25T19:31:20Z","timestamp":1508959880000},"page":"1-4","source":"Crossref","is-referenced-by-count":7,"title":["Skin Lesions Associated with Nutritional Management of Maple Syrup Urine Disease"],"prefix":"10.1155","volume":"2017","author":[{"given":"Jaraspong","family":"Uaariyapanichkul","sequence":"first","affiliation":[{"name":"Division of Nutrition, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand"},{"name":"Division of Nutrition, Department of Pediatrics, King Chulalongkorn Memorial Hospital, The Thai Red Cross Society, Bangkok 10330, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Puthita","family":"Saengpanit","sequence":"additional","affiliation":[{"name":"Division of Nutrition, Department of Pediatrics, King Chulalongkorn Memorial Hospital, The Thai Red Cross Society, Bangkok 10330, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ponghatai","family":"Damrongphol","sequence":"additional","affiliation":[{"name":"Center of Excellence for Medical Genetics, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand"},{"name":"Excellence Center for Medical Genetics, King Chulalongkorn Memorial Hospital, The Thai Red Cross Society, Bangkok 10330, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kanya","family":"Suphapeetiporn","sequence":"additional","affiliation":[{"name":"Center of Excellence for Medical Genetics, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand"},{"name":"Excellence Center for Medical Genetics, King Chulalongkorn Memorial Hospital, The Thai Red Cross Society, Bangkok 10330, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9712-2474","authenticated-orcid":true,"given":"Sirinuch","family":"Chomtho","sequence":"additional","affiliation":[{"name":"Division of Nutrition, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","reference":[{"key":"1","first-page":"906","volume-title":"Inherited metabolic disease: Amino acids, Organic acids, and Galactose","year":"2014"},{"key":"2","doi-asserted-by":"publisher","DOI":"10.3109\/10408363.2013.847896"},{"key":"3","first-page":"399","volume-title":"Disorders of amino acid metabolism, organic acidaemias and urea cycle defects; Maple syrup urine disease","year":"2015"},{"key":"4","doi-asserted-by":"publisher","DOI":"10.1007\/s13312-016-0922-0"},{"key":"5","doi-asserted-by":"publisher","DOI":"10.1016\/S0151-9638(06)70919-X"},{"issue":"3","key":"6","first-page":"244","volume":"11","year":"2001","journal-title":"European Journal of Dermatology"},{"key":"7","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(94)70364-7"},{"key":"8","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.1998.02415.x"},{"key":"9","doi-asserted-by":"publisher","DOI":"10.1684\/ejd.2010.1175"},{"key":"10","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.2008.00803.x"},{"key":"11","doi-asserted-by":"publisher","DOI":"10.1111\/ced.12876"},{"key":"12","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0b013e328335107f"},{"key":"13","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1993.01680240130027"}],"container-title":["Case Reports in Dermatological Medicine"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/downloads.hindawi.com\/journals\/cridm\/2017\/3905658.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/cridm\/2017\/3905658.xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/cridm\/2017\/3905658.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2017,10,25]],"date-time":"2017-10-25T19:31:20Z","timestamp":1508959880000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.hindawi.com\/journals\/cridm\/2017\/3905658\/"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017]]},"references-count":13,"alternative-id":["3905658","3905658"],"URL":"https:\/\/doi.org\/10.1155\/2017\/3905658","relation":{},"ISSN":["2090-6463","2090-6471"],"issn-type":[{"value":"2090-6463","type":"print"},{"value":"2090-6471","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017]]}}}