{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T20:21:29Z","timestamp":1791145289022,"version":"4.1.0"},"reference-count":12,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[1999,4,1]],"date-time":"1999-04-01T00:00:00Z","timestamp":922924800000},"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":[[1999,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Current dietary management of long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase (LCHAD; long\u2010chain\u2010(S)\u20103\u2010hydroxyacyl\u2010CoA:NAD+ oxido\u2010reductase, EC 1.1.1.211) deficiency (LCHADD) is based on avoiding fasting, andminimizing energy production from long\u2010chain fatty acids. We report the effects of various dietary manipulations on plasma and urinary laboratory values in a child with LCHADD. In our patient, a diet restricted to 9% of total energy from long\u2010chain fatty acids and administration of 1.5g medium\u2010chain triglyceride oil per kg body weight normalized plasma acylcarnitine and lactate levels, but dicarboxylic acid excretion remained approximately ten times normal. Plasma docosahexaenoic acid (DHA, 22:6<jats:italic>n<\/jats:italic>\u22123) was consistentlylow over a 2\u2010year period; DHA deficiency may be related to the development of pigmentary retinopathy seen in this patient population. We also conducted a survey of metabolic physicians who treat children with LCHADD to determine current dietary interventions employed and the effects of these interventions on symptoms of this disease. Survey results indicate that a diet low in long\u2010chain fatty acids, supplemented with medium\u2010chain triclyceride oil, decreased the incidence of hypoketotic hypoglycaemia, and improved hypotonia, hepatomegaly, cardiomyopathy, and lactic acidosis. However, dietary treatment did not appear to effect peripheral neuropathy, pigmentary retinopathy or myoglobinuria.<\/jats:p>","DOI":"10.1023\/a:1005437616934","type":"journal-article","created":{"date-parts":[[2002,12,21]],"date-time":"2002-12-21T22:25:23Z","timestamp":1040509523000},"page":"123-131","source":"Crossref","is-referenced-by-count":74,"title":["Dietary management of long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency (LCHADD). A case report and survey"],"prefix":"10.1002","volume":"22","author":[{"given":"M.","family":"Gillingham","sequence":"first","affiliation":[{"name":"The Biochemical Genetics Program, Waisman Center, Department of Pediatrics University of Wisconsin \u2013 Madison  Madison Wisconsin USA; and"},{"name":"The Department of Nutritional Sciences University of Wisconsin \u2013 Madison  Madison Wisconsin USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Van Calcar","sequence":"additional","affiliation":[{"name":"The Biochemical Genetics Program, Waisman Center, Department of Pediatrics University of Wisconsin \u2013 Madison  Madison Wisconsin USA; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Ney","sequence":"additional","affiliation":[{"name":"The Department of Nutritional Sciences University of Wisconsin \u2013 Madison  Madison Wisconsin USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Wolff","sequence":"additional","affiliation":[{"name":"The Biochemical Genetics Program, Waisman Center, Department of Pediatrics University of Wisconsin \u2013 Madison  Madison Wisconsin USA; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Harding","sequence":"additional","affiliation":[{"name":"The Biochemical Genetics Program, Waisman Center, Department of Pediatrics University of Wisconsin \u2013 Madison  Madison Wisconsin USA; and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[1999,4]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-4835(70)80046-X"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)83318-9"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/45.1.66"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1006\/bmme.1996.0033"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)43209-2"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01963564"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02535868"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/jn\/70.3.405"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1006895209833"},{"key":"e_1_2_1_11_1","first-page":"1501","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Roe CR","year":"1995"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840190211"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.92.3.841"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1023%2FA%3A1005437616934","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1023\/A%3A1005437616934","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T19:15:25Z","timestamp":1694805325000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1023\/A%3A1005437616934"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1999,4]]},"references-count":12,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1999,4]]}},"alternative-id":["10.1023\/A:1005437616934"],"URL":"https:\/\/doi.org\/10.1023\/a:1005437616934","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1999,4]]}}}