{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,11,3]],"date-time":"2023-11-03T21:45:51Z","timestamp":1699047951737},"reference-count":25,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[1984,8,1]],"date-time":"1984-08-01T00:00:00Z","timestamp":460166400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Pediatr"],"published-print":{"date-parts":[[1984,8]]},"DOI":"10.1007\/bf00442456","type":"journal-article","created":{"date-parts":[[2004,11,12]],"date-time":"2004-11-12T04:05:15Z","timestamp":1100232315000},"page":"224-228","source":"Crossref","is-referenced-by-count":16,"title":["Systemic carnitine deficiency: Benefit of oral carnitine supplements vs. persisting biochemical abnormalities"],"prefix":"10.1007","volume":"142","author":[{"given":"M.","family":"Duran","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. B. C.","family":"de Klerk","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S. K.","family":"Wadman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H. R.","family":"Scholte","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. P.","family":"Beekman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F. G. I.","family":"Jennekens","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","reference":[{"key":"CR1","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1002\/ana.410070104","volume":"7","author":"C Angelini","year":"1980","unstructured":"Angelini C, Philippart M, Borrone C, Bresolin N, Cantini M, Luck S (1980) Multisystem triglyceride storage disorder with impaired long-chain fatty acid oxidation. Ann Neurol 7:5?10","journal-title":"Ann Neurol"},{"key":"CR2","doi-asserted-by":"crossref","first-page":"742","DOI":"10.1016\/S0022-3476(81)80834-7","volume":"98","author":"PF Bougn\u00e8res","year":"1981","unstructured":"Bougn\u00e8res PF, Saudubray JM, Marsac C, Bernard O, Odievre M, Girard J (1981) Fasting hypoglycemia resulting from hepatic carnitine palmitoyl transferase deficiency. J Pediatr 98:742?746","journal-title":"J Pediatr"},{"key":"CR3","doi-asserted-by":"crossref","first-page":"1389","DOI":"10.1056\/NEJM198012113032403","volume":"303","author":"PR Chapoy","year":"1980","unstructured":"Chapoy PR, Angelini C, Brown WJ, Stiff JE, Shug AL, Cederbaum SD (1980) Systemic carnitine deficiency. A treatable inherited lipid-storage disease presenting as Reye's syndrome. N Engl J Med 303: 1389?1394","journal-title":"N Engl J Med"},{"key":"CR4","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1136\/jnnp.40.2.170","volume":"40","author":"F Cornelio","year":"1977","unstructured":"Cornelio F, Di Donato S, Peluchetti D, Bizzi A, Bertagnolio B, D'Angelo A, Wiesmann U (1977) Fatal cases of lipid storage myopathy with carnitine deficiency. J Neurol Neurosurg Psychiatry 40:170?178","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"CR5","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1136\/jnnp.40.4.313","volume":"40","author":"AG Engel","year":"1977","unstructured":"Engel AG, Banker BQ, Eiben RM (1977) Carnitine deficiency: clinical, morphological, and biochemical observations in a fatal case. J Neurol Neurosurg Psychiatry 40:313?322","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"CR6","doi-asserted-by":"crossref","first-page":"1083","DOI":"10.2337\/diab.28.12.1083","volume":"28","author":"SM Genuth","year":"1979","unstructured":"Genuth SM, Hoppel CL (1979) Plasma and urinary carnitine in diabetic ketosis. Diabetes 28:1083?1087","journal-title":"Diabetes"},{"key":"CR7","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1016\/S0022-3476(80)80571-3","volume":"96","author":"AM Glasgow","year":"1980","unstructured":"Glasgow AM, Eng G, Engel AG (1980) Systemic carnitine deficiency simulating recurrent Reye syndrome. J Pediatr 96: 889?891","journal-title":"J Pediatr"},{"key":"CR8","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1203\/00006450-198210000-00012","volume":"16","author":"N Gregersen","year":"1982","unstructured":"Gregersen N, Wintzensen H, K\u00f8lvraa S, Christensen E, Christensen MF, Brandt NJ, Rasmussen K (1982) C6-C10-Dicarboxylic aciduria: investigations of a patient with riboflavin responsive multiple acyl-CoA dehydrogenation defects. Pediatr Res 16:861?868","journal-title":"Pediatr Res"},{"key":"CR9","first-page":"E409","volume":"238","author":"CL Hoppel","year":"1980","unstructured":"Hoppel CL, Genuth SM (1980) Carnitine metabolism in normal-weight and obese human subjects during fasting. Am J Physiol 238:E409-E415","journal-title":"Am J Physiol"},{"key":"CR10","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/S0378-4347(00)81190-1","volume":"164","author":"JP Kamerling","year":"1979","unstructured":"Kamerling JP, Brouwer M, Ketting D, Wadman SK (1979) Gas chromatography of urinary N-phenylacetylglutamine. J Chromatogr 164:217?221","journal-title":"J Chromatogr"},{"key":"CR11","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1212\/WNL.25.1.16","volume":"25","author":"G Karpati","year":"1975","unstructured":"Karpati G, Carpenter S, Engel AG, Watters G, Allen J, Rothman S, Klassen G, Mamer OA (1975) The syndrome of systemic carnitine deficiency. Neurology 25:16?24","journal-title":"Neurology"},{"key":"CR12","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1002\/bms.1200070203","volume":"7","author":"OA Mamer","year":"1980","unstructured":"Mamer OA, Montgomery JA, Colle E (1980) Profiles in altered metabolism III. (?-1)-hydroxyacid excretion in a case of episodic hypoglycemia. Biomed Mass Spectrom 7:53?57","journal-title":"Biomed Mass Spectrom"},{"key":"CR13","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/0009-8981(80)90227-2","volume":"103","author":"PB Mortensen","year":"1980","unstructured":"Mortensen PB, Gregersen N (1980) Medium-chain triglyceride medication as a pitfall in the diagnosis of non-ketotic C6-C10-dicarboxylic acidurias. Clin Chim Acta 103:33?37","journal-title":"Clin Chim Acta"},{"key":"CR14","doi-asserted-by":"crossref","first-page":"394","DOI":"10.1016\/0005-2760(81)90298-8","volume":"666","author":"PB Mortensen","year":"1981","unstructured":"Mortensen PB, Gregersen N (1981) The biological origin of ketotic dicarboxylic aciduria. In vivo and in vitro investigations of the ?-oxidation of C6-C10-monocarboxylic acids in unstarved, starved and diabetic rats. Biochim Biophys Acta 666:394?404","journal-title":"Biochim Biophys Acta"},{"key":"CR15","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1007\/BF01810358","volume":"2","author":"PB Mortensen","year":"1983","unstructured":"Mortensen PB, Gregersen N, Rasmussen K, K\u00f8lvraa S (1983) The ?-oxidation of dicarboxylic acids in isolated mitochondria and peroxisomes. J Inherited Metab Dis 6 [suppl] 2:123?124","journal-title":"J Inherited Metab Dis 6"},{"key":"CR16","first-page":"9","volume":"38","author":"A Niederwieser","year":"1983","unstructured":"Niederwieser A, Steinmann B, Exner U, Neuheiser F, Redweik U, Wang M, Rampini S, Wendel U (1983) Multiple acyl-CoA dehydrogenation deficiency in a boy with nonketotic hypoglycemia, hepatomegaly, muscle hypotonia and cardiomyopathy. Helv Paediatr Acta 38:9?26","journal-title":"Helv Paediatr Acta"},{"key":"CR17","first-page":"1758","volume":"14","author":"DJ Pearson","year":"1969","unstructured":"Pearson DJ, Tubbs PK, Chase JFA (1969) Carnitine and acylcarnitines. Methods Enzymol 14:1758?1771","journal-title":"Methods Enzymol"},{"key":"CR18","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1016\/S0022-3476(81)80583-5","volume":"98","author":"JM Saudubray","year":"1981","unstructured":"Saudubray JM, Marsac C, Limal JM, Dumurgier E, Charpentier C, Ogier H, Coude FX (1981) Variation in plasma ketone bodies during a 24-hour fast in normal and in hypoglycemic children: relationship to age. J Pediatr 98:904?908","journal-title":"J Pediatr"},{"key":"CR19","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/0022-510X(79)90154-0","volume":"42","author":"HR Scholte","year":"1979","unstructured":"Scholte HR, Meyer AEFH, van Wijngaarden GK, Leenders KL (1979) Familial carnitine deficiency. A fatal case and subclinical state in a sister. J Neurol Sci 42:87?101","journal-title":"J Neurol Sci"},{"key":"CR20","first-page":"215","volume-title":"Mitochondrial Pathology in Muscle Diseases","author":"HR Scholte","year":"1983","unstructured":"Scholte HR, Busch HFM, Barth PG, Beekman RP, Duran M, Przyrembel H, Roth B, Luyt-Houwen IEM (1983) Carnitine deficiency and respiratory chain blockade In: Scarlata G, Cerri C (eds) Mitochondrial Pathology in Muscle Diseases. Piccin Med Books, Padua, pp 215?228"},{"key":"CR21","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/S0022-3476(79)80364-9","volume":"94","author":"RBH Schutgens","year":"1979","unstructured":"Schutgens RBH, Heymans H, Ketel A, Veder HA, Duran M, Ketting D, Wadman SK (1979) Lethal hypoglycemia in a child with a deficiency of 3-hydroxy-3-methylglutaryl-coenzyme A lyase. J Pediatr 94: 89?91","journal-title":"J Pediatr"},{"key":"CR22","doi-asserted-by":"crossref","first-page":"702","DOI":"10.1542\/peds.57.5.702","volume":"57","author":"CA Stanley","year":"1976","unstructured":"Stanley CA, Baker L (1976) Hyperinsulinism in infancy: diagnosis by demonstration of abnormal response to fasting hypoglycemia. Pediatrics 57:702?711","journal-title":"Pediatrics"},{"key":"CR23","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1007\/BF00442403","volume":"134","author":"HL Teijema","year":"1980","unstructured":"Teijema HL, van Gelderen HH, Giesberts MAH (1980) Hypoketosis as a cause of symptoms in childhood hypoglycemia. Eur J Pediatr 134:51?55","journal-title":"Eur J Pediatr"},{"key":"CR24","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1056\/NEJM198108133050707","volume":"305","author":"ME Tripp","year":"1981","unstructured":"Tripp ME, Katcher ML, Peters HA, Gilbert EF, Arya S, Hodach RJ, Shug AL (1981) Sysmtemic carnitine deficiency presenting as familial endocardial fibroelastosis. A treatable cardiomyopathy. N Engl J Med 305:385?390","journal-title":"N Engl J Med"},{"key":"CR25","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1016\/0006-2944(78)90072-8","volume":"20","author":"VWM Hinsberg Van","year":"1978","unstructured":"Van Hinsberg VWM, Veerkamp JH, van Moerkerk HTB (1978) An accurate and sensitive assay of long-chain fatty acid oxidation in human skeletal muscle. Biochem Med 20:256?266","journal-title":"Biochem Med"}],"container-title":["European Journal of Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/BF00442456.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/BF00442456\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/BF00442456","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,30]],"date-time":"2023-04-30T05:07:13Z","timestamp":1682831233000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/BF00442456"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1984,8]]},"references-count":25,"journal-issue":{"issue":"3","published-print":{"date-parts":[[1984,8]]}},"alternative-id":["BF00442456"],"URL":"https:\/\/doi.org\/10.1007\/bf00442456","relation":{},"ISSN":["0340-6199","1432-1076"],"issn-type":[{"value":"0340-6199","type":"print"},{"value":"1432-1076","type":"electronic"}],"subject":[],"published":{"date-parts":[[1984,8]]}}}