{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T15:39:42Z","timestamp":1790869182842,"version":"4.1.0"},"reference-count":56,"publisher":"American Academy of Pediatrics (AAP)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2002,1,1]]},"abstract":"<jats:p>Objectives. To assess the mode of presentation, biochemical abnormalities, clinical course, and effects of therapy in patients of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency.<\/jats:p>\n               <jats:p>Background. LCHAD deficiency is a rare, autosomal recessive inborn error of fatty acid oxidation. Although case reports and small series of patients have been published, these may not give a true picture of the clinical and biochemical spectrum associated with this disorder. To improve the early recognition and management of this potentially lethal disorder, we have reviewed a large cohort of LCHAD-deficient patients.<\/jats:p>\n               <jats:p>Methods. A questionnaire was sent to the referring physicians of 61 unselected patients with LCHAD deficiency diagnosed in our center. The standardized questionnaire requested information about the clinical signs and symptoms at presentation, the clinical history, family history, pregnancy, biochemical parameters at presentation, treatment, and clinical outcome.<\/jats:p>\n               <jats:p>Results. Questionnaires on 50 patients (82%) were returned and included in this study. The mean age of clinical presentation was 5.8 months (range: 1 day-26 months). Seven (15%) of the patients presented in the neonatal period. Thirty-nine patients (78%) presented with hypoketotic hypoglycemia, the classical features of a fatty acid oxidation disorder. Eleven patients (22%) presented with chronic problems, consisting of failure to thrive, feeding difficulties, cholestatic liver disease, and\/or hypotonia. In retrospect, most (82%) of the patients presenting with an acute metabolic derangement also suffered from a combination of chronic nonspecific symptoms before the metabolic crises. Mortality in this series was high (38%), all dying before or within 3 months after diagnosis. Morbidity in the surviving patients is also high, with recurrent metabolic crises and muscle problems despite therapy.<\/jats:p>\n               <jats:p>Conclusions. LCHAD deficiency often presents with a combination of chronic nonspecific symptoms. Early diagnosis is difficult in the absence of the classical metabolic derangement. Survival can be improved by prompt diagnosis, but morbidity remains alarmingly high despite current therapeutic regimes.<\/jats:p>","DOI":"10.1542\/peds.109.1.99","type":"journal-article","created":{"date-parts":[[2004,8,14]],"date-time":"2004-08-14T00:17:55Z","timestamp":1092442675000},"page":"99-104","source":"Crossref","is-referenced-by-count":180,"title":["Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency: Clinical Presentation and Follow-Up of 50 Patients"],"prefix":"10.1542","volume":"109","author":[{"given":"Margarethe E. J.","family":"den Boer","sequence":"first","affiliation":[{"name":"Department of Pediatrics Academic Medical Center, University of Amsterdam, the Netherlands"},{"name":"Department of Pediatrics Academic Medical Center, University of Amsterdam, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald J. A.","family":"Wanders","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Academic Medical Center, University of Amsterdam, the Netherlands"},{"name":"Department of Clinical Chemistry, Academic Medical Center, University of Amsterdam, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew A. M.","family":"Morris","sequence":"additional","affiliation":[{"name":"Department of Department of Child Health, University of Newcastle Upon Tyne, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lodewijk","family":"IJlst","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry, Academic Medical Center, University of Amsterdam, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hugo S. A.","family":"Heymans","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Academic Medical Center, University of Amsterdam, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frits A.","family":"Wijburg","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Academic Medical Center, University of Amsterdam, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2002,1,1]]},"reference":[{"key":"2021121023241470400_R1","doi-asserted-by":"crossref","unstructured":"Wanders RJA, IJlst L, van Gennip AH, et al. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of a new inborn error of mitochondrial fatty acid \u03b2-oxidation. J Inherit Metab Dis.1990;13:311\u2013314","DOI":"10.1007\/BF01799383"},{"key":"2021121023241470400_R2","unstructured":"Roe CR, Coates PM. Mitochondrial fatty acid oxidation disorders. In: The Metabolic and Molecular Basis of Inherited Disease. New York, NY: McGraw-Hill; 1995: 1513\u20131517"},{"key":"2021121023241470400_R3","unstructured":"Millington DS, Terada N, Chace DH, et al. The role of tandem mass spectometry in the diagnosis of fatty acid oxidation disorders. Prog Clin Biol Res.1992;375:339\u201354"},{"key":"2021121023241470400_R4","doi-asserted-by":"crossref","unstructured":"Wanders RJA, IJlst L, Poggi F, et al. Human trifunctional protein deficiency: a new disorder of mitochondrial fatty acid \u03b2-oxidation. Biochem Biophys Res Commun.1992;188:1139\u20131145","DOI":"10.1016\/0006-291X(92)91350-Y"},{"key":"2021121023241470400_R5","doi-asserted-by":"crossref","unstructured":"IJlst L, Ruiter JPN, Hoovers JMN, Jakobs ME, Wanders RJA. Common missense mutation G1528C in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. J Clin Invest.1996;98:1028\u20131033","DOI":"10.1172\/JCI118863"},{"key":"2021121023241470400_R6","doi-asserted-by":"crossref","unstructured":"den Boer MEJ, IJlst L, Wijburg FA, et al. Heterozygosity for the common LCHAD mutation (1528G&gt;C) is not a major cause of HELLP syndrome and the prevalence of the mutation in the Dutch population is low. Pediatr Res.2000;48:1\u20134","DOI":"10.1203\/00006450-200008000-00006"},{"key":"2021121023241470400_R7","doi-asserted-by":"crossref","unstructured":"Pons RP, Roig M, Riudor E, et al. The clinical spectrum of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Pediatr Neurol.1996;4:236\u2013243","DOI":"10.1016\/0887-8994(96)00021-5"},{"key":"2021121023241470400_R8","doi-asserted-by":"crossref","unstructured":"van Maldergem L, Tuerlinckx D, Wanders RJ, et al. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and early-onset liver cirrhosis in two siblings. Eur J Pediatr.2000;159:108\u2013112","DOI":"10.1007\/s004310050022"},{"key":"2021121023241470400_R9","doi-asserted-by":"crossref","unstructured":"Przyrembel H, Jakobs C, IJlst L, de Klerk JBC, Wanders RJA. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. J Inherit Metab Dis.1991;14:674\u201368","DOI":"10.1007\/BF01799932"},{"key":"2021121023241470400_R10","doi-asserted-by":"crossref","unstructured":"Das AM, Fingerhut R, Wanders RJA, Ullrich K. Secondary respiratory chain defect in a boy with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: possible diagnostic pitfalls. Eur J Pediatr.2000;159:243\u2013246","DOI":"10.1007\/s004310050063"},{"key":"2021121023241470400_R11","doi-asserted-by":"crossref","unstructured":"Jackson S, Bartlett K, Land J, et al. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Pediatr Res.1991;29:406\u2013411","DOI":"10.1203\/00006450-199104000-00016"},{"key":"2021121023241470400_R12","doi-asserted-by":"crossref","unstructured":"Perez-Cerda C, Merinero B, Jiminez A, et al. First report of prenatal diagnosis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency in a pregnancy at risk. Prenat Diagn.1993;13:529\u2013533","DOI":"10.1002\/pd.1970130616"},{"key":"2021121023241470400_R13","doi-asserted-by":"crossref","unstructured":"Wanders RJA, IJlst L, Duran M, et al. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: different clinical expression in three unrelated patients. J Inherit Metab Dis.1991;14:325\u2013328","DOI":"10.1007\/BF01811694"},{"key":"2021121023241470400_R14","doi-asserted-by":"crossref","unstructured":"Tyni T, Palotie A, Viinikka L, et al. Long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency with the G1528C mutation: Clinical presentation of 13 patients. J Pediatr.1997;130:67\u201376","DOI":"10.1016\/S0022-3476(97)70312-3"},{"key":"2021121023241470400_R15","doi-asserted-by":"crossref","unstructured":"Gillingham M, Van Calcar S, Ney D, Wolff J, Harding C. Dietary treatment of long-chain 3-hydroxyacyl-CoA dehyrogenase deficiency (LCHAD). A case report and survey. J Inherit Metab Dis.1999;2:123\u2013131","DOI":"10.1023\/A:1005437616934"},{"key":"2021121023241470400_R16","doi-asserted-by":"crossref","unstructured":"Sewell AC, Bender SW, Wirth S, Munterfering H, IJlst L, Wanders RJA Long-chain 3-hydroxyacylCoA dehydrogenase deficiency: a severe fatty acid oxidation disorder. Eur J Pediatr.153:745\u2013750","DOI":"10.1007\/BF01954492"},{"key":"2021121023241470400_R17","doi-asserted-by":"crossref","unstructured":"Tein I, Donner EJ, Hale DE, Murphy EG. Clinical and neurophysiologic response of myopathy and neuropathy in long-chain l-3-hydroxyacyl-CoA dehydrogenase deficiency to oral prednisone. Pediatr Neurol.1995;12:68\u201376","DOI":"10.1016\/0887-8994(94)00109-F"},{"key":"2021121023241470400_R18","doi-asserted-by":"crossref","unstructured":"Bertini E, Dionici-Vici C, Garavaglia B, et al. Peripheral sensory-motor polyneuropathy, pigmentary retinopathy, and fatal cardiomyopathy in long-chain 3-hydroxyacylCoA dehydrogenase deficiency. Eur J Pediatr.1992;51:121\u20136","DOI":"10.1007\/BF01958956"},{"key":"2021121023241470400_R19","doi-asserted-by":"crossref","unstructured":"Fryburg JS, Pelegano JP, Bennett MJ, Bebin EM. Long-chain 3-hydroxyacyl-Coenzyme A dehydrogenase (LCHAD) deficiency in a patient with the Bannayan-Riley-Ruvalcaba syndrome. Am J Med Genet.1994;52:97\u2013102","DOI":"10.1002\/ajmg.1320520119"},{"key":"2021121023241470400_R20","doi-asserted-by":"crossref","unstructured":"Glasgow AM, Engel AG, Bier DM, et al. Hypoglycemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain acylcarnitine excess responsive to medium triglyceride diet. Pediatr Res.1983;7:319\u2013326","DOI":"10.1203\/00006450-198305000-00003"},{"key":"2021121023241470400_R21","doi-asserted-by":"crossref","unstructured":"Hagenfeldt L, von Dobeln U, Holme E, et al. 3-Hydroxydicarboxylic aciduria-a fatty acid oxidation defect with a severe prognosis. J Pediatr.1990;116:387\u2013392","DOI":"10.1016\/S0022-3476(05)82826-4"},{"key":"2021121023241470400_R22","doi-asserted-by":"crossref","unstructured":"Wanders RJA, Vreken P, den Boer MEJ, Wijburg FA, van Gennip AH, IJlst L. Disorders of mitochondrial fatty acyl-CoA \u03b2-oxidation. J Inherit Metab Dis.1999;22:442\u2013487","DOI":"10.1023\/A:1005504223140"},{"key":"2021121023241470400_R23","doi-asserted-by":"crossref","unstructured":"Brivet M, Boutron A, Slama A, et al. Defects in activation and transport of fatty acids. J Inherit Metab Dis.1999;22:428\u2013441","DOI":"10.1023\/A:1005552106301"},{"key":"2021121023241470400_R24","doi-asserted-by":"crossref","unstructured":"Corr PB, Creer MH, Yamada KA, Sobel BE. Prophylaxis of early ventricular fibrillation by inhibition of acylcarnitine accumulation. J Clin Invest.1989;83:927\u2013936","DOI":"10.1172\/JCI113978"},{"key":"2021121023241470400_R25","doi-asserted-by":"crossref","unstructured":"Touma EH, Charpentier C. Medium chain acyl-CoA dehydrogenase deficiency. Arch Dis Child.1992;67:142\u2013145","DOI":"10.1136\/adc.67.1.142"},{"key":"2021121023241470400_R26","doi-asserted-by":"crossref","unstructured":"Iafolla AK, Thompson RJ, Roe CR. Medium-chain acyl-CoA dehydrogenase deficiency: clinical course in 120 affected children. J Pediatr.1994;124:409\u201315","DOI":"10.1016\/S0022-3476(94)70363-9"},{"key":"2021121023241470400_R27","doi-asserted-by":"crossref","unstructured":"Pollit RJ, Leonard JV. Prospective surveillance study of medium chain acyl-CoA dehydrogenase deficiency in the UK. Arch Dis Child.1998;79:116\u2013119","DOI":"10.1136\/adc.79.2.116"},{"key":"2021121023241470400_R28","doi-asserted-by":"crossref","unstructured":"Vianey-Saban C, Divry P, Brivet M, et al. Mitochondrial very-long-chain acyl-coenzyme A dehydrogenase deficiency: clinical characteristics and diagnostic considerations in 30 patients. Clin Chim Acta.1998;269:43\u201362","DOI":"10.1016\/S0009-8981(97)00185-X"},{"key":"2021121023241470400_R29","doi-asserted-by":"crossref","unstructured":"Tyni T, Majander A, Kalimo H, Rapola J, Pihko H. Pathology of skeletal muscle and impaired respiratory chain function in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with the G1528C mutation. Neuromuscul Disord.1996;6:327\u2013337","DOI":"10.1016\/0960-8966(96)00352-5"},{"key":"2021121023241470400_R30","doi-asserted-by":"crossref","unstructured":"Treem WR, Rinaldo P, Hale DE, et al. acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-CoA deficiency. Hepatology.1994;9:339\u2013345","DOI":"10.1016\/0270-9139(94)90010-8"},{"key":"2021121023241470400_R31","doi-asserted-by":"crossref","unstructured":"Moore R, Glasgow JFT, Bingham MA, et al. Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency: diagnosis, plasma carnitine fractions and management in a further patient. Eur J Pediatr.1993;152:433\u2013436","DOI":"10.1007\/BF01955905"},{"key":"2021121023241470400_R32","doi-asserted-by":"crossref","unstructured":"Duran M, Wanders RJA, Jager JP de, et al. 3-Hydroxydicarboxylic aciduria due to long chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency associated with sudden neonatal death: protective effect of medium-chain triglyceride treatment. Eur J Pediatr.1990;50:190\u2013195","DOI":"10.1007\/BF01963564"},{"key":"2021121023241470400_R33","doi-asserted-by":"crossref","unstructured":"Vaartjes WJ, Kemp A, Jr, Souverijn JHM, Van den Bergh. Inhibition of fatty acyl esters of adenine nucleotide translocation in rat-liver mitochondria. FEBS Lett.1972;23:303\u2013308","DOI":"10.1016\/0014-5793(72)80302-8"},{"key":"2021121023241470400_R34","doi-asserted-by":"crossref","unstructured":"Harris RA, Farmer B, Ozawa T. Inhibition of the mitochondrial adenosine nucleotide transport by oleyl CoA. Arch Biochem Biophys.1972;150:199\u2013209","DOI":"10.1016\/0003-9861(72)90027-6"},{"key":"2021121023241470400_R35","doi-asserted-by":"crossref","unstructured":"Halperin ML, Robinson BH, Fritz IB. Effects of palmitoyl CoA on citrate and malate transport by rat liver mitochondria. Proc Natl Acad Sci U S A.1972;69:1003\u20131007","DOI":"10.1073\/pnas.69.4.1003"},{"key":"2021121023241470400_R36","doi-asserted-by":"crossref","unstructured":"Morel F, Laquin G, Lunardi J, Duszynski J, Vignais PV. An appraisal of the functional significance of the inhibitory effect of long chain acyl-CoAs on mitochondrial transports. FEBS Lett.1974;39:133\u2013138","DOI":"10.1016\/0014-5793(74)80035-9"},{"key":"2021121023241470400_R37","doi-asserted-by":"crossref","unstructured":"Ventura FV, Ruiter JPN, IJlst L, Tavares de Almeida I, Wanders RJA. Inhibitory effect of 3-hydroxyacyl-CoAs and other long-chain fatty acid \u03b2-oxidation intermediates on mitochondrial oxidative phosphorylation. J Inherit Metab Dis.1995;19:161\u2013164","DOI":"10.1007\/BF01799419"},{"key":"2021121023241470400_R38","doi-asserted-by":"crossref","unstructured":"Moore KH, Dandurand DM, Kiechle FL. Fasting induced alterations in mitochondrial palmitoyl-CoA metabolism may inhibit adipocyte pyruvate dehydrogenase activity. Int J Biochem.1992;24:809\u2013814","DOI":"10.1016\/0020-711X(92)90017-U"},{"key":"2021121023241470400_R39","doi-asserted-by":"crossref","unstructured":"Sims HF, Brackett JC, Powell CK, et al. The molecular basis of pediatric long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with maternal acute fatty liver of pregnancy. Proc Natl Acad Sci U S A.1995;92:841\u2013845","DOI":"10.1073\/pnas.92.3.841"},{"key":"2021121023241470400_R40","doi-asserted-by":"crossref","unstructured":"Schoeman MN, Batey RG, Wilcken B. Recurrent acute fatty liver of pregnancy associated with fatty-acid oxidation defect in the offspring. Gastroenterology.1991;100:544\u2013548","DOI":"10.1016\/0016-5085(91)90228-D"},{"key":"2021121023241470400_R41","unstructured":"Treem WR, Shoup ME, Hale DE, et al. Acute fatty liver of pregnancy, hemolysis, elevated liver enzymes, and low platelets syndrome and long chain 3-hydroxyacyl-Coenzyme A dehydrogenase deficiency. Am J Gastroenterol.1996;91:2293\u20132300"},{"key":"2021121023241470400_R42","doi-asserted-by":"crossref","unstructured":"Wilcken B, Leung K, Hammond J, Kamath R, Leonard JV. Pregnancy and fetal long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Lancet.1993;341:407\u2013408","DOI":"10.1016\/0140-6736(93)92993-4"},{"key":"2021121023241470400_R43","doi-asserted-by":"crossref","unstructured":"Ibdah JA, Dasouki MJ, Strauss AW. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: variable expressivity of maternal illness during pregnancy and unusual presentation with infantile cholestasis and hypocalcaemia. J Inherit Metab Dis.1999;22:811\u2013814","DOI":"10.1023\/A:1005506024055"},{"key":"2021121023241470400_R44","doi-asserted-by":"crossref","unstructured":"Ibdah JA, Bennett MJ, Rinaldo P, et al. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N Engl J Med.1999;340:1723\u20131731","DOI":"10.1056\/NEJM199906033402204"},{"key":"2021121023241470400_R45","doi-asserted-by":"crossref","unstructured":"Strauss AW, Bennett MJ, Rinaldo P, et al. Inherited long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and a fetal-maternal interaction cause maternal liver disease and other pregnancy complications. Semin Perinatol.1999;23:110\u2013112","DOI":"10.1016\/S0146-0005(99)80044-5"},{"key":"2021121023241470400_R46","doi-asserted-by":"crossref","unstructured":"Tyni T, Ekholm E, Pihko H. Pregnancy complications are frequent in long-chain hydroxyacyl-CoA dehydrogenase deficiency. Am J Obstet Gynecol.1998;178:603\u2013608","DOI":"10.1016\/S0002-9378(98)70446-6"},{"key":"2021121023241470400_R47","doi-asserted-by":"crossref","unstructured":"Bartelds B, Gratema JW, Knoester H, et al. Perinatal changes in myocardial supply and flux of fatty, acids, carbohydrates, and ketone bodies in lambs. Am J Physiol.1998;274:H1962\u2013H1968","DOI":"10.1152\/ajpheart.1998.274.6.H1962"},{"key":"2021121023241470400_R48","doi-asserted-by":"crossref","unstructured":"Bartelds B, Knoester H, Beaufort-Krol GC, et al. Myocardial lactate metabolism in fetal and newborn lambs. Circulation.1999;99:1892\u20131897","DOI":"10.1161\/01.CIR.99.14.1892"},{"key":"2021121023241470400_R49","unstructured":"Jones CT. The development of the metabolism in fetal liver. In: Biochemical Development of the Fetus and Neonate. Amsterdam, the Netherlands: Elsevier; 1992: 249"},{"key":"2021121023241470400_R50","doi-asserted-by":"crossref","unstructured":"Walter JH. Inborn errors and pregnancy. J Inherit Metab Dis.2000;23:229\u2013236","DOI":"10.1023\/A:1005679928521"},{"key":"2021121023241470400_R51","unstructured":"Chakrapani A, Olpin S, Till J, et al. Clinical and biochemical features of trifunctional protein deficiency. J Inherit Metab Dis.2000;3(suppl 1):129"},{"key":"2021121023241470400_R52","doi-asserted-by":"crossref","unstructured":"Innes AM, Seargeant LE, Balachandra K, et al. Hepatic carnitine Palmitoyltransferase I deficiency presenting as maternal illness in pregnancy. Pediatr Res.2000;47:43\u201345","DOI":"10.1203\/00006450-200001000-00010"},{"key":"2021121023241470400_R53","doi-asserted-by":"crossref","unstructured":"Nelson J, Lewis B, Walters B. The HELLP syndrome associated with fetal MCAD deficiency. J Inherit Metab Dis.2000;3:518\u2013519","DOI":"10.1023\/A:1005676600975"},{"key":"2021121023241470400_R54","unstructured":"Matern D, Murtha AP, Vockley G, Gregersen N, Millington DS, Treem WR. Broadening the spectrum of fetal fatty acid \u03b2-oxidation disorders causing liver disease in pregnant women. Am J Hum Genet.1999;65:A45"},{"key":"2021121023241470400_R55","doi-asserted-by":"crossref","unstructured":"Rocchiccioli F, Wanders RJA, Aubourg P, et al. Deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase: a cause of lethal myopathy and cardiomyopathy in early childhood. Pediatr Res.1990;28:657\u2013662","DOI":"10.1203\/00006450-199012000-00023"},{"key":"2021121023241470400_R56","doi-asserted-by":"crossref","unstructured":"Tyni T, Kivela\u0308 T, Lappi M, Summanen P, Nikoskelainen E, Pihko H. Ophthalmologic findings in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation. Ophthalmology.1998;105:810\u2013824","DOI":"10.1016\/S0161-6420(98)95019-9"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/109\/1\/99\/986963\/99.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/109\/1\/99\/986963\/99.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,11]],"date-time":"2021-12-11T07:59:52Z","timestamp":1639209592000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/109\/1\/99\/79785\/Long-Chain-3-Hydroxyacyl-CoA-Dehydrogenase"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2002,1,1]]},"references-count":56,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2002,1,1]]},"published-print":{"date-parts":[[2002,1,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.109.1.99","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2002,1,1]]}}}