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Significant adaptation of mitochondrial\/energy\u2010, carbohydrate\u2010, lysosomal\u2010, and amino acid\u2010metabolism occurs during early prenatal and neonatal development. In utero, metabolic environment has an impact on the development of the fetus as well as fetal organ maturation. Defects of distinct metabolic pathways could therefore already be of significant relevance in utero and for clinical manifestations in the early fetal and neonatal period. Disturbances of these pathways may influence intrauterine growth and health. Production of a toxic intrauterine milieu, energy\u2010deficiency, modification of membrane function, or disturbance of the normal intrauterine expression of genes may be responsible for fetal compromise and developmental disorders. Three categories of metabolic disorders will be discussed: the \u201cintoxication type\u201d (classical galactosemia, ornithine transcarbamylase deficiency, and \u201cmaternal phenylketonuria\u201d), the \u201cstorage type\u201d (Morbus Niemann Pick type C), and the \u201cenergy deficient type\u201d (including long\u2010chain fatty acid oxidation disorders, pyruvate dehydrogenase deficiency, and respiratory chain defects). For these disorders, the pathophysiology of early manifestation, special aspects regarding the prenatal and neonatal period, and diagnostic as well as therapeutic options are presented. \u00a9 2010 IUBMB IUBMB Life, 62(6): 403\u2013413, 2010<\/jats:p>","DOI":"10.1002\/iub.336","type":"journal-article","created":{"date-parts":[[2010,4,27]],"date-time":"2010-04-27T23:19:08Z","timestamp":1272410348000},"page":"403-413","source":"Crossref","is-referenced-by-count":41,"title":["Impact of selected inborn errors of metabolism on prenatal and neonatal development"],"prefix":"10.1002","volume":"62","author":[{"given":"Sabine","family":"Illsinger","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anibh M.","family":"Das","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2010,4,26]]},"reference":[{"key":"e_1_2_4_2_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.56.10.1265"},{"key":"e_1_2_4_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clp.2009.06.004"},{"key":"e_1_2_4_4_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1095292"},{"key":"e_1_2_4_5_2","first-page":"156","article-title":"Principles and practice of Screening for Disease","volume":"34","author":"Wilson J. 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