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The main clinical presentation of this metabolic disease is different from other inherited defects of fatty acid \u03b2\u2212oxidation as the hypoglycemia is associated with hyperinsulinism. We present the clinical, biochemical and molecular findings of four new Caucasian patients with HADH deficiency. These new cases contribute to a more comprehensive description of the phenotype, diagnostic biomarkers and treatment options for this poorly defined disease.<\/jats:p>","DOI":"10.1007\/s10545-011-9287-7","type":"journal-article","created":{"date-parts":[[2011,2,23]],"date-time":"2011-02-23T06:56:37Z","timestamp":1298444197000},"page":"835-842","source":"Crossref","is-referenced-by-count":25,"title":["Short\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency: the clinical relevance of an early diagnosis and report of four new cases"],"prefix":"10.1002","volume":"34","author":[{"given":"Esmeralda","family":"Martins","sequence":"first","affiliation":[{"name":"Unidade de Doen\u00e7as Metab\u00f3licas Hospital de Crian\u00e7as Maria Pia Rua da Boavista, 827 Porto 4050\u2010111 Portugal"}]},{"given":"M. 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