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Conditions for which this effect has been demonstrated are: glutaric aciduria II, methyl malonic aciduria, propionic acidemia, and medium chain fatty acylCoA dehydrogenase deficiency.7,8 Autointoxication occurs in these patients due to the accumulation of acylCoA compounds that disrupt intermediary metabolism
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However, none of the patients enrolled in this study was overweight (BMI = 17.0 4.4 kg/m 2 ), and the lean body mass did not differ between the congenital hypothyroid patients and the healthy controls (data not shown), suggesting that the hepatocytes might be the primary site of TG deposition in these patients
