Pediatrics in review
-
Many inborn errors of intermediary metabolism present during periods of poor oral intake or intercurrent illness. Under these conditions, the body switches to a predominantly catabolic state, when glycogen, fat, and protein are mobilized as alternative energy sources. If a genetic defect already exists in any of these catabolic pathways, the problem is accentuated because the catabolic state demands a high flux of metabolite through these pathways. ⋯ A partial list of more commonly encountered IEMs is contained in the Table. The different parts of the biochemical pathways discussed previously are combined into Figure 6. Hopefully, for some readers, the Figure also will lead to the affirmation that biochemistry is not so bad after all!