PAH and hyperinsulinemic hypoglycemia, familial, 4: This finding is not surprising given that high intake of Phe in the context of Pah deficiency increases the synthesis of several weak organic acids, including phenyllactate and phenylacetate, which must be excreted to maintain acid–base balance (Lemann et al. 2003; Hennermann et al. 2013; Schuck et al. 2015).