Galactosemia dietary restriction

Jakobs C, Kleijer WJ, Bakker HD, Van Gennip A, Przyrembel H, Niermeijer ME. Dietary restriction of maternal lactose intake does not prevent accumulation of galactitol in the amniotic fluid of fetuses affected with galactosemia. Prenat Diagn 1988 8 641-645. 

Despite the dietary restriction of galactose, individuals with galactosemia have elevated concentrations of galactose metabolites in blood and urine. This phenomenon is believed to be due, in part, to endogenous production of galactose (i.e., galactose that is produced by the body) (Fig. 25.2). 

Once diagnosed, galactosemia can be treated by restricting dietary galactose, especially by excluding lactose from infant formulas. 

Because patients with galactosemia need to avoid weight loss as a result of restrictive dietary measures, the authors suggested that this is an interesting example of weight gain as a positive side effect of clozapine, not necessarily associated with increased appetite and higher caloric intake. 

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