Inborn errors of metabolism: the need for sugar.

Inborn errors of metabolism are conditions in which the body fails to 
metabolise certain chemicals due to an inherited enzyme deficiency. The inborn 
errors of metabolism considered in this paper are those which affect 
the metabolism of dietary nutrients and which require manipulation of the 
carbohydrate intake. In these cases the need for sugar - either 
as a source of energy when other energy-supplying nutrients have to 
be suppressed, or for the control of hypoglycaemia - is vitally 
important. Inborn errors of protein metabolism include phenylketonuria, maple-syrup urine disease, 
organic acidaemias, and hyperammonaemia. Inborn errors of fat metabolism include abetalipoprotinaemia, 
type I lipoprotein-lipase deficiency, and lymphangiectasia. Inborn errors of carbohydrate metabolism 
include diabetes, galactosaemia, fructosaemia, and glucose-6-phosphate deficiency (type I glycogen storage 
disease). Inborn errors of multiple nutrients include cystic fibrosis. Dietary carbohydrate, 
psychological considerations, and dental caries are examined. 