After speaking of lactose intolerance, I find it a comment on another disease closely linked to dairy products, but much less known: The galactosemia. This is a disease that is characterized by the unusual presence of galactose in the blood after taking foods containing lactose and / or galactose. It can be caused by three different genetic defects of enzymes in the metabolism of galactose: The galactosemia (galactose or severe, characterized by a deficiency of galactose-1-phosphate uridiltransferasi), galactosemia II (due to deficiency of galactokinase), and finally, galactosemia III (due to deficiency of UDP-galactose-4-epimerase in red and white).
Galactosemia serious if left untreated, leads to a reduction of the potentially fatal kidney and liver function, enlarged liver, cirrhosis of the liver, and brain damage in newborns, cataracts. Galactokinase deficiency (galactosemia II), but generally is associated with cataracts and mental retardation, but this phenomenon is not certain that can be linked directly to the enzyme deficiency. Finally, galactosemia III is characterized by symptoms similar to The form, when the enzyme deficiency is widespread. Conversely, when the deficit is peripheral or isolated, the disease is asymptomatic.
Regarding the diagnosis, this can be done by evaluating the blood concentration of galactose (galactosemia I and II), or by enzymatic test on red blood cells (galactosemia III), assuming that the subject has not been transfusions. The diagnosis can be confirmed by high levels of galactose or galactitolo urine.
Clinical management of all types of galactosemia include, where possible, eliminating all sources of galactose, including human milk. In addition to dairy products, a potential source of this sugar is also represented by various fruits (blueberries, winter melon, pineapple), the vegetable and meat stuffed and preserved. Unfortunately, even in the event of early dietary management, most patients will experience delayed development and a deficit in growth. Foods that contain milk (or lactose as an ingredient) and dairy products should be avoided as far as possible, so that the daily total intake of lactose is at least 25 mg/100 kcal. The current test to indicate how "the absence of lactose content of less than 10 mg kcal lattosio/100 on labels of products for infants and food formulas, which allows these formulas can be safely used for the dietary management of patients with galactosemia. A precise threshold for galactose of lactose intake below which adverse effects can be excluded is not never been established. The milk (or drink), in which lactose has been enzymatically hydrolyzed to glucose and galactose (milk type HD) is not recommended for patients with galactosemia, unless the galactose then is not completely removed.
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