Question

In: Chemistry

Congenital Galactosemia typically appears only in infants because with maturation, most children develop another enzyme capable...

Congenital Galactosemia typically appears only in infants because with maturation, most children develop another enzyme capable of metabolizing galactose. Its incidence is about 1 per 18,000 births. If the infant does not receive treatment then there is a 75% chance of death. Thus, in the United States infants are screened for galactosemia. Galactosemia can be confused with lactose intolerance but galactosemia is a considerably more serious condition because consumption of galactose can cause permanent damage to their bodies. What dietary changes should be made?

Solutions

Expert Solution

Galactosemia is caused due to the missing of an enzyme called galactose-1-phosphate uridyl transferase (GALT). This enzyme changes galactose/lactose into glucose. So due to the absence of this enzyme there is an excess of galactose in the body which can cause damage to the liver, eyes, kidneys, and brain. It can also be life threatening for the infants. Symptoms of it in infants are

  • Poor feeding
  • Lethargy
  • jaundice
  • poor weight gain
  • vomiting

On the other hand, lactose intolerance is inability to digest lactose. it is usually caused by the deficiency of lactase enzyme.

Difference between galactosemia and lactose intolerance is that Galactosemia is life threatening but lactose intolerance is not. When galactosemia is left untreated it cause brain damage, speech difficulty and other reproductive problems. Whereas when lactose intolerance is left untreated it causes diarrhea,bloating and cramping of intestine.

Dietary changes in case of galactosemia-

Galactosemia can not be cured but we can make it under control by a milk free diet. Infants are not allowed to have food containing lactose/galactose such as breast or regular milk, instead of it they can have soy milk. Food which must be avoided:

  • milk and milk products
  •   cheese
  • fats like butter, vegetable spreads
  • sauces like white sauce, butter sauce, cheese sauce
  • bread
  • desserts like puddings, custard made with milk,ice cream, pancakes
  • beverages like Bournvita, Horlicks

Food suitable for people having galactosemia-

Person on galactose restricted diet should have protein containing food like beef,poultry and eggs.Instead of regular or breast milk , soy milk,oat milk, nut milks can be given to the infants. Diet enriched with vegetables and fruits is considered to be agood . As one having galactosemia can not consume milk products so their calcium level can become low so to compensate for that one should take calcium and vitamin D supplements. Cheese like Gruyere, Italian Parmesan, French Comte are suitable for people with galactosemia. These are vey good source of calcium.

Home made bread with milk free ingredients is a safe option.


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