<prompt> The enzyme deficient in Galactosemia is:Hexoseaminidase BHexosaminidase AGalactose 1 phosphate uridyltransferaseGlucocerebrosidase  <response>: Ref. Textbook of Biochemistry for Medical Students. Page. 183Galactosemia is an autosomal recessive trait that results from a defective gene encoding either galactokinase or galactose 1-P uridyltransferase. There are over 100 heritable mutations that can cause galactosemia, and the incidence is approximately 1 in 60,000 births.Galactose will be present in elevated amounts in the blood and urine and can result in decreased glucose synthesis and hypoglycemia.Galactosemia symptoms often begin around day 3 in a newborn and include the hallmark cataracts.Jaundice and hyperbilirubinemia do not resolve if the infant is treated with phototherapySevere bacterial infections E.coli sepsis are common in untreated galactosemic infants. Failure to thrive, lethargy, hypotonia, and mental retardation are other common and apparent features 