Final answer:
Hereditary Fructose Intolerance (HFI) is a genetic disorder that results from deficient activity of the enzyme aldolase-B, leading to problems metabolizing fructose and necessitating a fructose-restricted diet to manage the condition.
Step-by-step explanation:
Hereditary Fructose Intolerance (HFI)
Hereditary Fructose Intolerance, often referred to as HFI, is a metabolic disorder caused by the absence or deficient activity of the enzyme aldolase-B. Unlike fructosuria, which involves the excretion of fructose in the urine due to faults in fructokinase, or G6PD deficiency, which is a broader condition affecting red blood cells, HFI specifically prevents the proper metabolism of fructose once ingested. Individuals with fructose intolerance generally display a natural aversion to fructose and foods containing fructose because their bodies lack the means to process it. This condition is an inherited autosomal recessive trait and can lead to severe health complications if one consumes fructose without being aware of the intolerance. It is imperative to identify HFI early to manage dietary intake and prevent potential damage from unmetabolized fructose in the body.