Urinalysis and Body Fluids 6th Edition by Majorie DiLorenzo, Susan Strasinger

Exercise 30

Hurler, Hunter, and Sanfilippo syndromes are hereditary disorders affecting metabolism of:

A. Porphyrins

B. Purines

C. Mucopolysaccharides

D. Tryptophan

Step 1 of 2

Mucopolysaccharide disorders:

Inherited disorder of Mucopolysaccharide metabolism prevent whole breakdown of polysaccharide portion, which leads to incompletely metabolized part of polysaccharide accumulation in lysosome present in cells of connective tissue, and increased urine excretion of polysaccharide.

• Hurler syndrome, sanfilippo syndrome, and hunter syndrome are hereditary disorders associated with Mucopolysaccharide metabolism

• In hurler, and hunter syndromes abnormal skeletal structure, and severe mental retardation is found

• In hurler syndrome, accumulation of mucopolysaccharide in cornea of eye is seen

• In sanfilippo syndrome mental retardation is found

Therefore, Hurler syndrome, sanfilippo syndrome, hunter syndrome are hereditary disorders affecting “Mucopolysaccharides” metabolism. Hence, the correct answer is option.