If urine sample darkens on standing: the most likely conditions is ?
Ans. is 'b' i.e., AlkaptonuriaAlkaptonuriaIt is due to deficiency of homogentisate oxidase. As a result homogentisic acid (homogentisate) is excreted execessively in urine. There arc three impoant characteristic features in alkaptonuria?i) Urine becomes dark after being exposed to air. It is due spontaneous oxidation of homogentisate into benzoquinone acetate, which polymerse to form black-brown pigment alkapton which impas a characteristic black-brown colour to urine.ii) Alkapton deposition occurs in sclera, ear, nose, cheeks and interveebral disc space. A condition called ochronosis. There may be calcification of interveebral discs.iii) Onchronosis ahritis affecting shoulder, hips, knee.Benedict's test is strongly positive in urine and so is the ferric chloride (FeC1 3)test. Benedict's reagent gives a greenish brown precipitate with brownish black supernatent. Fehling's reagent (FeC13) gives blue green colour.
Phenylketonuria
Alkaptonuria
Maple syrup disease
Tyrosinemia