If urine sample darkens on standing: the most likely conditions is -
Ans. is 'b' Le.t Alkaptonuria Alkaptonuriao It is due to deficiency of homogentisate oxidase* As a result homogentisic acid (homogentisate) is excreted execessively in urine. There are three important characteristic features in alkaptonuria-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 imparts a characteristic black-brown colour to urine.Alkapton deposition occurs in sclera, ear. nose, cheeks and intervertebral disc space. A condition called ochronosis. There may be calcification of intervertebral discs.Onchronosis arthritis affecting shoulder, hips, knee.o Benedict's test is strongly positive in urine and so is the ferric chloride (FeCl3) test. Benedict's reagent gives a greenish brown precipitate with brownish black supematent. Fehling's reagent (FeCl3 gives blue green colour.
Phenylketonuria
Alkaptonuria
Maple syrup disease
Tyrosinemia
Amino Acid Metabolic Disorder