Boiled cabbage or rancid butter smelling urine is seen in
Correct Answer: Tyrosinemia
Description: Tyrosinemia REF: Sapira's a & science of bedside diagnosis - Page 138, Textbook of Pediatric Emergency Medicine by Gary R. Fleisher, Stephen Ludwig Page 1566 Tyrosinaemia is an error of metabolism, inherited, in which the body cannot effectively break down the amino acid tyrosine, found in most animal and plant proteins. It is an autosomal recessive, which means two copies of an abnormal gene must be present in order for this to develop. There are three types of tyrosinemia, each with distinctive symptoms and caused by the deficiency of a different enzyme. One of the symptoms of Tyrodinaemia type 1 is an odor like cabbage or rancid butter.
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Biochemistry
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