Figure 1From: Alkaptonuria diagnosed in a 4-month-old baby girl: a case reportIntermediates in Tyrosine metabolism is shown. Homogentisate oxidase1 which converts thomogentisate to maleyl acetoacetate is deficient in alkaptonuria. 4-Hydroxy phenyl pyruvate Hydroxylase2 can be blocked by the newer drug Nitisinone. In that case there is definite rise of phenylalanine and tyrosine level in blood which is harmful. The drug is not recommended till date for pediatric use.Back to article page