A 25-year-old male presented to the OPD with complaints of pain in B/L knee, hip joints and lower spine. On examination, the joints were found to be erythematous and tender. Note was also made of dark black spots on sclera and ear cailage. X-ray of the affected joints were taken. Which of the following drug has been approved for the above condition: –
Correct Answer: Nitisinone
Description: This is a case of Alkaptonuria. 1st image shows ochronosis. 2nd and 3rd images show the narrowing of the joint spaces and calcification of the interveebral discs- classical of alkaptonuric ahritis It is caused by deficiency of homogentisic acid oxidase (homogentisate 1,2-dioxigenase). In alkaptonuria, large amounts of homogentisic acid are formed which are excreted in urine or deposited in tissues. Nitisinone is a synthetic reversible inhibitor of 4-hydroxyphenylpyruvate dioxygenase. Nitisinone efficiently reduces homogentisic acid production in alkaptonuria. If presymptomatic individuals are detected, treatment with Nitisinone, combined with a phenylalanine- and tyrosine-restricted diet can be used.
Category:
Anatomy
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