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Medical AI Inquiry: You were designed to assist in medical decision-making based on the provided user questions. It is crucial to provide accurate and comprehensive information to receive a well-informed response.
user
A child brought with seizures and impairment of development is diagnosed having phenylketonuria. What is the initial line of treatment in this child?
assistant
The correct answer is  Phenylketonuria: It is an autosomal recessive metabolic disorder, where the level of hepatic enzyme phenylalanine hydroxylase is low, therefore phenylalanine cannot be conveed to tyrosine. Phenylalanine accumulates in the blood, cerebrospinal fluid and tissues. Accessory metabolic pathways sta operating which conve phenylalanine to phenylpyruvic acid, phenyl-lactic acid and o-hydroxyphenyl-acetic acid. Restriction of phenylalanine in the diet is the mainstay of treatment of phenylketonuria. Ref: Essential Pediatrics by O.P. Ghai, 6th edition, Page 609.