Define the following inborn errors of metabolism and also give dietary management of these diseases: 

i) Tyrosinemia

 ii) Homocystinuria 

iii) Maple Syrup Urine Disease 

b) Name the enzyme whose deficiency causes PKU. 

i) Tyrosinemia

Ans:- Tyrosinemia or tyrosinaemia is an error of metabolism, usually inborn, in which the body cannot effectively break down the amino acid tyrosine. Symptoms of untreated tyrosinemia include liver and kidney disturbances. Without treatment, tyrosinemia leads to liver failure.

The treatment for tyrosinemia is a combination of a low-protein diet and a drug called Nitisinone. Nitisinone prevents the build up of toxic breakdown products. Meats, dairy products, and other protein rich foods such as nuts and beans should be avoided. Good nutrition and adequate vitamin and mineral intake allow children to grow normally. 

 ii) Homocystinuria

Ans:- Homocystinuria is an autosomal recessive inborn error of amino acid metabolism that results in inability to break down homocysteine to cystathionine due to deficiency in the enzyme __________ __________ ___ __________ _________.
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