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Infants with severe metabolic diseases like PKU and MSUD should use different formulas instead of regular foods or breast milk.
Infants with severe metabolic diseases like PKU and MSUD should use different formulas instead of regular foods or breast milk.

Database for Metabolic Diseases in Infants

From among every 600 infants in Iran, one suffers from inherited metabolic diseases

Database for Metabolic Diseases in Infants

A comprehensive ‘Data Registration System’ has been developed for the first time in the country where all data on infants diagnosed with metabolic disorders as well as information on the dietary products they require, will be registered.
The database will be officially unveiled on April 22, during the National Health Week, observed April 20-26, IRNA reported.
Metabolic disorders are genetic diseases that interfere with the body’s ability to process specific substances. Often, the body is missing an enzyme that is needed to process a certain type of amino acid.
“As a result, these acids can build up in the body causing problems like intellectual disability and other serious health problems,” said Amir Hussein Jamshidi, director general of the Health Ministry’s Office for Overseeing Natural, Traditional and Supplementary Products at the Food and Drug Administration (FDA), on Sunday.
“The comprehensive data system can help us to better plan infant formula imports,” he said.
“From among every 600 infants in Iran, one suffers from inherited metabolic diseases (2,500 infants per year),” he noted.
Some infants with severe diseases like Phenylketonuria (PKU) a genetic condition requiring lifelong management, and Maple syrup urine disease (MSUD), a rare inherited metabolic disorder, should use different formulas and other essential products instead of regular foods or breast milk.
As an example, infants with PKU should consume PKU formula which does not contain any phenylalanine. The formula is nutritionally complete and contains all the necessary vitamins, minerals, and other essential amino acids needed for an infant’s normal growth and development.

  Special Products
Children with the disease also should consume special products, synthetically made as a nutritional substitute for foods which they can’t have such as milk, dairy products, meat, fish, chicken, eggs, and beans in order to have normal growth.  Consuming such foods causes high blood Phe (phenylalanine) levels for people with PKU which can cause problems in the way they think, feel, and act.
In children, symptoms of metabolic disorders may include being cranky or fretful; inability to focus; anxiety and severe intellectual disability (if left untreated).
In adults, symptoms include: headache, forgetfulness, fatigue, trouble focusing, depression and anxiety.
Stating that that latest technologies are needed for manufacturing products for the patients, he said, “The foods are expensive and produced in very few countries. Therefore the products should be ordered and imported one year ahead,” to meet domestic demand.
Since 2007, a plan to identify patients with metabolic disorders including PKU was developed and implemented in the country. Now information about all patients born after 2007 has been registered in the system and the measure will facilitate accurate and timely supply of products for patients, by the FDA.
Metabolic diseases, specifically PKU and MSUD, are serious disorders that are life-threatening unless addressed promptly. Careful monitoring and adherence to dietary restrictions are essential.
When a strict diet is initiated early and maintained well, affected infants and children can expect normal development and a normal life span. It is also necessary to control blood Phe levels every day. Treatment should start at birth and continue for life. 

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