HN-01010049_Phenylketonuria: Challenges of Food Production for PKU Patients in Iran

Introduction: Phenylketonuria (PKU) is an inherited metabolic disorder due to lack of phenylalanine (Phe) hydroxylase enzyme (PAH), which converts Phe to tyrosine. PKU resulted in brain damage and microcephaly. Untreated patients developed serious mental disorders. The utilization of a low Phe diet could inhibited mentioned disorders. The goal of this study to assess the regulation status of food Production for PKU Patients in Iran for PKU production.Methods: The whole regulations of food and drug administration of Iran and Institute of Standards and Industrial Research of Iran regarding PKU food production were investigated and compared with regulations in other countries.Results: Our finding indicated there are approximately 7000 PKU patients in Iran. There is sufficient food for these patients in Iran. The most important problem for these patients is the lack of access to low Phe food. The number of PKU food produces in Iran was very low. The Food and Drug Administration does not have any specific regulations regarding the definition and production of these foods and no standards have been established in Iran for these products. Because of this, it was very worrying and the contractors would not be able to produce these products under the current conditions.Conclusion: In general, regulation and standard establishment for low-Phe food production are inevitable. The support of the manufacturer is a necessity. By producing and access to low-Phe food, the further disorders of the disease have been reduced and patients enjoy a life similar to normal people in the community.

کلیدواژه ها:

Phenylketonuria ، Food industry ، nutrition ، new strategies