Phenylketonuria (PKU) is a rare inherited disease that prevents you from properly assimilating proteins. To facilitate patients, who must follow a very strict diet, the reference associations in collaboration with the restaurateurs have developed safe recipes to be found in the places that adhere to the initiative.
No banned cured meats, meat and fish, dairy products weighed with the slingbar: the choice of a dish at the restaurant is really difficult for those suffering from phenylketonuria (PKU), a rare hereditary disease that in Italy affects one child for every 3 thousand births and which is characterized by reduced or no activity of the enzyme that has the task of transforming a particular amino acid, phenylalanine, which consequently accumulates in the blood and nervous system with toxic effects. From today, these patients too will have the opportunity to find recipes at the restaurant specially designed for them by a team of specialists, validated by a chef. Since the phenylalanine contained in proteins, those suffering from the disease must drastically limit their intake, under penalty of extremely severe consequences, especially if no action is taken immediately after birth.
The diagnosis
The accumulation of the amino acid in the blood and brain associated with progressive intellectual deterioration and a range of additional symptoms, which can include eczematous rush, autism, seizures and motor deficits explains Elvira Verduci, pediatrician at the Vittore Buzzi University Children’s Hospital. Milan with the growth of the child often also appear problems in development, behavioral disorders and psychiatric symptoms.
The disease is identified thanks to a screening test carried out at birth; once the disease is diagnosed, patients with phenylketonuria are immediately placed on a diet. The goal of the diet is to keep the levels of phenylanine in the blood around normal values with an intervention based on three fundamental principles, recalls the dietician Fina Belli, abolition of natural proteins, use of foods for special medical purposes and integration of protein intake with mixtures of amino acids. In addition to the exclusion of products of animal origin, cereals, legumes and nuts, the diet includes the use of protein-free foods for special medical purposes such as pasta, bread, biscuits, and mixtures of amino acids free of phenylalanine and enriched with minerals and vitamins. Following this diet, children with PKU undergo absolutely normal development.
The diet
If the greatest damage of the excess of phenylalanine affects children, adults who interrupt the diet for prolonged periods of time can also experience problems and consequently the diet must be continued for life. This is not always easy, especially when the meal is eaten away from home. In the case of children, it is generally the parents who bring the protein-free foods for their children, for example the protein-free pizza bases, while the adult generally tends to adjust to what is in the restaurant, remembers Niko Costantino, patient and representative of the ‘Association for the Study of Hereditary Metabolic Diseases ODV This means that most of the time he ends up eating a plate of vegetables.
The initiative
The PKUISINE project, PKU friendly kitchen, created thanks to the contribution of PIAM Medifood, with the support of FIPE (Italian Federation of Public Exercises), various patient associations, scientific societies (SIMMESN) and a board of specialists, wants be an answer to these needs. It is a unique initiative of its kind in which a team of experts has created guidelines and a recipe book that collects specially developed recipes. In practice, the businesses participating in the project will include in their menus some dishes made according to recipes that include protein-free foods that are easily manageable by the individual restaurateur and the object of a first free supply.
what chef
An important contribution to the project came from chef Maurizio Fantinato who validated the recipes developed by the scientific committee. The theoretical part was very well prepared by the technicians, says the chef, I adapted the recipes to a professional kitchen. I must say that they correspond very well not only to the needs of those with this disease, but also to the restaurant market. In fact, these are simple and easily understandable recipes, thanks also to the easy-to-read Cookbook.
Where are
Finding PKU friendly restaurants shouldn’t be difficult. provided that they are geolocalized and can also be identified thanks to the window stickers applied on the windows. Furthermore, the list of all the restaurants will be found on the PKUISINE website to which patients will also have the opportunity to report a restaurant to invite it to join the project.
July 2, 2022 (change July 2, 2022 | 09:36)
© Time.News