Developing Medical Food Formulas for Pediatric Patients with Organic Acid Metabolism Disorders: A Project by Mahidol University

2023-09-19 15:14:02

Health19 Sept. 2023 22:11

Protein is a nutrient that is essential to the body at all ages. Protein is made up of 20 types of amino acids in nature, which vary according to the type of protein. Amino acids include essential amino acids that the body cannot synthesize. and non-essential amino acids which the body can synthesize itself When we eat food that contains protein, The body will have to digest and absorb amino acids. And there is a metabolic process that causes those amino acids to perform various necessary functions. in the body normally

However, there are some diseases that prevent the normal metabolism of amino acids from occurring. such as “Organic Acid Metabolism Disease” which is a genetic disorder.

Diseases in this group are very rare. It is classified as a rare disease, found to occur in 1 person in 25,000 people. It is an abnormality of the metabolism of amino acids called leucine, isoleucine, and valine. Symptoms have been present since birth. This is because the body is unable to process the amino acids it receives from milk. Causes toxic substances, resulting in brain symptoms such as depression, convulsions, fetuses, or abnormal body odors caused by those toxic substances.

Some patients with severe symptoms Resulting in cerebral palsy. or death In cases where the disease is less severe, symptoms may include developmental delays and frequent vomiting. Symptoms usually occur in the early stages of life.

Important treatments for this group of diseases include nutritional therapy. Patients with metabolic defects in this organic acid Protein is required from food for growth. and used in the functioning of the body But the intake of problematic amino acids, including leucine, isoleucine, and valine, must be limited in order to avoid causing symptoms of toxicity.

However, eating protein foods in nature The amount of such amino acids cannot be controlled. Therefore it is necessary to eat food together with “Special medical food” that is produced to treat specific diseases in appropriate proportions under the supervision of a medical professional

Treatment of patients with organic acid metabolism disorders From the past to the present in Thailand There are no special medical food products for use within the country. But periodically it receives donations from abroad to use. which results in uncertainty There is a risk of product shortages during various crisis situations. such as a major flood event The outbreak of COVID-19, for example, affects treatment. and the patient’s condition is very serious

Assistant Professor Dr. Oraporn M.D. Damrongwongsiri, Head of Nutrition Department Department of Pediatrics Faculty of Medicine Ramathibodi Hospital Mahidol University and research team Therefore, there is an idea to develop a protein product to treat patients with organic acid metabolism disorders that can be produced and used in Thailand. and has a reasonable price To be sustainable and food security for this group of patients, therefore, a “project to develop medical formulas for treating pediatric patients with organic acid metabolism defects” has been undertaken.

This project is a further development from Ms. Suthida’s idea. Chatwuttinun PhD student from the Institute of Nutrition Mahidol University Thinking of searching for foods that are low in the amino acids leucine, isoleucine, and valine.

Until it became a “prototype food innovation” that used important ingredients from “Sunflower seeds” and “Fish maw” and used in real trials in patients This study has been published in an international academic journal. This leads to hope to develop food products for patients with such genetic diseases in Thailand.

Later, the research team “Project to develop medical food formulas for treating pediatric patients with organic acid metabolism disorders” has further developed food products from prototype studies. To aim to increase the efficiency of nutritional treatment for patients with such diseases.

In this development The research team selected the raw materials: “Sunflower seeds” and supplemented with synthetic amino acids according to a formula specifically developed for various metabolic diseases. Before adding flavor and flavor to mellow The product has undergone physical product testing. and sensory testing in adult volunteers has been completed. And the research team is preparing to test the product in treating real patients.

Although “organic acid metabolism disorder” is a rare disease, But when it occurs, it is severe and affects the patient’s development greatly. The main treatment is nutritional treatment. which at present in Thailand There is no such product available for use within the country.

Mahidol University ready to do duty “Wisdom of the Land” according to the aspiration of Mahidol University Ready to provide knowledge to develop more effective treatment for patients with such diseases. and expand the results to further develop the care system for sustainability. With the heart of humanity that never abandons each other

Follow interesting news from Mahidol University at www.mahidol.ac.th.

Source: Mahidol University

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