Inborn Errors of Metabolism
Inborn errors of metabolism are rare genetic (inherited) disorders in which the body cannot properly turn food into energy. The disorders are usually caused by defects in specific proteins (enzymes) that help break down (metabolize) parts of food. Inborn errors of metabolism are a heterogeneous group of disorders that may be inherited or may occur as the result of spontaneous mutations. These diseases involve failure of the metabolic pathways involved in either the break-down or storage of carbohydrates, fatty acids, and proteins.
Biovencer Healthcare specialises in manufacturing of Inborn Errors of Metabolism (IEM) Supplements, we manufacture different specialised nutrition for Metabolic disorders like specialised Protein for PKU- Phenylketonuria Nutrition, Phenylalanine free Supplement, Protein Free Dietary Supplements, Supplement for Chronic Kidney Disease.
There are very few companies around the world that manufacture Medical Foods for Inborn Errors of Metabolism & Biovencer Healthcare is one of them.
There are many different disorders of protein metabolism. A common factor in their management is restriction of natural dietary protein to avoid the accumulation of toxins in the body. Disorder-specific protein substitutes are used to ensure protein requirements are met.
Biovencer produces a comprehensive range of innovative and lifestyle-friendly protein substitutes for the various disorders. We have also developed support products for this area, which can typically be used in any of the protein disorders.
There are many different disorders of fat metabolism. These include long-chain fatty acid oxidation disorders (LC-FAOD). Dietary fat is a rich source of energy. The overwhelming majority of regular dietary fat in western diets is long-chain triglyceride (LCT). Individuals with an LC-FAOD cannot release the energy from LCT. Instead, LCT leads to the release of toxins. Replacing LCT with a different type of fat known as medium-chain triglyceride (MCT) provides an alternative form of energy and helps prevent toxin accumulation.
There are many different disorders of carbohydrate metabolism. Carbohydrate metabolism is a fundamental biochemical process that ensures a constant supply of energy to living cells. The most important carbohydrate is glucose, which can be broken down via glycolysis, enter into the Kreb's cycle and oxidative phosphorylation to generate ATP.
PKU Formula 1
Biovencer Healthcare is the leading manufacturer & exporter of PKU Supplement.
PKU Powder is a supplement designed specifically for people with Phenylketonuria. In Phenylketonuria, the body cannot deal with the amino acid - Phenylalanine due to the lack of a particular enzyme.
In this case, a Glycomacropeptide based protein supplement is designed which will have all Amino acids but it's a Phenylalanine free supplement. This formula is suitable upto 3 years of age.
Composition: Contains all Amino acids except for Phenylalanine
Packaging- 400g Tin Pack
PKU Formula 2
Biovencer Healthcare is the leading manufacturer & exporter of PKU Supplement.
PKU Powder is a supplement designed specifically for people with Phenylketonuria. In Phenylketonuria, the body cannot deal with the amino acid - Phenylalanine due to the lack of a particular enzyme.
In this case, a Glycomacropeptide based protein supplement is designed which will have all Amino acids but it's a Phenylalanine free supplement. This formula is suitable Above 3 years of age.
Composition: Contains all Amino acids except for Phenylalanine
Packaging- 400g Tin Pack
PKU Formula 3
Biovencer Healthcare is the leading manufacturer & exporter of PKU Supplement.
PKU Powder is a supplement designed specifically for people with Phenylketonuria. In Phenylketonuria, the body cannot deal with the amino acid - Phenylalanine due to the lack of a particular enzyme.
In this case, a Glycomacropeptide based protein supplement is designed which will have all Amino acids but it's a Phenylalanine free supplement. This formula is suitable Above 3 years of age.
Composition: Contains all Amino acids except for Phenylalanine
Packaging- 400g Tin Pack