Vegetables that are High in methionine/ per 100 gm (more than 25mg – 50mg of methionine/ 100 gm of the product) Broccoli, Mushroom, Cauliflower, Avocado, Bean sprouts, potatoes, Vegetables that are High in methionine/ per 100 gm (more than 50mg of methionine/ 100 gm of the product) Spinach, Green Peas, corn
Read moreMSUD (Maple Syrup Urine Disease)
MSUD is an autosomal recessive metabolic disorder affecting branched-chain amino acids. It is one type of organic academia. The condition gets its name from the distinctive sweet odor of affected infants’ urine, particularly prior to diagnosis, and during times of acute illness. Maple syrup urine disease can be classified by
Read moreFood and Formula Vendors
Vendor Products Website 1 ABBOTT LAB Formula https://abbottnutrition.com/therapeutic 2 CAMBROOKE THERAPEUTICS Formula & Food Products https://www.cambrooke.com/ 3 FLAVIS Food Products https://www.flavis.com/en 4 LILSDIETARY Food Products https://lilsdietary.com/ 5 MEDJOHNSON NUTRITION Formula https://www.meadjohnson.com/ 6 NUTRICIA METABOLICS Formula & Food Products http://medicalfood.com/ 7 PKU PERSPECTIVES Food Products https://www.pkuperspectives.com/ 8 PHARMA NORTH AMERICA Formula
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General Approach
Low-protein food products are recommended to persons with particular types of metabolic disorders such as Homocystinuria (HCU), Phenylketonuria (PKU), Methylmalonic Acidemia(MMA) and Tyrosinemia. Each of these disorders require food products which are low in particular type of amino-acid. i.e. methonine in the case of HCU, phenylalanine in the case of
Read moreTyrosinemia
Tyrosinemia is an error of metabolism, usually inborn, in which the body cannot effectively break down the amino acid tyrosine. Symptoms of untreated tyrosinemia include liver and kidney disturbances. Without treatment, tyrosinemia leads to liver failure. Today, tyrosinemia is increasingly detected on newborn screening tests before any symptoms appear. With
Read moreOrganic Acedemia(MMA,PCC)
Methylmalonic acidemia ( MMA )Methylmalonic acidemia , also called methylmalonic acidurias is an autosomal recessive metabolic disorder that disrupts normal amino acid metabolism. It is a classical type of organic acidemia. The result of this condition is the inability to properly digest specific fats and proteins, which in turn leads
Read morePKU (Phenylketonuria)
Phenylketonuria (PKU) is a metabolic disorder that results in decreased metabolism of the amino acid phenylalanine. Untreated, PKU can lead to intellectual disability, seizures, behavioral problems. A baby born to a mother who has poorly treated PKU may have heart problems, a small head, and low birth weight., and
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