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Metabolic disorders & formulas

The process of digestion involves the body using chemical reactions to break down food into energy. Food contains proteins, carbohydrates and fats that your digestive system converts into fuel in the form of sugar and acids.

For some people, this normal metabolic process doesn’t work properly. When the body can’t carry on the chemical reactions needed to digest food, or they’re disrupted in some way, it may be as a result of a metabolic disorder. To help those with metabolic disorders get the nutrition they need, part of their treatment plan may involve using metabolic formulas, which are designed specifically to aid in digestion and absorption of nutrients.

Below is a list of some of the metabolic disorders that may involve using formula to aid in managing the condition.

 

PKU (phenylketonuria)

A disorder characterized by elevated levels of the amino acid Phe (phenylalanine). In abnormally high levels, Phe is toxic to the brain. If left untreated, it can cause problems with brain development, leading to progressive mental retardation, brain damage, seizures, mood and behavioral disorders.

 

Homocystinuria

An inherited disorder characterized by elevated levels of the amino acid methionine. Symptoms often show up during infancy and may include mental retardation, skeletal abnormalities and blood clots.

 

Tyrosinemia

An inherited disorder characterized by elevated levels of the amino acid tyrosine. The buildup of tyrosine can lead to serious medical problems, including abnormalities of the liver and kidneys.

 

Glutaric aciduria

A disorder characterized by elevated levels of the amino acids lysine, hydroxylysine and tryptophan. Glutaric aciduria is divided into multiple types, with symptoms that include damage to the brain region that controls movement.

 

MSUD (maple syrup urine disease)

A disorder characterized by elevated levels of specific amino acids. The condition gets its name from the distinctive sweet odor produced in the urine. Symptoms can start in early infancy and include poor feeding, vomiting, lethargy and developmental delay. If left untreated, MSUD can be life threatening.

 

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SOURCES
www.nlm.nih.gov/medlineplus/metabolicdisorders.html
www.nlm.nih.gov/medlineplus/ency/article/001166.htm
www.merckmanuals.com/home/print/childrens_health_issues/hereditary_metabolic_disorders/disorders_of_amino_acid_metabolism.html
www.nlm.nih.gov/medlineplus/ency/article/001199.htm
www.rarediseases.info.nih.gov/gard/6522/glutaric-acidemia-type-i/resources/1
www.rarediseases.info.nih.gov/gard/3228/maple-syrup-urine-disease/resources/1