Sapropterin dihydrochloride
Sapropterin Dihydrochloride
Sapropterin dihydrochloride (pronunciation: sah-proh-TEER-in dye-HYE-droe-KLOR-ide) is a synthetic form of a naturally occurring substance in the body called tetrahydrobiopterin (BH4). It is used to treat phenylketonuria (PKU), a genetic disorder that can cause intellectual disability if not managed.
Etymology
The term "sapropterin" is derived from the Greek words "sapros" meaning rotten and "pterin" meaning wing, referring to the chemical structure of the compound. "Dihydrochloride" refers to the presence of two hydrogen chloride groups in the compound.
Usage
Sapropterin dihydrochloride is used in conjunction with a phenylalanine restricted diet to reduce blood phenylalanine levels in patients with hyperphenylalaninemia (HPA) and PKU. It works by helping the body to break down phenylalanine more effectively.
Related Terms
- Phenylketonuria: A genetic disorder in which the body can't break down the amino acid phenylalanine.
- Hyperphenylalaninemia: A condition characterized by an abnormally high level of phenylalanine in the blood.
- Tetrahydrobiopterin: A coenzyme that helps break down several amino acids, including phenylalanine.
Side Effects
Common side effects of sapropterin dihydrochloride include headache, runny nose, and gastrointestinal problems. Serious side effects may include allergic reactions and mood changes.
Precautions
Before taking sapropterin dihydrochloride, patients should inform their healthcare provider if they have a history of stomach ulcers or esophagus problems. It is also important to monitor phenylalanine levels regularly while taking this medication.
External links
- Medical encyclopedia article on Sapropterin dihydrochloride
- Wikipedia's article - Sapropterin dihydrochloride
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