Argininemia is caused by genetic mutation of the AGR1 gene and results in urea cycle disorder that leads to the overproduction of ammonia and arginine in the blood. Moreover, this disease leads to an imbalanced proliferation of ammonia and arginine in the body which results in serious health issues like coma, kidney and liver failure, UTI, and even death. However, the treatments of argininemia are dietary and some other drugs can support this treatment:
The treatments for Argininemia are as follows:
- Limited protein intake: in such conditions, the body can’t digest protein-rich foods and supplements. However, the unbroken protein elements reach the blood and result in an overproduction of toxins in the blood.
- Sodium benzoate: argininemia results in a lack of appetite that can be fixed by using drugs rich in sodium benzoate.
- Sodium phenylbutyrate: this salt is used with a proper diet to cure inherited disorders of the urea cycle. Moreover, it helps in removing ammonia from the body.
- Carglumic acid: this drug is also used to remove excess ammonia from the blood. This medicine is prescribed with the diet to prevent and treat hyperammonemia.
- Glycerol Phenylbutyrate: this is a drug used for patients having urea cycle disorder caused due to an inherited genetic disorder.
Causes harmful amounts of arginine and ammonia to build up in the body
Intravenous (IV) fluids,Protein intake limited
Sodium benzoate,Sodium phenylbutyrate,Carglumic acid,Glycerol phenylbutyrate,Palonosetron,Ondansetron hydrochloride
Lethargy,Dehydration,Hypotonia,Growth is stunted,Microcephaly,Seizures