Hyperargininemia Summary

76 relevant articles (0 outcomes, 2 trials/studies) found for this Disease

Description: A rare autosomal recessive disorder of the urea cycle. It is caused by a deficiency of the hepatic enzyme ARGINASE. Arginine is elevated in the blood and cerebrospinal fluid, and periodic hyperammonemia may occur. Disease onset is usually in infancy or early childhood. Clinical manifestations include seizures, microcephaly, progressive mental impairment, hypotonia, ataxia, spastic diplegia, and quadriparesis. (From Hum Genet 1993 Mar;91(1):1-5; Menkes, Textbook of Child Neurology, 5th ed, p51)

Also Known As: Deficiency Disease, Arginase; Arginase Deficiency Diseases; Deficiency Diseases, Arginase; Hyperargininemias; Arginase Deficiency Disease Show All >>

Key Drugs and Agents for Hyperargininemia

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Drugs and Important Biological Agents (IBA) related to treatments:

Homoarginine : 2 studies in 6 results : IBA
argininic acid : 2 studies in 5 results : IBA
Arginine (L-Arginine) : 1 study in 24 results : FDA 26
Adenosine Triphosphatases (ATPase) : 1 study in 1 result : IBA
Arginase : 44 results : IBA
Urea (Carbamide) : 11 results : FDA 6 Generic
Lysine (L-Lysine) : 5 results : FDA 30
Ammonia : 5 results : IBA
Sodium Benzoate : 4 results : FDA 2
DNA (Deoxyribonucleic Acid) : 3 results : IBA

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Diseases Related to Hyperargininemia

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Key Therapies for Hyperargininemia

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