Genetic Deficiencies-Elevated blood Ammonia/Urea cycle?
Although rare, humans are occasionally born with genetic deficiencies in the enzymes of the urea cycle. Such individuals have elevated blood ammonia, which can be life threatening. One useful therapy is to feed these people benzoic acid, which is secreted in the urine as hippuric acid, an amide formed between benzoic acid and glycine. What is the logic behind this therapy?
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In urea cycle disorders, the nitrogen accumulates in the form of ammonia, a highly toxic substance, resulting in hyperammonemia. mmonia then reaches the brain through the blood, where it can cause irreversible brain damage, coma and/or death.The treatment of urea cycle disorders consists of dietary management to limit ammonia production in conjunction with medications and/or supplements which provide alternative pathways for the removal of ammonia from the bloodstream.When optimal treatment fails,liver transplantation is doing