Urea cycle disorder--argininosuccinic lyase deficiency.
An increased level of ammonia in the bloodstream, or hyperammonemia, is a symptom associated with metabolic disorders referred to as inborn errors of metabolism. Urea cycle disorder is a congenital abnormality or absence of one of the six enzymes involved in the elimination of ammonia. Administration of certain medications, high protein diet, excessive exercise, surgical procedures, or trauma can precipitate symptoms of mental confusion, seizure-like activity, and ataxia. This paper reviews the literature with insight into current treatment and management options of the disorder and modification of treatment for the dental patient.
Department of Advanced Dentistry, School of Dentistry, University of Mississippi, Jackson, Mississippi, USA. nmehta@umc.edu
Neeta
N
Mehta
Pia Chatterjee
PC
Kirk
Ray
R
Holder
Harry V
HV
Precheur
D056807:Argininosuccinic Aciduria / Q000150:complications / Q000201:enzymology / Q000473:pathology; D051544:Cytochrome P-450 CYP3A / Q000378:metabolism; D017742:Dental Care for Chronically Ill; D004347:Drug Interactions; D006801:Humans; D022124:Hyperammonemia / Q000150:complications; D009059:Mouth Diseases / Q000209:etiology* / Q000628:therapy; D053210:Pharmaceutical Preparations, Dental / Q000009:adverse effects*
D053210:Pharmaceutical Preparations, Dental; D051544:Cytochrome P-450 CYP3A