Profound metabolic acidosis and oxoprolinuria in an adult. 2007

Michael J Hodgman, and James F Horn, and Christine M Stork, and Jeanna M Marraffa, and Michael G Holland, and Richard Cantor, and Patti M Carmel
Bassett Healthcare, Cooperstown, NY, USA. hodgmanm@upstate.edu <hodgmanm@upstate.edu>

BACKGROUND Profound metabolic acidosis in critically ill adults sometimes remains unexplained despite extensive evaluation. METHODS A 58-year-old female presented in a confused state to the emergency department; she had been confused for several days. Laboratory evaluation revealed a high anion gap metabolic acidosis and modestly elevated acetaminophen level. Lactic acid was only modestly elevated. There was no evidence of ketoacids, salicylate, methanol, or ethylene glycol. A urine sample submitted on day 1 of hospitalization revealed a markedly elevated level of 5-oxoproline. CONCLUSIONS Originally described in children with an inherited defect of glutathione synthetase, 5-oxoproline is an unusual cause of metabolic acidosis. More recently this disturbance has been recognized in critically ill adults without a recognized inherited metabolic disorder. In most of these cases there has been the concomitant use of acetaminophen. Any causal relationship between acetaminophen and this disturbance is speculative. CONCLUSIONS In critically ill adults with unexplained metabolic acidosis, 5-Oxoproline should be considered in the differential.

UI MeSH Term Description Entries
D008659 Metabolic Diseases Generic term for diseases caused by an abnormal metabolic process. It can be congenital due to inherited enzyme abnormality (METABOLISM, INBORN ERRORS) or acquired due to disease of an endocrine organ or failure of a metabolically important organ such as the liver. (Stedman, 26th ed) Thesaurismosis,Diseases, Metabolic,Disease, Metabolic,Metabolic Disease,Thesaurismoses
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011761 Pyrrolidonecarboxylic Acid A cyclized derivative of L-GLUTAMIC ACID. Elevated blood levels may be associated with problems of GLUTAMINE or GLUTATHIONE metabolism. 5-Oxoproline,Pidolic Acid,Pyroglutamic Acid,5-Ketoproline,5-Oxopyrrolidine-2-Carboxylic Acid,Magnesium Pidolate,Pyroglutamate,Pidolate, Magnesium
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000082 Acetaminophen Analgesic antipyretic derivative of acetanilide. It has weak anti-inflammatory properties and is used as a common analgesic, but may cause liver, blood cell, and kidney damage. Acetamidophenol,Hydroxyacetanilide,Paracetamol,APAP,Acamol,Acephen,Acetaco,Acetominophen,Algotropyl,Anacin-3,Datril,N-(4-Hydroxyphenyl)acetanilide,N-Acetyl-p-aminophenol,Panadol,Tylenol,p-Acetamidophenol,p-Hydroxyacetanilide,Anacin 3,Anacin3
D000138 Acidosis A pathologic condition of acid accumulation or depletion of base in the body. The two main types are RESPIRATORY ACIDOSIS and metabolic acidosis, due to metabolic acid build up. Metabolic Acidosis,Acidoses,Acidoses, Metabolic,Acidosis, Metabolic,Metabolic Acidoses
D016638 Critical Illness A disease or state in which death is possible or imminent. Critically Ill,Critical Illnesses,Illness, Critical,Illnesses, Critical
D018712 Analgesics, Non-Narcotic A subclass of analgesic agents that typically do not bind to OPIOID RECEPTORS and are not addictive. Many non-narcotic analgesics are offered as NONPRESCRIPTION DRUGS. Non Opioid Analgesic,Non-Opioid Analgesic,Nonopioid Analgesic,Nonopioid Analgesics,Analgesics, Nonnarcotic,Analgesics, Nonopioid,Non-Opioid Analgesics,Analgesic, Non Opioid,Analgesic, Non-Opioid,Analgesic, Nonopioid,Analgesics, Non Narcotic,Analgesics, Non-Opioid,Non Opioid Analgesics,Non-Narcotic Analgesics,Nonnarcotic Analgesics,Opioid Analgesic, Non

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