title: A rare case of acquired methemoglobinemia associated with alkaptonuria.
abstract: We herein present a rare case of acquired methemoglobinemia associated with alkaptonuria. Alkaptonuria is a congenital error of metabolism caused by the deficiency of homogentisic acid oxidase, which subsequently results in the accumulation of homogentisic acid (HGA) in body tissues. As renal dysfunction progresses, the level of HGA excretion in the urine decreases and the blood concentration of HGA increases. HGA oxidizes oxyhemoglobin to methemoglobin, which can induce multiple organ failure accompanied by tissue hypoxia, intravascular hemolysis and metabolic acidosis. The mortality of this disease is high when alkaptonuria is associated with the presence of methemoglobinemia; therefore, treatment should be carefully planned in such cases.
authors.affiliation: Critical Care Medicine, University Hospital of Occupational and Environmental Health, Japan.
authors.forename: Yasuki
authors.initials: Y
authors.lastname: Isa
authors.forename: Shun-ichi
authors.initials: S
authors.lastname: Nihei
authors.forename: Yuna
authors.initials: Y
authors.lastname: Irifukuhama
authors.forename: Tomoya
authors.initials: T
authors.lastname: Ikeda
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Matsumoto
authors.forename: Keiji
authors.initials: K
authors.lastname: Nagata
authors.forename: Nobuya
authors.initials: N
authors.lastname: Harayama
authors.forename: Keiji
authors.initials: K
authors.lastname: Aibara
authors.forename: Masayuki
authors.initials: M
authors.lastname: Kamochi
mesh_terms: D000368:Aged; D000474:Alkaptonuria / Q000150:complications* / Q000175:diagnosis* / Q000378:metabolism; D017809:Fatal Outcome; D005260:Female; D006713:Homogentisic Acid / Q000378:metabolism; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology; D008708:Methemoglobinemia / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D035583:Rare Diseases
chemical_list: D006713:Homogentisic Acid