title: Perioperative management of glucose 6 phosphate dehydrogenase deficiency. A review of the literature.
abstract: Glucose 6 phosphate dehydrogenase (G6PDH) deficiency is the most frequent cause of hemolytic anemias due to enzyme abnormality. Perioperative management must be careful to avoid the onset of hemolytic crisis. We present a complete review of the literature on this illness and describe the perioperative management of an adult with known G6PD deficiency and the pathogenesis and clinical manifestations of the disorder and its possible anesthetic implications are discussed. A 49-year-old patient had undergone varum osteotomy in her left knee due to genu valgum. She had been diagnosed as having G6PDH deficiency sixteen years earlier provoked by ingesting beans. The perioperative circumstances capable of causing autohemolysis are described and discussed. In spite of the fact that the pattern is self-limited, it provokes the onset of jaundice and anemia which can complicate the recovery. Simple elimination of those elements which precipitate with oxyhemoglobin will allow an uneventful anesthetic procedure.
authors.affiliation: Clinica Puerta de Hierro Madrid, España.
authors.forename: L
authors.initials: L
authors.lastname: Muñoz Corsini
authors.forename: E
authors.initials: E
authors.lastname: Dominguez
authors.forename: I
authors.initials: I
authors.lastname: Mourelle
authors.forename: S
authors.initials: S
authors.lastname: Galindo
authors.forename: M C
authors.initials: MC
authors.lastname: Porras
mesh_terms: D000743:Anemia, Hemolytic / Q000517:prevention & control; D005260:Female; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000628:therapy*; D006801:Humans; D007432:Intraoperative Period; D016916:Joint Deformities, Acquired / Q000601:surgery; D007719:Knee Joint / Q000601:surgery; D008875:Middle Aged; D010027:Osteotomy; D011184:Postoperative Period